Pediatric Demiatology Vol. 8 No. 4 300-305

Nevus Comedonicus: Case Report and Brief Review of the Literature Tania F. Cestari, M.D. M.Sc, Mercedes Rubim, M.D., and Berenice C. Valentini, M.D. Pediatric Dermatology Service. General Hospital of Porto Alegre, Federal University of Rio Grande do Sul, Brazil

Abstract: We report extensive nevus comedonicus in a female patient that involved ha!f of her body ent!re!y, with !nfected cystic lesions as well as typica! scars, lim!ted by the mid!!ne. The lesions worsened at the beginning of puberty. A brief review of the literature highlights the histopathologic, et!opathogenic, and therapeutic aspects.

Nevus comedonicus (NO. a rare disorder, was first described in 1895 by Kofman (1). Gomez et al (2) found few more than 100 cases reported. It appears sporadically, irrespective of sex and race. Familial cases are not often found (3,4). It is generally present at birth, but may start in adolescence (5.6) and, rarely, in adulthood (7). The disorder is usually an isolated manifestation, but has been associated with skeletal defects, abnormalities of the central nervous syslem. absence of the fifth finger, ocular lesions such as congenital cataract, and other cutaneous defects (5.8. 9). The latter include ichthyosis (10), trichoiemmal cysts (11). linear basal cell nevus (12), SturgeWeber syndrome (13), and Klippel-Trenaunay syndrome (8). The disorder may also follow in the wake of other conditions such as herpes zoster, longstanding lichen planus, pyoderma, and smallpox vaccination (14,15). CASE REPORT

A 4-day-oid girl was noted by her mother to have a "crushed" appearance of her right face. At 2 months of age, lesions developed on the right half of her body. She was asymptomatic until 9 years of Address correspondence to Tania F. Cestari, M.D.. Dinarte Ribeiro 200 ap 501, Pono Aiegre 90460 RS Brazil.


age, when abscesses appeared in the cervical, axillary, and inguinal regions. During this period the limb lesions began to subside, with clear improvement during the summer. At around 12 years of age the inflammatory lesions worsened and began to have an acneiform appearance. There were no other physical or cutaneous abnormalities and no family history of this disorder. On examination we found an uncountable number of giant comedones with central comeal plugs, measuring I to 3 mm in diameter, clustered in an arciform distribution (Fig. 1). The lesions covered the entire right face from the chin to the ear (Fig. 2), the cervical region, nape, and trunk, with a large concentration around the breast and nipple (Fig. 3), extending to the pubis. The right arm was totally involved, with sparse lesions on the forearm and hand. Similar lesions were observed on the back in the scapular and lumbar regions, on the buttock, thigh and leg, dorsum of the foot, and toes. Varicelliform spongelike scars were observed, especially on the face (Fig. 4). Cysts and purulent nodules were present on Ihe neck. nape, and scapuJa, and in the axilla and inguinal region, forming plaques in different stages of development (Fig. 5). As the infected lesions subsided, they left deep, irregular scars. We also found punctate, keratotic areas and

Cestari et al: Nevus Comedonicus

Figure 1. Giant comedones clustered in an arciform distribution involving the entire half of the body.

depressed scars on a hypochromic base, distributed in zones corresponding to the areas of regression involving the forearm, hand, finger, and entire leg. The lesions were restricted to the right midbody, sparing the scalp and paJmoplantar regions. Physical examination was essentially normal. The patient had well-developed secondary sex characteristics. Neurologic examination, roentgenograms of the skull and chest, and laboratory studies including a complete blood cell count, blood chemistries, and urinalysis were also normal. A biopsy specimen showed foci of epidermal invagination, with hyperkeratosis and orthokeratosis. and retention of stratum corneum. Little cysts or corneal plugs developed in some areas, as in hair follicles, forming comedones. The epidermis adjoining the invaginaled areas sometimes demonstrated discreet papillomatosis with hyperkeratosis and orthokeratosis. The dermis did not show any relevant changes (Fig. 6).


Figure 2. Lesions on the right face, ear, and cervical region. DISCUSSION

Nevus comedonicus is characterized clinically by groups of enlarged follicuiar openings with corneal plugs resembling comedones. These plugs may be easily removable or adherent, and protrude from the skin surface, giving it a rough texture. They are associated with scars, cysts, and miiia, resulting in an acneiform appearance. The interfollicular skin is often normal. Lesions tend to be grouped or scattered in zones and parallel the course of nonradicular nerves or Voight"s lines. Engber (8) suggested that the distribution follows Blaschko's lines, originating either by mosaicism or by influences on the juxtaepidermal mesenchymal tissue during embryogenesis. Lesions are generally confined to a portion of the integument, usually limited by the midline, as in our patient and the ones described by others (9.16). References to bilateral occurrences are rare (17). The most frequent sites of involvement are the face.,


Pediatric Dennatology Vol. 8 No. 4 December 1991

Figure 3, Comedones and scars on the breast, sparing the midiine.

chest, shoulders, trunk, and abdomen (2,5.6). Palmoplantar lesions are rare, as hair follicles are absent in those areas; the few reported cases are believed to derive from eccrine structures (14.18). Nevus comedonicus can also present in the scalp (5,17). and even on the glans penis (5). Sweitzer and Winer (19) in 1932 classified NC into two groups clinically: one with a predominance of comedones and the other, occurring less frequently, with inflammatory lesions, suppuration, and acneiform characteristics. This form is usually difficult to manage, with patients developing frequent and sometimes severe infections as well as unesthetic scars (20). Nevus comedonicus is not commonly altered by menarche. menopause, or pregnancy, although there have been reports to the contrary, including premenstrual exacerbation of the disease (9). We believe that the worsening of our patient's condition was coincident with puberty, due to the characteristics it assumed and to the severe inflammatory alterations associated with an increase in sweating and scalp seborrhea.

The association between NC and tricholemmal cysts or basal cell nevi suggests that, in some cases. this disease may represent a disorder of growth regulation, affecting other appendageal structures besides the follicular epithelium (4), Several authors regard NC as a hamartoma of the piiosebaceous follicle, originating in a defect of the mesodermal component of the follicle, with imperfect differentiation of the epithelial portion (14). The affected follicular structure is unable to develop terminal hair and sebaceous glands; it only produces soft keratin and, more rarely, vellus hair that obstructs the adnexal orifice. The absence of the arrector pili muscle as well as sebaceous glands supports this hypothesis. It does not imply, however, a pilar origin. Cases are reported in sebaceous nevus, in superficial lipomatous nevus, and related to eccrine sudoriparous ducts (21). Comedones can therefore be created through several mechanisms. Nevus comedonicus would then behave as a homologous hamartoma when it appears in areas where sebaceous follicles are present, and heterologous in areas where these follicles are absent, such as the palms and soles (9).

Cestari et ai: Nevus Comedonicus


Figure 4. Typical depressed scars with a spongiform appearance.

Figure 5. Axillary region with inflammatory lesions and abscesses.

The disorder also can be a marker of systemic disorders in syndromic association, characterizing the nevus comedonicus syndrome. Gomez et a! (2) regarded it as a variant of the epidermal nevus syndrome, especially when associated with defects of the bone and central nervous system. The presence of epidermolytic hyperkeratosis in the keratinocytes of the follicular wall is a histologic finding connected with both entities (2.4.15,22.23). Cabrera et al (24) regarded the epidermal nevus and sebaceous nevus syndromes as pathologically identical. Taking into account that the organic defects in some cases of NC also occur in association with epidermal nevus or linear sebaceous nevus, other abnormalities should be investigated when such patients are diagnosed. Histoiogically. there are ample epidermal invaginations with moderate atrophy, resembling enlarged hair foliicies. Hair shafts and some lobes of

sebaceous glands can be seen in the depth of the invagination. and the arrector pili muscle is absent (4.23). The invaginated wali shows a certain degree of acanthosis, with continuous production of keratin resulting in concentric iaminae. Epidermoiytic hyperkeratosis may be present in the keratinocytes that comprise the foilicuiar epithelial wall. The differential diagnosis of NC includes ulerythemalosis reliculata. sebaceous nevus, acne neonatorum. grouped comedone acne, chioracne, and Darrier disease (17). Treatment of NC is advisable for esthetic reasons and aiso because of recurrent infections. Locaiized lesions may be removed surgically with good results. Several therapeutic approaches have been attempted for extensive lesions—none of them satisfactory—such as manual extraction of the comedones, shaving, dermabrasion, and the use of keratoiytic agents (21). In 1972 Decherd et a! (25)


Pediatric Dermatology Vol. 8 No, 4 December 1991

medications that will control and minimize such disfiguring lesions. In addition, the study of patients with NC may improve our understanding of the pathogenetic mechanisms involving the piiosebaceous unit. REFERENCES 1. Kofman S. Ein Fall von seltener Lokalisation und

Figure 6, Epidermal invagination with corneal plugs and discrete papillomatosis. (Hematoxylin & eosin; magnification 40x.)

used a 0,1% retinoic acid solution on two patients with NC because of the keratolytic effect of this medication and because of the similarity of the disease to acne vulgaris. and obtained good results. Milton et al (20) prescribed a 12% lactic acid lotion neutralized with ammonium hydroxide to a pH of 4,5 to 5.5 (ammonium lactate lotion), mainly in patients with extensive lesions resistant to other treatments. The systemic drugs that have been given are basically antibiotics to control infections (20), Oral isotretinoin has not been effective (9,20). Acceptable results were reported with combined progesterone and estrogen therapy in cases worsened by hormonal influence (9). Our patient was treated with erythromycin and cephalexin for her acute exacerbations. Good longterm control was then maintained with sulfamethoxazole and trimethoprim. Discontinuation of these medications led to recurrence in a few weeks. We drained the cysts and abscesses and used a 12% ammonium lactate solution, as advised by Milton et al (20). After 40 days the response was good, with easy removal of the comedones and smoothing of the keratotic. roughened areas on the limbs. This treatment has been in progress for 18 months with no side effects. The facial lesions will certainly require dermabrasion to minimize scarring. We are also concerned about the possibility of worsening of the nevus on the breast after hormonal stimulation such as pregnancy. Due to the patient's young age we did not prescribe hormones. Nevus comedonicus is a relatively benign disease, in spite of its serious cosmetic implications. Nevertheless, it is important to report and follow these patients to encourage the development of


7. 8. 9. 10


12. 13.



16 17


Verbreitung von Komedon. Arch Dermatol Syph 1895:32:177-178. Gomez ML, Pietropaolo N. Cabrera H, Rodriguez A, Alvarez M. Nevo comedonico: a proposito de seis casos (dos famiiiares). Rev. Argent Dermatol 1987; 68:86-93. Carneiro SJC. Dickson JE. Knox JM. Familial dyskeratotic comedones. Arch Dermatol 1972:105:249253. Dudley K. Barr WG. Armin A. Massa MC. Nevus comedonicus in association with widespread, welldifferentiated follicular tumors. J Am Acad Dermatol 1986:15:1123-1127. Suarez Tl, Prado A, Cordero AA. El nevo comedonico. Med Cutan Iber Lat Am 1985:13:371-376. Rodrigues JCF. Torres VM, Leite LFO. Lopes JMC. Nevus comedonicus. Trab Soc Port Dermatol Venereol 1985:43:245-249. Nabai H. Mehregan AH. Nevus comedonicus. Acta Dermato! Venereol 1973:53:71-74. Engber PB. The nevus comedonicus syndrome: a case report with emphasis on associated internal manifestations. Int J Dermatol 1978:17:745-749. Beck MH, Dave VK. Extensive nevus comedonicus. Arch Dermatol 1980:n6:IO48-105O. Piers F. Linear comedo nevus and ichthyosis. Br J Dermatol 1945:57:138-147. Leppard BJ. Trichilemmal cysts arising in an extensive comedo naevus. BrJ Dermato! 1977:96:545-548. Bleiberg J, Brodkin RH. Linear unilateral basal-cell nevus with comedones. Arch Dermatol l%9;100: 187-190. Rook AJ. Naevus comedonicus uni!ateralis with partia! Sturge-Weber syndrome and extensive vascular naevi with hemangiectatic hypertrophy of !eg. Proceedings of the !Oth International Dermatology Congress—London. London: British Medica! Association. 1953:421-422. Wood MG, Thew MA. Nevus comedonicus—a case with pa!mar involvement and review of the literature. Arch Dermatol !968:98:!!!-! 16. Kim SC. Kang WH. Nevus comedonicus associated with epidermal nevus. J Am Acad Dermatol !989;21: 1085-1088. Rodriguez JM. Nevus comedonicus (letter]. Arch Dermatol 1975:1!1:!363-1364, Paige TN, Mendelson CG. Bilateral nevus comedonicus. Arch Dermatol !967;%:I72-!75. Harper KE. Spielvoge! RL. Nevus comedonicus of the pa!m and wrist. J Am Acad Dermato! !985:12: 185-188.

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19. Sweitzer SE, Winer LH. Naevus unilateralis comedonicus: naevus follicularis of White. Arch Dermatol Syph I932;26:694-706. 20. Milton GP, DiGiovanna JJ, Peck GL. Treatment of nevus comedonicus with ammonium lactate lotion. J Am Acad Dermatol 1989;20:324-328. 21. Blanchard L. Hodge SJ, Owen LG. Linear eccrine nevus with comedones. Arch Dermatol 1981:117: 357-359. 22. Aloi FG, Molinero A. Nevus comedonicus with epi-


dermolytic hyperkeratosis. Dermatologica 1987;174: 140-143. 23. Lever WF. Schaumburg-Lever G. Histopathology of the skin, 7th ed. Philadelphia: JB Lippincott. 1990. 24. Cabrera H, Cosentino R, Savoia J, Costa J. Sindrome del novo epidermico-sebaceo linear. Rev Argent Dermatol 1983;54:214-218. 25. Decherd JW. Mills O, Leyden JJ. Naevus comedonicus—treatment with retinoic acid. Br J Dermatol I972;86:528-529.

Nevus comedonicus: case report and brief review of the literature.

We report extensive nevus comedonicus in a female patient that involved half of her body entirely, with infected cystic lesions as well as typical sca...
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