REVIEW
Clinical, dermoscopic, and histopathologic features of body hair disorders Ratchathorn Panchaprateep, MD, PhD,a Aline Tanus, MD,b and Antonella Tosti, MDc Bangkok, Thailand; Rio de Janeiro, Brazil; and Miami, Florida Dermoscopic examination of hair and scalp, also named ‘‘trichoscopy,’’ is an essential tool in diagnosis of hair and scalp diseases. Trichoscopy is fast and noninvasive and can be used to evaluate hair disorders in all body areas. Body hair disorders are uncommon, and most publications on their dermoscopic features are limited to case reports or series. In this review we present the available information on the dermoscopic diagnosis of body hair disorders including keratosis pilaris, trichostasis spinulosa, pili multigemini, circle hairs, rolled hairs, eruptive vellus hair cyst, and ingrown hairs. ( J Am Acad Dermatol http://dx.doi.org/ 10.1016/j.jaad.2015.01.024.) Key words: circle hairs; dermoscopy; eruptive vellus hair cysts; ingrown hairs; keratosis pilaris; pili multigemini; pseudofolliculitis; rolled hairs; trichoscopy; trichostasis spinulosa.
T
he use of dermoscopy dramatically changed the diagnostic approach to hair and scalp disorders as it is fast, noninvasive, and easy to use. It has become an essential tool for all dermatologists and other physicians in evaluation of patients with hair problems. Dermoscopy is not only useful for diagnosis of scalp hair disorders, but also applicable to body hair disorders including conditions such as keratosis pilaris, trichostasis spinulosa, pili multigemini, circle hairs, rolled hairs, and eruptive vellus hair cyst (EVHC). Dermoscopy also allows physicians to scan the abnormalities of entire body hairs quickly. Furthermore, dermoscopy reduces the necessity of performing painful diagnostic procedures such as hair plucking and skin biopsy. The aim of this article is to review the role of dermoscopy in the diagnosis of body hair disorders and review clinical features and diagnosis of body hair disorders.
METHODS We identified relevant articles by the systematic search of scientific and medical electronic search engines (PubMed, MEDLINE and Scopus) in September 2014. Key terms for searches included: From the Division of Dermatology, Department of Medicine, King Chulalongkorn Memorial Hospital, Bangkoka; Instituto de Dermatologia Professor Rubem David Azulay, Rio de Janeirob; and Department of Dermatology and Cutaneous Surgery, University of Miami L. Miller School of Medicine.c Funding sources: None. Conflicts of interest: None declared. Accepted for publication January 14, 2015. Reprint requests: Ratchathorn Panchaprateep, MD, PhD, Division of Dermatology, Department of Medicine, Faculty of Medicine,
‘‘body hairs,’’ ‘‘body hair disorders,’’ ‘‘dermoscopy,’’ ‘‘dermatoscopy’’ and ‘‘trichoscopy’’ combined with ‘‘keratosis pilaris,’’ ‘‘trichostasis spinulosa,’’ ‘‘pili multigemini,’’ ‘‘pili bifurcati,’’ ‘‘circle hairs,’’ ‘‘rolled hairs,’’ ‘‘eruptive vellus hair cyst,’’ ‘‘ingrown hairs,’’ and ‘‘pseudofolliculitis barbae.’’ Relevant titles, abstracts, and full articles identified from the search were reviewed and included if pertinent to clinical, dermoscopic, and histopathologic features of body hair disorders. Additional articles were also retrieved from the cited reference list of articles identified from our initial systematic online literature search. We limited our search to articles available in the English language. Biology of body hair Human hairs can be classified into 7 main types based on their physical and functional properties: scalp, eyebrow and eyelash, beard and moustache, body hair, pubic hair, axillary hair, and arm and leg1 (Table I). Physically, hair shafts from different locations on the body reveal distinguishable length, structure, and cuticle protein profiles that are different from scalp hairs.2 Body hair or nonscalp Chulalongkorn University, King Chulalongkorn Memorial Hospital, 1873, Rama IV Road, Pathumwan, Bangkok, Thailand 10330. E-mail: [email protected]. Published online March 5, 2015. 0190-9622/$36.00 Ó 2015 by the American Academy of Dermatology, Inc. http://dx.doi.org/10.1016/j.jaad.2015.01.024
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hair shows marked variation in density, distribution, approximately 1 mm in size, resembling gooseflesh and patterning. Hair cycle of body hair follicles is with varying degrees of perifollicular erythema/ also dissimilar to scalp. The body hair follicles have inflammation. Keratosis pilaris lesions often contain prolonged and increased telogen frequency.3 Most a fine-coiled, brittle hair.11 Most lesions are usually 4 body hairs begin as vellus hairs. Increasing asymptomatic but may be pruritic. The common affected body sites are the extensor surfaces of the androgen levels during puberty cause them to transupper aspect of arms, thighs, face, buttocks, and form into terminal hairs over many areas of the body. eyebrows.6,11 To our knowlThere is a sexual differentiation in the amount and disedge, there is just 1 publicaCAPSULE SUMMARY tribution of androgenic hair, tion about dermoscopy of with men tending to have keratosis pilaris reporting Body hair disorders are uncommon and more terminal hairs in more dermal vascular ectasia and can be congenital or acquired. areas including the face, follicular hyperkeratosis.12 In Dermoscopy is useful to confirm chest, abdomen, legs, arms, our experience, in mild diagnosis. and feet.5 cases, dermoscopy shows Clinical presentation and dermoscopic vellus hairs, frequently findings of common body hair disorders twisted or coiled, surBody hair disorders are reviewed including keratosis pilaris, rounded by peripilar casts. Body hair disorders are a trichostasis spinulosa, pili multigemini, Groups of 2 or 3 hairs may group of congenital or accircle hairs, rolled hairs, and eruptive emerge together. In more sequired alterations. Congenital vellus hair cyst. vere cases, vellus hairs are disorders can be an isolated coiled and embedded in the finding or a part of complex Dermatologists will be able to use horny layer. Perifollicular ersyndromes, for example, dermoscopyea fast, noninvasive, and ythema and hyperpigmentakeratosis pilaris or follicular cost-efficient methodeto diagnose body tion is frequently seen keratosis. Acquired body hair disorders. The painful diagnostic (Fig 1). These dermoscopic hair disorders can be caused procedures such as hair plucking and findings are correlated with by trauma. The diagnostic skin biopsy are necessary only in classic histopathologic finddermoscopic findings for uncharacteristic cases. ings described as distention most common body hair disof the follicular orifice by a orders are summarized in keratinous plug that may contain 1 or more twisted Table II. Body hair disorders can be categorized hairs. Although diagnosis of keratosis pilaris rubra into 2 main groups: follicular disorders with 1 or and erythromelanosis follicularis faciei et colli is more vellus hairs emerging from the same follicular mainly clinical, dermoscopy can be helpful in opening (Table III) and circle, rolled, or twisted hairs showing perifollicular erythema and vascular ectasia and ingrown hairs. (Fig 2).13,14 Dermoscopy is also helpful in differentiating keratosis pilaris rubra from follicular lichen Keratosis pilaris planus that shows similar clinical features of follicKeratosis pilaris is a common autosomal domiular spinous papules on the body. In follicular lichen nant disorder of follicular hyperkeratosis characplanus, dermoscopy shows follicular keratotic plugs terized by keratinous plugs in the follicular without broken or twisted hairs (Fig 3). orifices and varying degrees of perifollicular eryDermoscopy is useful not only for diagnosis of thema. In general, keratosis pilaris is cosmetically keratosis pilaris, but also for monitoring unpleasant but medically harmless. Studies noted treatment outcome. A recent study demonstrated a prevalence of 16% to 44% in dermatologic that dermoscopy is a promising tool to assess patients.6,7 The survey showed 61% were female localized improvement in skin architecture after and 39% were male.6,8,9 Age of onset of keratosis receiving treatment in pediatric patients with pilaris was within the first decade in 51%, second atopic dermatitis, ichthyosis vulgaris, and keradecade in 35%, and third and fourth decades in tosis pilaris.15 14%.6 The pathogenesis of keratosis pilaris is still not totally understood. The most accepted theory proposes defective keratinization of the follicular Trichostasis spinulosa epithelium resulting in a keratotic infundibular Trichostasis spinulosa is a relatively common plug.10 but underdiagnosed follicular disorder characterized by hyperkeratosis of a dilated hair follicle Clinical features of keratosis pilaris are multiple with retention of multiple telogen vellus hairs. The small grayewhite, folliculocentric keratotic papules, d
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Table I. Characteristics of scalp and body hairs in each location Hair type
Length, range, mm
Scalp Eyebrow and eyelash
100-1000 5-10
Beard and moustache
50-300
Body (chest, trunk, back)
3-60
Pubic
10-60
Axillary
10-50
Arm and leg
5-20
Description
Medullated with tapered tip in uncut hair Large medullated and coarse, curved with punctuate tip Complex medullary processes, more irregular in structure, blunt tip Irregular medullated, fine long tip, irregular in structure Coarse, kinked, irregular and asymmetric cross-section, many constrictions and twists Coarse, less kinked than public hair, blunt tip, often abraded as a result of friction Irregular medullated, blunt tip
Table II. Diagnostic dermoscopic findings in most common body hair disorders Body hair disorders
Keratosis pilaris
Trichostasis spinulosa Pili multigemini
Eruptive vellus hair cysts Circle hairs
Rolled hairs Ingrowing hairs
Diagnostic dermoscopic findings
- 1-3 Vellus hairs emerging from a single opening - Coiled hairs often embedded in the horny layer - Peripilar casts - Perifollicular erythema - Tuft of short, vellus hairs emerging from the same follicular openings - Keratotic plugs within dilated follicle - Bundles of multiple pigmented hairs of similar thickness emerging from the same follicular openings - Each tuft is surrounded by a peripilar cast - Perifollicular erythema - Circular structure, with central yellowish to whitish papule - Erythematous brownish halo - Dark hairs regularly coiled to form a circle under a translucent layer of stratum corneum - No signs of perifollicular inflammation - Hairs irregularly twisted in spirals - Peripilar casts - U-shaped ingrowing hairs beneath a papular lesion (transfollicular penetration)
origin of trichostasis spinulosa is still unknown. Congenital dysplasia of the hair follicles and external factors such as dust, oils, heat, ultraviolet light, and industrial irritants have been proposed.16 One study reported the presence of Pityrosporum (Malassezia yeast) (82.6%) and
Duration of anagen phase
A:T ratio
2-6 y 4-8 wk
90:10 10:90
4-14 wk
60:40
11-12 wk
30:70
16 wk
30:70
16 wk
30:70
6-12 wk (arm) 19-26 wk (leg)
20:80
bacteria especially Propionibacterium acnes (73.3%) in the extracted follicular material and biopsied specimen of trichostasis spinulosa, suggesting that these micro-organisms may be some of the possible etiologic factors.17 Clinically, blackhead comedo-like lesions or horny, spinous plugs are seen primarily on the tip of nose, alae nasi, and cheeks. The lesions themselves are usually asymptomatic, but a mild degree of folliculitis can be found. Trichostasis spinulosa can also manifest as itchy papules on the trunk and upper extremities in young adults.16,18 Diffuse trichostasis spinulosa has been reported in chronic renal failure.19 Histologically, dilated hair follicle infundibulum with numerous pigmented vellus hairs (up to 50 hairs) and keratinous material are observed. On histopathology, hair shaft entrapment in trichostasis spinulosa results from hyperkeratosis in the follicular infundibulum.14 Many methods have been described to assist in diagnosis such as microscopic examination and standard skin surface biopsy of the contents of black papules, however dermoscopy is the most helpful as it is fast and easy.20 Dermoscopic findings include tufts of multiple, slightly pigmented to pigmented, vellus hairs with varying diameters projecting or emerging from dilated follicular openings. Other follicular openings are occluded by keratotic plugs (Fig 4). Pili multigemini Pili multigemini is an uncommon developmental defect of the hair follicles in which more than 1 hair shaft emerges from the same hair follicle. Each hair has its own papilla, matrix, and inner root sheath but
Keratosis pilaris
Trichostasis spinulosa
Eruptive vellus hair cysts
Occlusion and cyst dilatation of vellus hair follicle
Follicular disorder that results from retention of multiple vellus hairs within sebaceous follicles Middle aged or elderly Face, especially the nose; chest and back (interscapular area) Comedone-like or horny, spinous plugs, sometimes dilated orifice
Multiple small red or brown papules
Microscopy of plucked hairs
Usually a single coiled vellus hair surrounded by keratin
Histopathologic findings
Dilated vellus hair follicle containing keratin and a coiled vellus hair; mild perifollicular mononuclear cell infiltration may be present
Material from eruptive vellus hair cysts is difficult to obtain as lesions locate in dermis Epithelial-lined cyst with several vellus hairs in a keratinaceous matrix
Clinical differential diagnosis
Trichostasis spinulosa, phrynoderma from vitamin A deficiency, follicular keratosis in several nutritional deficiencies, lichen nitidus, lichen spinulosus, folliculitis, pityriasis rubra pilaris, scurvy
Several vellus hairs organized in a bundle within a keratotic material Acanthotic epidermis surrounding a dilated vellus hair follicle containing several vellus hairs arranged in an organized bundle embedded in a keratin plug; mononuclear cell infiltration may be present around follicles and arrector pili muscle Comedogenic acne, keratosis pilaris, eruptive vellus hair cysts, Favre-Racouchot syndrome
Age Common location
Clinical presentation
Childhood, second decade of life Chest and abdominal wall
Follicular nevi (congenital vellus hamartoma)
Pili multigemini
Composite papilla forms several hair shafts separated from each other by IRS but enclosed in a common ORS Childhood to adolescence Face, especially along the lines of the jaw Remarkably thicker and fuller hairs in the affected area or report of recurrent inflammatory papules, leaving scars Multiple hair shafts from composite dermal papilla enclosed in a common ORS Multiple separated hair shafts enclosed in a common ORS but separated from each other by layers of IRS
Tufted hairs; composite hairs/ compound follicles
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Follicular keratotic disorders characterized by a prominent plug of keratin within the follicular orifice Childhood to adolescence Extensor surfaces of the upper aspect of arms, thighs, and buttocks Multiple keratotic follicular papules, often with a rim of erythema; easily removed
Definition
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Table III. Common disorder of follicular disorders with multiple hairs projecting from hair follicles
Benign and cancerous lesions such as melanocytic nevi, seborrheic keratoses, syringomas, epidermoid cysts, eruptive vellus hair cysts, nodular basal cell carcinomas
Overlapping with steatocystoma multiplex, chronic renal failure, pachyonychia congenita
Trichofollicular-like organoid nevus
Keratolytic agents, cyanoacrylic adhesive, hair removal lasers; no resolutionpermanent
Topical retinoid, curettage, laser (carbon dioxide, erbium:yttrium-aluminiumgarnet)
Q-switched ruby laser (case report)
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Treatment
Atopic dermatitis, ichthyosis vulgaris, acne vulgaris, diabetic type 1, Graham-Little syndrome, keratosis follicularis, spinulosa decalvans, erythromelanosis follicularis faciei et colli, hypotrichosis with keratosis pilaris, monilethrix, pachyonychia congenital, ectodermal dysplasia, taking systemic steroid or lithium therapy, Down syndrome, Noonan and cardiofacial-cutaneous syndromes, renal insufficiency, Fairbanks syndrome, Olmsted syndrome Improve with increasing age; no absolute cure; emollient, keratolytic agents, topical retinoids, topical steroids, laser therapy
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Associated conditions
IRS, Inner root sheath; ORS, outer root sheath.
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Fig 1. Keratosis pilaris. A, Clinical photograph of keratosis pilaris on upper aspect of arms. B, Dermoscopy shows irregularly coiled vellus hairs embedded into the horny layer. Perifollicular erythema is also observed. C, High magnification of a coiled hair embedded in the horny layer. D, In milder cases, twisted velllus hair with thin peripilar casts.
Fig 2. Keratosis pilaris rubra. Dermoscopy shows irregularly coiled vellus hairs embedded into the horny layer with perifollicular erythema and underlying vascular ectasia.
shares a common outer root sheath.21 Most cases of pili multigemini have been found incidentally. A recent survey shows only 2% of patients presenting with other dermatologic symptoms had
the condition.22 Pili multigemini is observed in association with folliculitis and rare malformations such as cleidocranial dysostosis.23,24 One reported case revealed pili multigemini in linear distribution following Blaschko lines.25 Clinically, this condition should be suspected in male patients showing remarkably thick hair. Patients may report recurrent inflammatory papules, leaving atrophic/hypertrophic scars. Multigeminate follicles occur mainly on the face especially along jaw lines, however, they can be found in all body regions.26 To our knowledge, there are no published articles on dermoscopy of pili multigemini. In our recent case, dermoscopy showed bundles of multiple pigmented hairs of similar thickness emerging from each hair follicle (Fig 5). Each tuft of hairs was surrounded by a peripilar cast. Histologic examination of pili multigemini reveals complicated follicular structures forming from 2 to as many as 10 hair shafts. Each hair is formed by a single
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that are representative of common overlapping features of steatocystoma multiplex and EVHC.14,35
Fig 3. Follicular lichen planus. Dermoscopy shows follicular keratotic plugs without broken or twisted hairs.
branch of dermal papilla that is surrounded by all layers present in a normal follicle except for the outer root sheath cells. The outer root sheath surrounds the entire follicle. Irregularities in configuration of the hairs, longitudinal grooving, and areas of bifurcation and re-adhesion of the hair shafts can be demonstrated.27 Eruptive vellus hair cyst EVHC was first described by Esterly et al28 in 1977. EVHC presents as multiple asymptomatic papules, with a smooth or centrally umbilicated surface.29,30 The lesions usually affect children and young adults. They are located mostly on the chest and abdominal wall.30,31 Most often EVHC is sporadic; however, some cases with autosomal dominant inheritance have been reported.32 Coexistence of EVHC and steatocystoma multiplex in the same area is quite common. They are believed to be a result of the same disease process originating in pilosebaceous duct.33,34 Dermoscopic findings of EVHC include welldefined, whitish to yellowish, circular structures with erythematous brownish halos. A central gray and blue color point can be observed because of the presence of melanin within the pigmented hair shaft in the cyst (Fig 6).32 A recent study described dermoscopy of EVHC of the labia, demonstrating the follicular openings of the cysts in the epidermis.31 Histopathologic findings of EVHC reveal small epidermoid cyst containing multiple vellus hairs
Circle hair Circle hair, also called spiral hair, is characterized by the presence of dark hairs with perfect circle arrangement located under a thin layer of stratum corneum, without any signs of perifollicular inflammation.36-39 Circle hair occurs in overweight, middle-aged men with abundant body hairs. Most cases of circle hair are found only incidentally, on the abdomen, back, trunk, thighs, and upper aspect of the legs, where they are surrounded by neighboring normal hairs.37 There is no consensus on the pathogenesis of the circle hair. Some authors believe that circle hair has a very thin diameter that is incapable of perforating the stratum corneum and thus remains, growing in a spiral manner, in subcorneum. However, other authors think that the follicular ostium is always open and hair does not need to penetrate the corneal layer.38 Currently, circle hair is considered to be a genetically determined disorder corresponding to vestigial (or remnants) of the mammal undercoat.39 Dermoscopy demonstrates a hair shaft with a slightly smaller diameter rolled in perfect or nearly perfect concentric circles40 (Fig 7). The circle hair is easily extracted after superficial scraping revealing a hair with a question mark shape that can reach 2 to 2.5 cm. When released, the hair returns back to its original format with partially or fully recoiled shape.38,39 Under microscopy, circle hair shows dystrophic bulb in anagen phase, absence of inner root sheath, and narrowing of distal hair shaft forming circular track. Histopathology of circle hair includes presence of ‘‘river bed’’ dilated follicle infundibulum with entrapped hair shafts. No evidence of perifollicular inflammation, follicular obstruction, or reduction of hair shaft diameter was seen.40 Rolled hair The incidence of rolled hair is more frequent than circle hair. Rolled hair has been associated with many conditions including corticosteroid and cyclosporin therapy, ichthyosis, keratosis pilaris, xerosis, neurodermatitis, and palmoplantar keratoderma.41-44 However, some authors believe that rolled hair is caused by mechanical trauma as a result of repeated and vigorous rubbing.45 Rolled hair most commonly affects the extensor extremities, but also the back, shoulders, and buttocks.40 On dermoscopy, rolled hair shows irregularly twisted hair into a hyperkeratotic papule.46 When the keratin follicular plug is removed, it reveals the rolled hair that is inside the follicle
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Fig 4. Trichostasis spinulosa. A, Clinical photograph of trichostasis spinulosa on the nose. B and C, Dermoscopy shows tufts of short, vellus hairs emerging together and keratotic plugs of some follicular openings.
Fig 5. Pili multigemini. Dermoscopy shows bundles of pigmented hairs emerging from the same hair follicle. These hairs are equal in thickness. Each tuft is surrounded by a peripilar cast. Erythema can be observed around some openings.
(Fig 8).43 Histologic findings are mostly follicular hyperkeratosis, and it is believed that the rolled hair is a consequence of the incapacity of the hair follicle to emerge through the skin surface. Ingrown hairs Ingrown hair was first described by Dubreuilh47 and named ‘‘pseudofolliculitis of the beard’’ by Kligman and Strauss in 1956.48 Other names for ingrown hairs include ‘‘pseudofolliculitis barbae,’’ ‘‘pili incarnati,’’ ‘‘folliculitis traumatic barbae,’’
Fig 6. A, Clinical photograph of eruptive vellus hair cyst on anterior abdominal wall. B, Dermoscopy shows well-defined, circular whitish structure.
‘‘sycosis barbae,’’ and ‘‘razor bumps.’’49 It more often affects the beard and the nape of African Americans and Hispanic men, but also occurs in
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Fig 8. Rolled hair. Dermoscopy shows hairs rolled in spiral with peripilar casts.
Fig 7. Circle hair. A, Circle hairs interspersed among normal hairs. B, Dermoscopy shows dark concentric hair forming a perfect circle under the horny layer.
women and can affect any hair-bearing area where traumatic hair removal occurs such as axilla, legs, pubic area, and nape.50,51 Ingrown hair is usually precipitated by shaving or plucking. Two types of hair ingrowing can be distinguished: (1) transfollicular penetration, caused by a thick curled hair with a sharp edge that transfixes the follicle under the skin producing a foreign-body reaction; and (2) extrafollicular penetration when the sharpened hair re-enters the skin 1 to 2 mm away from its emergence.52 Although shaving habits have an important role in precipitating the disorder, a recent study demonstrated that the Ala12Thr polymorphism of keratin K6hf may be responsible for the phenotypic expression of ingrown hair in susceptible individuals.53 The clinical presentation of ingrown hair includes papulopustular lesions located mainly in the beard, neck, and submental region, which is the primary site of involvement in men, whereas in women, they
are commonly seen on the chin and inguinal area. Lesions may be normochromic, erythematous, or hyperpigmented and can be accompanied by itch, pain, or secondary infection. They can produce hypertrophic scarring and keloid formation.54,55 Dermoscopy is not widely used for the diagnosis of ingrown hair, but may serve as an adjuvant in monitoring patient compliance to treatment, and is very useful to educate the patient who can see and understand the cause of the problem. In lesions of the chin and inguinal area dermoscopy shows U-shaped ingrowing hairs beneath a papular lesion representing transfollicular penetration (Fig 9, A).56 Histopathologically, a reaction of foreign body, giant cells, and fibrosis can be observed.57 Extrafollicular penetration is commonly seen in lesions affecting the beard or the neck of patients of African descent (Fig 9, B). The differential diagnosis includes acne vulgaris, bacterial folliculitis, traumatic folliculitis, tinea barbae, and sarcoidal papules.58 Conclusion Dermoscopy is a fast and noninvasive new tool in the diagnosis of body hair disorders. It enables dermatologists to identify morphologic structures of hair not visible by the naked eye. It not only assists in diagnosis, but it also gives information about severity and activity of disease. Furthermore, it can be used to observe disease progression and for
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Fig 9. U-shaped ingrown hairs. A, Dermoscopy shows transfollicular penetration with pustular lesions. B, Ingrown hairs in the neck area show extrafollicular penetration.
assessment at follow-up during and after treatment. For managing hair and scalp disorders, dermoscopy must be emphasized as a standard investigation for use in clinical practice. REFERENCES 1. Garn SM. Types and distribution of the hair in man. Ann N Y Acad Sci. 1951;53:498-507. 2. Laatsch CN, Durbin-Johnson BP, Rocke DM, et al. Human hair shaft proteomic profiling: individual differences, site specificity and cuticle analysis. Peer J. 2014;2:e506. 3. Vogt A, McElwee KJ, Blume-Peytavi U. Biology of the hair follicle. In: Blume-Peytavi U, Tosti A, Whiting DA, Trueb RM, eds. Hair growth and disorders. Berlin, Germany: SpringerVerlag; 2008. 4. Blume U, Ferracin J, Verschoore M, Czernielewski JM, Schaefer H. Physiology of the vellus hair follicle: hair growth and sebum excretion. Br J Dermatol. 1991;124:21-28. 5. Beek CH. A study on extension and distribution of the human body-hair. Dermatologica. 1950;101:317-331. 6. Poskitt L, Wilkinson JD. Natural history of keratosis pilaris. Br J Dermatol. 1994;130:711-713. 7. Mevorah B, Marazzi A, Frenk E. The prevalence of accentuated palmoplantar markings and keratosis pilaris in atopic dermatitis, autosomal dominant ichthyosis and control dermatological patients. Br J Dermatol. 1985;112: 679-685. 8. Schmitt JV, de Lima BZ, de Souza MC, Miot HA. Keratosis pilaris and prevalence of acne vulgaris: a cross-sectional study. An Bras Dermatol. 2014;89:91-95. 9. Yosipovitch G, Mevorah B, Mashiach J, Chan YH, David M. High body mass index, dry scaly leg skin and atopic conditions are highly associated with keratosis pilaris. Dermatology. 2000;201:34-36. 10. Castela E, Chiaverini C, Boralevi F, Hugues R, Lacour JP. Papular, profuse, and precocious keratosis pilaris. Pediatr Dermatol. 2012;29:285-288. 11. Hwang S, Schwartz RA. Keratosis pilaris: a common follicular hyperkeratosis. Cutis. 2008;82:177-180. 12. Sallakachart P, Nakjang Y. Keratosis pilaris: a clinico-histopathologic study. J Med Assoc Thai. 1987;70: 386-389. 13. Lalit G, Anubhav G, Kumar KA, Asit M. Familial erythromelanosis follicularis faciei et colli with extensive keratosis pilaris. Int J Dermatol. 2011;50:1400-1401.
14. Otberg N, Shapiro J. Tufted folliculitis. In: Bolognia J, Jorizzo J, Schaffer JV, eds. Dermatology. Philadelphia: Elsevier Saunders; 2012:1109. 15. Silverberg NB. A pilot trial of dermoscopy as a rapid assessment tool in pediatric dermatoses. Cutis. 2011;87:148-154. 16. Deshmukh SD, Anand M, Yadav GE, Joshi AR. Trichostasis spinulosa presenting as itchy papules in a young lady. Int J Trichology. 2011;3:44-45. 17. Chung TA, Lee JB, Jang HS, Kwon KS, Oh CK. A clinical, microbiological, and histopathologic study of trichostasis spinulosa. J Dermatol. 1998;25:697-702. 18. Strobos MA, Jonkman MF. Trichostasis spinulosa: itchy follicular papules in young adults. Int J Dermatol. 2002;41: 643-646. 19. Sidwell RU, Francis N, Bunker CB. Diffuse trichostasis spinulosa in chronic renal failure. Clin Exp Dermatol. 2006; 31:86-88. 20. Gunduz O, Aytekin A. Trichostasis spinulosa confirmed by standard skin surface biopsy. Int J Trichology. 2012;4:273-274. 21. Pinkus H. Multiple hairs (Flemming-Giovannini; report of two cases of pili multigemini and discussion of some other anomalies of the pilary complex. J Invest Dermatol. 1951; 17(5):291-301. 22. Lester L, Venditti C. The prevalence of pili multigemini. Br J Dermatol. 2007;156:1362-1363. 23. Mehregan AH, Thompson WS. Pili multigemini: report of a case in association with cleidocranial dysostosis. Br J Dermatol. 1979;100:315-322. 24. Naysmith L, De Berker D, Munro CS. Multigeminate beard hairs and folliculitis. Br J Dermatol. 2001;144:427-428. 25. Schoenlaub P, Hacquin P, Roguedas A, Leroy J, Plantin P. Pili multigemini: a pilar dysplasia with linear disposition [in French]. Ann Dermatol Venereol. 2000;127:205-207. 26. Cambiaghi S, Barbareschi M, Cambiaghi G, Caputo R. Scanning electron microscopy in the diagnosis of pili multigemini. Acta Derm Venereol. 1995;75:170-171. 27. Sperling LC, Milde P, Landis LV, Sargent L. Pili multigemini/trichofolliculoma-like organoid nevus. J Am Acad Dermatol. 2014;71:e83-e85. 28. Esterly NB, Fretzin DF, Pinkus H. Eruptive vellus hair cysts. Arch Dermatol. 1977;113:500-503. 29. Piepkorn MW, Clark L, Lombardi DL. A kindred with congenital vellus hair cysts. J Am Acad Dermatol. 1981;5: 661-665. 30. Lee S, Kim JG, Kang JS. Eruptive vellus hair cysts. Arch Dermatol. 1984;120:1191-1195.
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31. Oiso N, Matsuda H, Kawada A. Eruptive vellus hair cysts of the labia majora: detection of openings of the cysts to the epidermis by dermoscopy. Eur J Dermatol. 2013;23: 417-418. 32. Alfaro-Castellon P, Mejia-Rodriguez SA, Valencia-Herrera A, Ramirez S, Mena-Cedillos C. Dermoscopy distinction of eruptive vellus hair cysts with molluscum contagiosum and acne lesions. Pediatr Dermatol. 2012;29:772-773. 33. Torchia D, Vega J, Schachner LA. Eruptive vellus hair cysts: a systematic review. Am J Clin Dermatol. 2012;13:19-28. 34. Kiene P, Hauschild A, Christophers E. Eruptive vellus hair cysts and steatocystoma multiplex: variants of one entity? Br J Dermatol 1996;134:365-367. 35. Lazarov A, Amichai B, Cagnano M, Halevy S. Coexistence of trichostasis spinulosa and eruptive vellus hair cysts. Int J Dermatol. 1994;33:858-859. 36. Adatto R. Spiral hair (coiled hair) [in French]. Dermatologica. 1963;127:145-147. 37. Levit F, Scott MJ Jr. Circle hairs. J Am Acad Dermatol. 1983;8: 423-425. 38. Smith JB, Hogan DJ. Circle hairs are not rolled hairs. J Am Acad Dermatol. 1996;35:634-635. 39. Contreras-Ruiz J, Duran-McKinster C, Tamayo-Sanchez L, Orozco-Covarrubias L, Ruiz-Maldonado R. Circle hairs: a clinical curiosity. J Eur Acad Dermatol Venereol. 2000;14: 495-497. 40. Lacarrubba F, Misciali C, Gibilisco R, Micali G. Circle hairs: clinical, trichoscopic and histopathologic findings. Int J Trichology. 2013;5:211-213. 41. Fergusson AG, Derblay PR. Rolled hairs: a possible complication of corticosteroid therapy. Arch Dermatol. 1963;87:311-314. 42. Seckin D, Gulec TO, Haberal M. Higher incidence of rolled hairs in renal transplant recipients: a possible complication of corticosteroid and cyclosporine therapy. Acta Derm Venereol. 1998;78:384. 43. Ortonne JP, Juhlin L, el Baze P, Pautrat G. Familial rolled and spiral hairs with palmoplantar keratoderma. Acta Derm Venereol. 1985;65:250-254. 44. Itin PH, Bircher AJ, Lautenschlager S, Zuberbuhler E, Guggenheim R. A new clinical disorder of twisted and rolled
45. 46.
47.
48. 49. 50. 51.
52. 53.
54.
55.
56.
57.
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body hairs with multiple, large knots. J Am Acad Dermatol. 1994;30:31-35. Resnik KS. Twisted and rolled body hairs. J Am Acad Dermatol. 1994;31:1076. Kanerva L, Niemi KM, Lassus A, Rantanen T. Scanning electron microscopic visualization of rolled hair (Poils en spirale). Acta Derm Venereol. 1983;63:61-64. Dubreuilh MW. Eruption papuleuse de la barbe causee par le recourbement des poils. Bull Soc Franc Dermat et Syph. 1922; 29:80-81. Kligman AM, Strauss JS. Pseudofolliculitis of the beard. AMA Arch Dermatol. 1956;74:533-542. Ribera M, Fernandez-Chico N, Casals M. Pseudofolliculitis barbae [in Spanish]. Actas Dermosifiliogr. 2010;101:749-757. Crutchfield CE III. The causes and treatment of pseudofolliculitis barbae. Cutis. 1998;61:351-356. Alexander AM, Delph WI. Pseudofolliculitis barbae in the military: a medical, administrative and social problem. J Natl Med Assoc. 1974;66:459-464, 479. Kelly AP. Pseudofolliculitis barbae and acne keloidalis nuchae. Dermatol Clin. 2003;21:645-653. McLean WH. Close shave for a keratin disorder-K6hf polymorphism linked to pseudofolliculitis barbae. J Invest Dermatol. 2004;122:xi-xiii. Kundu RV, Patterson S. Dermatologic conditions in skin of color, part II: disorders occurring predominately in skin of color. Am Fam Physician. 2013;87:859-865. Edlich RF, Haines PC, Nichter LS, Silloway KA, Morgan RF. Pseudofolliculitis barbae with keloids. J Emerg Med. 1986;4: 283-286. Chuh A, Zawar V. Epiluminescence dermatoscopy enhanced patient compliance and achieved treatment success in pseudofolliculitis barbae. Australas J Dermatol. 2006;47:60-62. Rodney IJ, Onwudiwe OC, Callender VD, Halder RM. Hair and scalp disorders in ethnic populations. J Drugs Dermatol. 2013; 12:420-427. Perry PK, Cook-Bolden FE, Rahman Z, Jones E, Taylor SC. Defining pseudofolliculitis barbae in 2001: a review of the literature and current trends. J Am Acad Dermatol. 2002; 46(Suppl):S113-S119.
Clinical, dermoscopic, and histopathologic features of body hair disorders Ratchathorn Panchaprateep, MD, PhD,a Aline Tanus, MD,b and Antonella Tosti, MDc Bangkok, Thailand; Rio de Janeiro, Brazil; and Miami, Florida Dermoscopic examination of hair and scalp, also named ‘‘trichoscopy,’’ is an essential tool in diagnosis of hair and scalp diseases. Trichoscopy is fast and noninvasive and can be used to evaluate hair disorders in all body areas. Body hair disorders are uncommon, and most publications on their dermoscopic features are limited to case reports or series. In this review we present the available information on the dermoscopic diagnosis of body hair disorders including keratosis pilaris, trichostasis spinulosa, pili multigemini, circle hairs, rolled hairs, eruptive vellus hair cyst, and ingrown hairs. ( J Am Acad Dermatol http://dx.doi.org/ 10.1016/j.jaad.2015.01.024.) Key words: circle hairs; dermoscopy; eruptive vellus hair cysts; ingrown hairs; keratosis pilaris; pili multigemini; pseudofolliculitis; rolled hairs; trichoscopy; trichostasis spinulosa.
T
he use of dermoscopy dramatically changed the diagnostic approach to hair and scalp disorders as it is fast, noninvasive, and easy to use. It has become an essential tool for all dermatologists and other physicians in evaluation of patients with hair problems. Dermoscopy is not only useful for diagnosis of scalp hair disorders, but also applicable to body hair disorders including conditions such as keratosis pilaris, trichostasis spinulosa, pili multigemini, circle hairs, rolled hairs, and eruptive vellus hair cyst (EVHC). Dermoscopy also allows physicians to scan the abnormalities of entire body hairs quickly. Furthermore, dermoscopy reduces the necessity of performing painful diagnostic procedures such as hair plucking and skin biopsy. The aim of this article is to review the role of dermoscopy in the diagnosis of body hair disorders and review clinical features and diagnosis of body hair disorders.
METHODS We identified relevant articles by the systematic search of scientific and medical electronic search engines (PubMed, MEDLINE and Scopus) in September 2014. Key terms for searches included: From the Division of Dermatology, Department of Medicine, King Chulalongkorn Memorial Hospital, Bangkoka; Instituto de Dermatologia Professor Rubem David Azulay, Rio de Janeirob; and Department of Dermatology and Cutaneous Surgery, University of Miami L. Miller School of Medicine.c Funding sources: None. Conflicts of interest: None declared. Accepted for publication January 14, 2015. Reprint requests: Ratchathorn Panchaprateep, MD, PhD, Division of Dermatology, Department of Medicine, Faculty of Medicine,
‘‘body hairs,’’ ‘‘body hair disorders,’’ ‘‘dermoscopy,’’ ‘‘dermatoscopy’’ and ‘‘trichoscopy’’ combined with ‘‘keratosis pilaris,’’ ‘‘trichostasis spinulosa,’’ ‘‘pili multigemini,’’ ‘‘pili bifurcati,’’ ‘‘circle hairs,’’ ‘‘rolled hairs,’’ ‘‘eruptive vellus hair cyst,’’ ‘‘ingrown hairs,’’ and ‘‘pseudofolliculitis barbae.’’ Relevant titles, abstracts, and full articles identified from the search were reviewed and included if pertinent to clinical, dermoscopic, and histopathologic features of body hair disorders. Additional articles were also retrieved from the cited reference list of articles identified from our initial systematic online literature search. We limited our search to articles available in the English language. Biology of body hair Human hairs can be classified into 7 main types based on their physical and functional properties: scalp, eyebrow and eyelash, beard and moustache, body hair, pubic hair, axillary hair, and arm and leg1 (Table I). Physically, hair shafts from different locations on the body reveal distinguishable length, structure, and cuticle protein profiles that are different from scalp hairs.2 Body hair or nonscalp Chulalongkorn University, King Chulalongkorn Memorial Hospital, 1873, Rama IV Road, Pathumwan, Bangkok, Thailand 10330. E-mail: [email protected]. Published online March 5, 2015. 0190-9622/$36.00 Ó 2015 by the American Academy of Dermatology, Inc. http://dx.doi.org/10.1016/j.jaad.2015.01.024
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hair shows marked variation in density, distribution, approximately 1 mm in size, resembling gooseflesh and patterning. Hair cycle of body hair follicles is with varying degrees of perifollicular erythema/ also dissimilar to scalp. The body hair follicles have inflammation. Keratosis pilaris lesions often contain prolonged and increased telogen frequency.3 Most a fine-coiled, brittle hair.11 Most lesions are usually 4 body hairs begin as vellus hairs. Increasing asymptomatic but may be pruritic. The common affected body sites are the extensor surfaces of the androgen levels during puberty cause them to transupper aspect of arms, thighs, face, buttocks, and form into terminal hairs over many areas of the body. eyebrows.6,11 To our knowlThere is a sexual differentiation in the amount and disedge, there is just 1 publicaCAPSULE SUMMARY tribution of androgenic hair, tion about dermoscopy of with men tending to have keratosis pilaris reporting Body hair disorders are uncommon and more terminal hairs in more dermal vascular ectasia and can be congenital or acquired. areas including the face, follicular hyperkeratosis.12 In Dermoscopy is useful to confirm chest, abdomen, legs, arms, our experience, in mild diagnosis. and feet.5 cases, dermoscopy shows Clinical presentation and dermoscopic vellus hairs, frequently findings of common body hair disorders twisted or coiled, surBody hair disorders are reviewed including keratosis pilaris, rounded by peripilar casts. Body hair disorders are a trichostasis spinulosa, pili multigemini, Groups of 2 or 3 hairs may group of congenital or accircle hairs, rolled hairs, and eruptive emerge together. In more sequired alterations. Congenital vellus hair cyst. vere cases, vellus hairs are disorders can be an isolated coiled and embedded in the finding or a part of complex Dermatologists will be able to use horny layer. Perifollicular ersyndromes, for example, dermoscopyea fast, noninvasive, and ythema and hyperpigmentakeratosis pilaris or follicular cost-efficient methodeto diagnose body tion is frequently seen keratosis. Acquired body hair disorders. The painful diagnostic (Fig 1). These dermoscopic hair disorders can be caused procedures such as hair plucking and findings are correlated with by trauma. The diagnostic skin biopsy are necessary only in classic histopathologic finddermoscopic findings for uncharacteristic cases. ings described as distention most common body hair disof the follicular orifice by a orders are summarized in keratinous plug that may contain 1 or more twisted Table II. Body hair disorders can be categorized hairs. Although diagnosis of keratosis pilaris rubra into 2 main groups: follicular disorders with 1 or and erythromelanosis follicularis faciei et colli is more vellus hairs emerging from the same follicular mainly clinical, dermoscopy can be helpful in opening (Table III) and circle, rolled, or twisted hairs showing perifollicular erythema and vascular ectasia and ingrown hairs. (Fig 2).13,14 Dermoscopy is also helpful in differentiating keratosis pilaris rubra from follicular lichen Keratosis pilaris planus that shows similar clinical features of follicKeratosis pilaris is a common autosomal domiular spinous papules on the body. In follicular lichen nant disorder of follicular hyperkeratosis characplanus, dermoscopy shows follicular keratotic plugs terized by keratinous plugs in the follicular without broken or twisted hairs (Fig 3). orifices and varying degrees of perifollicular eryDermoscopy is useful not only for diagnosis of thema. In general, keratosis pilaris is cosmetically keratosis pilaris, but also for monitoring unpleasant but medically harmless. Studies noted treatment outcome. A recent study demonstrated a prevalence of 16% to 44% in dermatologic that dermoscopy is a promising tool to assess patients.6,7 The survey showed 61% were female localized improvement in skin architecture after and 39% were male.6,8,9 Age of onset of keratosis receiving treatment in pediatric patients with pilaris was within the first decade in 51%, second atopic dermatitis, ichthyosis vulgaris, and keradecade in 35%, and third and fourth decades in tosis pilaris.15 14%.6 The pathogenesis of keratosis pilaris is still not totally understood. The most accepted theory proposes defective keratinization of the follicular Trichostasis spinulosa epithelium resulting in a keratotic infundibular Trichostasis spinulosa is a relatively common plug.10 but underdiagnosed follicular disorder characterized by hyperkeratosis of a dilated hair follicle Clinical features of keratosis pilaris are multiple with retention of multiple telogen vellus hairs. The small grayewhite, folliculocentric keratotic papules, d
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Table I. Characteristics of scalp and body hairs in each location Hair type
Length, range, mm
Scalp Eyebrow and eyelash
100-1000 5-10
Beard and moustache
50-300
Body (chest, trunk, back)
3-60
Pubic
10-60
Axillary
10-50
Arm and leg
5-20
Description
Medullated with tapered tip in uncut hair Large medullated and coarse, curved with punctuate tip Complex medullary processes, more irregular in structure, blunt tip Irregular medullated, fine long tip, irregular in structure Coarse, kinked, irregular and asymmetric cross-section, many constrictions and twists Coarse, less kinked than public hair, blunt tip, often abraded as a result of friction Irregular medullated, blunt tip
Table II. Diagnostic dermoscopic findings in most common body hair disorders Body hair disorders
Keratosis pilaris
Trichostasis spinulosa Pili multigemini
Eruptive vellus hair cysts Circle hairs
Rolled hairs Ingrowing hairs
Diagnostic dermoscopic findings
- 1-3 Vellus hairs emerging from a single opening - Coiled hairs often embedded in the horny layer - Peripilar casts - Perifollicular erythema - Tuft of short, vellus hairs emerging from the same follicular openings - Keratotic plugs within dilated follicle - Bundles of multiple pigmented hairs of similar thickness emerging from the same follicular openings - Each tuft is surrounded by a peripilar cast - Perifollicular erythema - Circular structure, with central yellowish to whitish papule - Erythematous brownish halo - Dark hairs regularly coiled to form a circle under a translucent layer of stratum corneum - No signs of perifollicular inflammation - Hairs irregularly twisted in spirals - Peripilar casts - U-shaped ingrowing hairs beneath a papular lesion (transfollicular penetration)
origin of trichostasis spinulosa is still unknown. Congenital dysplasia of the hair follicles and external factors such as dust, oils, heat, ultraviolet light, and industrial irritants have been proposed.16 One study reported the presence of Pityrosporum (Malassezia yeast) (82.6%) and
Duration of anagen phase
A:T ratio
2-6 y 4-8 wk
90:10 10:90
4-14 wk
60:40
11-12 wk
30:70
16 wk
30:70
16 wk
30:70
6-12 wk (arm) 19-26 wk (leg)
20:80
bacteria especially Propionibacterium acnes (73.3%) in the extracted follicular material and biopsied specimen of trichostasis spinulosa, suggesting that these micro-organisms may be some of the possible etiologic factors.17 Clinically, blackhead comedo-like lesions or horny, spinous plugs are seen primarily on the tip of nose, alae nasi, and cheeks. The lesions themselves are usually asymptomatic, but a mild degree of folliculitis can be found. Trichostasis spinulosa can also manifest as itchy papules on the trunk and upper extremities in young adults.16,18 Diffuse trichostasis spinulosa has been reported in chronic renal failure.19 Histologically, dilated hair follicle infundibulum with numerous pigmented vellus hairs (up to 50 hairs) and keratinous material are observed. On histopathology, hair shaft entrapment in trichostasis spinulosa results from hyperkeratosis in the follicular infundibulum.14 Many methods have been described to assist in diagnosis such as microscopic examination and standard skin surface biopsy of the contents of black papules, however dermoscopy is the most helpful as it is fast and easy.20 Dermoscopic findings include tufts of multiple, slightly pigmented to pigmented, vellus hairs with varying diameters projecting or emerging from dilated follicular openings. Other follicular openings are occluded by keratotic plugs (Fig 4). Pili multigemini Pili multigemini is an uncommon developmental defect of the hair follicles in which more than 1 hair shaft emerges from the same hair follicle. Each hair has its own papilla, matrix, and inner root sheath but
Keratosis pilaris
Trichostasis spinulosa
Eruptive vellus hair cysts
Occlusion and cyst dilatation of vellus hair follicle
Follicular disorder that results from retention of multiple vellus hairs within sebaceous follicles Middle aged or elderly Face, especially the nose; chest and back (interscapular area) Comedone-like or horny, spinous plugs, sometimes dilated orifice
Multiple small red or brown papules
Microscopy of plucked hairs
Usually a single coiled vellus hair surrounded by keratin
Histopathologic findings
Dilated vellus hair follicle containing keratin and a coiled vellus hair; mild perifollicular mononuclear cell infiltration may be present
Material from eruptive vellus hair cysts is difficult to obtain as lesions locate in dermis Epithelial-lined cyst with several vellus hairs in a keratinaceous matrix
Clinical differential diagnosis
Trichostasis spinulosa, phrynoderma from vitamin A deficiency, follicular keratosis in several nutritional deficiencies, lichen nitidus, lichen spinulosus, folliculitis, pityriasis rubra pilaris, scurvy
Several vellus hairs organized in a bundle within a keratotic material Acanthotic epidermis surrounding a dilated vellus hair follicle containing several vellus hairs arranged in an organized bundle embedded in a keratin plug; mononuclear cell infiltration may be present around follicles and arrector pili muscle Comedogenic acne, keratosis pilaris, eruptive vellus hair cysts, Favre-Racouchot syndrome
Age Common location
Clinical presentation
Childhood, second decade of life Chest and abdominal wall
Follicular nevi (congenital vellus hamartoma)
Pili multigemini
Composite papilla forms several hair shafts separated from each other by IRS but enclosed in a common ORS Childhood to adolescence Face, especially along the lines of the jaw Remarkably thicker and fuller hairs in the affected area or report of recurrent inflammatory papules, leaving scars Multiple hair shafts from composite dermal papilla enclosed in a common ORS Multiple separated hair shafts enclosed in a common ORS but separated from each other by layers of IRS
Tufted hairs; composite hairs/ compound follicles
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Follicular keratotic disorders characterized by a prominent plug of keratin within the follicular orifice Childhood to adolescence Extensor surfaces of the upper aspect of arms, thighs, and buttocks Multiple keratotic follicular papules, often with a rim of erythema; easily removed
Definition
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Table III. Common disorder of follicular disorders with multiple hairs projecting from hair follicles
Benign and cancerous lesions such as melanocytic nevi, seborrheic keratoses, syringomas, epidermoid cysts, eruptive vellus hair cysts, nodular basal cell carcinomas
Overlapping with steatocystoma multiplex, chronic renal failure, pachyonychia congenita
Trichofollicular-like organoid nevus
Keratolytic agents, cyanoacrylic adhesive, hair removal lasers; no resolutionpermanent
Topical retinoid, curettage, laser (carbon dioxide, erbium:yttrium-aluminiumgarnet)
Q-switched ruby laser (case report)
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Treatment
Atopic dermatitis, ichthyosis vulgaris, acne vulgaris, diabetic type 1, Graham-Little syndrome, keratosis follicularis, spinulosa decalvans, erythromelanosis follicularis faciei et colli, hypotrichosis with keratosis pilaris, monilethrix, pachyonychia congenital, ectodermal dysplasia, taking systemic steroid or lithium therapy, Down syndrome, Noonan and cardiofacial-cutaneous syndromes, renal insufficiency, Fairbanks syndrome, Olmsted syndrome Improve with increasing age; no absolute cure; emollient, keratolytic agents, topical retinoids, topical steroids, laser therapy
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Associated conditions
IRS, Inner root sheath; ORS, outer root sheath.
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Fig 1. Keratosis pilaris. A, Clinical photograph of keratosis pilaris on upper aspect of arms. B, Dermoscopy shows irregularly coiled vellus hairs embedded into the horny layer. Perifollicular erythema is also observed. C, High magnification of a coiled hair embedded in the horny layer. D, In milder cases, twisted velllus hair with thin peripilar casts.
Fig 2. Keratosis pilaris rubra. Dermoscopy shows irregularly coiled vellus hairs embedded into the horny layer with perifollicular erythema and underlying vascular ectasia.
shares a common outer root sheath.21 Most cases of pili multigemini have been found incidentally. A recent survey shows only 2% of patients presenting with other dermatologic symptoms had
the condition.22 Pili multigemini is observed in association with folliculitis and rare malformations such as cleidocranial dysostosis.23,24 One reported case revealed pili multigemini in linear distribution following Blaschko lines.25 Clinically, this condition should be suspected in male patients showing remarkably thick hair. Patients may report recurrent inflammatory papules, leaving atrophic/hypertrophic scars. Multigeminate follicles occur mainly on the face especially along jaw lines, however, they can be found in all body regions.26 To our knowledge, there are no published articles on dermoscopy of pili multigemini. In our recent case, dermoscopy showed bundles of multiple pigmented hairs of similar thickness emerging from each hair follicle (Fig 5). Each tuft of hairs was surrounded by a peripilar cast. Histologic examination of pili multigemini reveals complicated follicular structures forming from 2 to as many as 10 hair shafts. Each hair is formed by a single
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that are representative of common overlapping features of steatocystoma multiplex and EVHC.14,35
Fig 3. Follicular lichen planus. Dermoscopy shows follicular keratotic plugs without broken or twisted hairs.
branch of dermal papilla that is surrounded by all layers present in a normal follicle except for the outer root sheath cells. The outer root sheath surrounds the entire follicle. Irregularities in configuration of the hairs, longitudinal grooving, and areas of bifurcation and re-adhesion of the hair shafts can be demonstrated.27 Eruptive vellus hair cyst EVHC was first described by Esterly et al28 in 1977. EVHC presents as multiple asymptomatic papules, with a smooth or centrally umbilicated surface.29,30 The lesions usually affect children and young adults. They are located mostly on the chest and abdominal wall.30,31 Most often EVHC is sporadic; however, some cases with autosomal dominant inheritance have been reported.32 Coexistence of EVHC and steatocystoma multiplex in the same area is quite common. They are believed to be a result of the same disease process originating in pilosebaceous duct.33,34 Dermoscopic findings of EVHC include welldefined, whitish to yellowish, circular structures with erythematous brownish halos. A central gray and blue color point can be observed because of the presence of melanin within the pigmented hair shaft in the cyst (Fig 6).32 A recent study described dermoscopy of EVHC of the labia, demonstrating the follicular openings of the cysts in the epidermis.31 Histopathologic findings of EVHC reveal small epidermoid cyst containing multiple vellus hairs
Circle hair Circle hair, also called spiral hair, is characterized by the presence of dark hairs with perfect circle arrangement located under a thin layer of stratum corneum, without any signs of perifollicular inflammation.36-39 Circle hair occurs in overweight, middle-aged men with abundant body hairs. Most cases of circle hair are found only incidentally, on the abdomen, back, trunk, thighs, and upper aspect of the legs, where they are surrounded by neighboring normal hairs.37 There is no consensus on the pathogenesis of the circle hair. Some authors believe that circle hair has a very thin diameter that is incapable of perforating the stratum corneum and thus remains, growing in a spiral manner, in subcorneum. However, other authors think that the follicular ostium is always open and hair does not need to penetrate the corneal layer.38 Currently, circle hair is considered to be a genetically determined disorder corresponding to vestigial (or remnants) of the mammal undercoat.39 Dermoscopy demonstrates a hair shaft with a slightly smaller diameter rolled in perfect or nearly perfect concentric circles40 (Fig 7). The circle hair is easily extracted after superficial scraping revealing a hair with a question mark shape that can reach 2 to 2.5 cm. When released, the hair returns back to its original format with partially or fully recoiled shape.38,39 Under microscopy, circle hair shows dystrophic bulb in anagen phase, absence of inner root sheath, and narrowing of distal hair shaft forming circular track. Histopathology of circle hair includes presence of ‘‘river bed’’ dilated follicle infundibulum with entrapped hair shafts. No evidence of perifollicular inflammation, follicular obstruction, or reduction of hair shaft diameter was seen.40 Rolled hair The incidence of rolled hair is more frequent than circle hair. Rolled hair has been associated with many conditions including corticosteroid and cyclosporin therapy, ichthyosis, keratosis pilaris, xerosis, neurodermatitis, and palmoplantar keratoderma.41-44 However, some authors believe that rolled hair is caused by mechanical trauma as a result of repeated and vigorous rubbing.45 Rolled hair most commonly affects the extensor extremities, but also the back, shoulders, and buttocks.40 On dermoscopy, rolled hair shows irregularly twisted hair into a hyperkeratotic papule.46 When the keratin follicular plug is removed, it reveals the rolled hair that is inside the follicle
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Fig 4. Trichostasis spinulosa. A, Clinical photograph of trichostasis spinulosa on the nose. B and C, Dermoscopy shows tufts of short, vellus hairs emerging together and keratotic plugs of some follicular openings.
Fig 5. Pili multigemini. Dermoscopy shows bundles of pigmented hairs emerging from the same hair follicle. These hairs are equal in thickness. Each tuft is surrounded by a peripilar cast. Erythema can be observed around some openings.
(Fig 8).43 Histologic findings are mostly follicular hyperkeratosis, and it is believed that the rolled hair is a consequence of the incapacity of the hair follicle to emerge through the skin surface. Ingrown hairs Ingrown hair was first described by Dubreuilh47 and named ‘‘pseudofolliculitis of the beard’’ by Kligman and Strauss in 1956.48 Other names for ingrown hairs include ‘‘pseudofolliculitis barbae,’’ ‘‘pili incarnati,’’ ‘‘folliculitis traumatic barbae,’’
Fig 6. A, Clinical photograph of eruptive vellus hair cyst on anterior abdominal wall. B, Dermoscopy shows well-defined, circular whitish structure.
‘‘sycosis barbae,’’ and ‘‘razor bumps.’’49 It more often affects the beard and the nape of African Americans and Hispanic men, but also occurs in
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Fig 8. Rolled hair. Dermoscopy shows hairs rolled in spiral with peripilar casts.
Fig 7. Circle hair. A, Circle hairs interspersed among normal hairs. B, Dermoscopy shows dark concentric hair forming a perfect circle under the horny layer.
women and can affect any hair-bearing area where traumatic hair removal occurs such as axilla, legs, pubic area, and nape.50,51 Ingrown hair is usually precipitated by shaving or plucking. Two types of hair ingrowing can be distinguished: (1) transfollicular penetration, caused by a thick curled hair with a sharp edge that transfixes the follicle under the skin producing a foreign-body reaction; and (2) extrafollicular penetration when the sharpened hair re-enters the skin 1 to 2 mm away from its emergence.52 Although shaving habits have an important role in precipitating the disorder, a recent study demonstrated that the Ala12Thr polymorphism of keratin K6hf may be responsible for the phenotypic expression of ingrown hair in susceptible individuals.53 The clinical presentation of ingrown hair includes papulopustular lesions located mainly in the beard, neck, and submental region, which is the primary site of involvement in men, whereas in women, they
are commonly seen on the chin and inguinal area. Lesions may be normochromic, erythematous, or hyperpigmented and can be accompanied by itch, pain, or secondary infection. They can produce hypertrophic scarring and keloid formation.54,55 Dermoscopy is not widely used for the diagnosis of ingrown hair, but may serve as an adjuvant in monitoring patient compliance to treatment, and is very useful to educate the patient who can see and understand the cause of the problem. In lesions of the chin and inguinal area dermoscopy shows U-shaped ingrowing hairs beneath a papular lesion representing transfollicular penetration (Fig 9, A).56 Histopathologically, a reaction of foreign body, giant cells, and fibrosis can be observed.57 Extrafollicular penetration is commonly seen in lesions affecting the beard or the neck of patients of African descent (Fig 9, B). The differential diagnosis includes acne vulgaris, bacterial folliculitis, traumatic folliculitis, tinea barbae, and sarcoidal papules.58 Conclusion Dermoscopy is a fast and noninvasive new tool in the diagnosis of body hair disorders. It enables dermatologists to identify morphologic structures of hair not visible by the naked eye. It not only assists in diagnosis, but it also gives information about severity and activity of disease. Furthermore, it can be used to observe disease progression and for
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Fig 9. U-shaped ingrown hairs. A, Dermoscopy shows transfollicular penetration with pustular lesions. B, Ingrown hairs in the neck area show extrafollicular penetration.
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