Arch Dermatol Res DOI 10.1007/s00403-014-1509-z
REVIEW
Papular elastorrhexis: report of four cases and review of literature Di-Qing Luo • Juan-Hua Liu • Ming-Chun Chen Zhuo Wang • Wen-Lin Xie
•
Received: 5 April 2013 / Revised: 8 September 2014 / Accepted: 15 September 2014 Ó Springer-Verlag Berlin Heidelberg 2014
Abstract Papular elastorrhexis is a rare cutaneous disorder; only 27 cases have been reported before the present review. We added four new cases, and reviewed the published literature in both English and Chinese language. To data, 31 cases have been reported, in the range of 4–40 years (n = 31, median 20 years, mean age 19.5 years). Among them, only a familial cluster was reported, the ratio of male/female was 11:20. The age for the first detection of the lesions was from 3 to 35.5 years (n = 26, median age 14 years, mean age 15.3 years). The duration between the initial detection of the lesion to consultation was from 3 weeks to 20 years (n = 27, median time 1 year, mean time 3.1 years). Of the 26 patients with details, 9 detected their initial lesions in the first decade, 11 in the second decade, 5 in the third decade and only 1 older than 30 years. Twenty out of 31 patients showed nonfollicular papules while the rest had been not mentioned the condition. The lesions may distribute over
D.-Q. Luo and J.-H. Liu contributed equally. D.-Q. Luo (&) J.-H. Liu Department of Dermatology, The Eastern Hospital of the First Affiliated Hospital, Sun Yat-sen University, 183 Huangpu Rd. E., Guangzhou 510700, China e-mail:
[email protected];
[email protected] M.-C. Chen (&) Department of Dermatology, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, 107 Yanjiang Xi Lu, Guangzhou 510120, China e-mail:
[email protected] Z. Wang W.-L. Xie Department of Pathology, The Eastern Hospital of the First Affiliated Hospital, Sun Yat-sen University, 183 Huangpu Rd. E., Guangzhou 510700, China
trunk, limbs, and rarely on shoulders, scalp, face, mandibula, retroauricular region, occipitocervical, neck, armpits, thighs. Histopathological examination shows focal loss or decrease of elastic fibers, that may also appear thin and fragmented. There may be mild perivascular lymphocytic infiltrate in the dermis. The collagen bundles can be thickened and homogenized, or normal. No suggestive therapies were proposed at present. Keywords Dermis Elastic fibers Loss Papules Papular elastorrhexis Review
Introduction Connective tissue mainly includes elastic and collagen fibers, respectively. Elastic fibers in the extracellular matrix, for its resilience and elasticity, are an integral part of dermal connective tissue vital for normal cutaneous function and structure [18, 30]. Connective tissue nevi are hamartomas characterized predominantly by an imbalance in relative amount and distribution of various dermal connective tissues, including collagen, elastic fiber, or proteoglycan; the lesions are not monomorphous and have the tendency to be grouped into plaques [7]. Several acquired disorders, which present cutaneously with small yellow-to-white papules and histopathologically with loss of dermal elastic tissue, have been described including papular elastorrhexis (PE) [4, 19], nevus anelasticus (NA) [9, 19], mid-dermal elastolysis [14, 19], anetoderma [19], pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) [2, 19, 21], postinflammatory elastolysis and cutis laxa [19], etc. Owing to their scarcity and similar clinical features, such cutaneous disorders are still poorly understood, and are also rather hard to diagnose clinically,
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and are reported rarely. PE was first described in 1987 by Bordas and colleagues [4] as a possible variant of NA, it was even considered an abortive form of Buschke–Ollendorff syndrome [24] or a distinct variant of connective tissue nevus [8, 25], but now it is generally accepted that PE is a distinct entity [5, 6, 11, 19, 23]. Using keywords ‘‘papular elastorrhexis,’’ we searched medline, pubmed on May 31, 2014 for all articles published in English language or published with English abstract details, and also searched the Chinese database CNKI for Chinese articles using the Chinese keywords. Only the cases diagnosed as PE and fitted the criteria were selected for review. The definitions of PE for the present review we proposed include: clinically, the lesion is acquired, asymptomatic, multiple, discrete, firm, welldemarcated white papules distributed symmetrically, with diameter less than 1 cm, and without extracutaneous involvement; historically, the involved areas have no previous medical problems or antecedent trauma; and histopathologically, the collagen bundles may be thickened and homogenized in focal areas, or normal, without predominant elements of connective tissue, while staining for elastic tissue shows substantial fragmentation, decrease or nearly complete loss in the reticular dermis; and finally, other kinds of diseases mimicking PE or lesions with perifollicular papules were excluded. The cases diagnosed as PE but lacking histopathology examination are also excluded from the present review. We also added four new Chinese cases into the present review.
Case reports
Fig. 1 Discrete, firm white papules on the trunk
Case 2 A 15-year-old girl presented with 5-year history of multiple asymptomatic white papules over her back and extremities. The lesions increased in numbers gradually. The patient denied any medical problems over the involved skin, as well as significant past medical history. No significant family history was reported. Cutaneous examination showed that tens of nonfollicular, discrete, white papules (2–3 mm in diameter) distributed over her back (Fig. 3) and the upper limbs. Biopsy from a papule revealed homogenized and condensed collagen in the upper dermis (Fig. 4a, b), and decrease of elastic fibers with fragmentation and thinning of remaining elastic tissue in the upper dermis (Fig. 4c, d). No positive results of biochemistries were present, and X-ray for the bone was also negative. The patient refused any treatment.
Case 1 Case 3 A 7-year-old boy was referred with more than a year history of asymptomatic white papules over his trunk, which increased gradually in size and numbers. The lesions were also noticed presenting as white macules initially, then becoming papules within months. The patient was absent for treatment and denied any other medical problems over the involved areas. No other family members including his elder brother were similarly affected. Cutaneous examination showed that there were more than 10 of nonfollicular, firm, white, 3–5 mm in diameter maculopapules and papules over his trunk (Fig. 1). Biopsy from a maculopapule which occurred about 3 months ago revealed a localized area of mild homogenized and condensed collagen (Fig. 2a, b), and localized fragmentation and thinning of elastic fibers in the superficial dermis of the lesional area (Fig. 2c, d). No positive results for biochemistries and X-ray were noted. The lesions had poor response to a 2-month treatment of topical 0.1 % tretinoin gel.
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A 36-year-old Chinese male worker was referred with 6 months history of asymptomatic papules which increased gradually in both size and numbers since its first detection. There was no history of bleeding or morphologic change of the lesions during the course. The patient denied no history of trauma, acne or folliculitis, prior excessive sun exposure, or other medical problems over the affected skin, and had no history of thyroiditis, arthritis or ultraviolet (UV) light therapy. No other family members and his close colleagues were similarly affected. No prior medications for the lesions were administered. Cutaneous examination showed numerous mildly indurative, flesh-colored, discrete nonfollicular papules distributed over the shoulders, neck and upper arms, and only a few localized on the back and hips. The lesions were asymmetric, about 3- to 5-mm in diameter, and not
Arch Dermatol Res
Fig. 2 a, b Focal area of mild homogenized and condensed collagen in the upper dermis, the epidermis is normal (hematoxylin–eosin stain). c, d Focal area of fragmented and thinned elastic fibers in the
superficial dermis (Aldehyde-Fuchsin stain) (the length of the white bar is 1 mm for a and c; and 200 lm for b and d, respectively)
Biopsy specimen from one of the papules on the back showed normal epidermis and mild perivascular lymphocytic infiltrate in the superficial dermis, but the collagen bundles were normal. Aldehyde-fuchsin stain revealed a focal loss of elastic tissue, and fragmentation and rarefaction of the residual elastic fibers in the dermis, but no change was present in the deep dermis. Laboratory investigations including complete blood count and serum biochemistry tests were normal. X-ray showed no changes of bone density. The lesions had poor response to a 3-week treatment of topical 0.1 % tretinoin gel, twice daily. Case 4 Fig. 3 Multiple disseminated white papules on the back (the inset is the magnification of the papules)
indurated and tender. On palpation, none of the papules had the aspect of bladderlike soft protuberances as it presents in anetoderma.
A 12-year-old boy was referred because of 1 year history of asymptomatic papules on his trunk, especially on his back aspect of abdomen, which increased gradually in both size and numbers. He denied any other prior cutaneous lesions on the involved areas. Cutaneous examination revealed tens of discrete, mildly indurative, flesh-colored,
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white, 2–5 mm in diameter nonfollicular papules distributed over his back aspect of trunk. Biopsy from a papule displayed normal epidermis and moderate fibrosis of collagen in the dermis. Elastin stain disclosed a localized decrease of elastic fibers, and prominent fragmentation of the residual elastic fibers in the dermis. A radiologic workup yielded negative findings. The patient refused no treatment.
Results Based on the literature, 27 cases who were fitted our PE definitions mentioned above were rolled in the present review. In whom, four were described in Chinese [10, 15, 31, 33], the rest were reported in English language or with English abstract. Adding the present 4 cases, a total of 31 cases have been described (Table 1) [1, 4–8, 10–13, 15, 17, 23, 25, 28, 29, 31, 33]. Of these patients, 11 (35.5 %) were male and 20 (64.5 %) were female, with an age range of 4to 40-year old (n = 31, median age 20 years, mean age 19.5 years). The duration between the initial detection of the lesions to consultation was from 3 weeks to 20 years (n = 27, median time 1 year, mean time 3.1 years). The age for the first detection of the lesions was from 3 to 35.5 years (n = 26, median age 14 years, mean age 15.3 years). Of the 26 patient with details, 9 (34.6 %) detected their initial lesions in the first decade, 11 (42.3 %) in the second decade, 5 (19.2 %) in the third decade and only 1 (3.8 %) older than 30 years. Among the 31 patients, only a familial cluster was reported [24]. There are two cases diagnosed as PE by their authors, because one case had lesional diameter larger than 1 cm [20] and another one refused biopsy [24], both were excluded from the review. Of the 31 patients, only 1 associated with atopic eczema [6], and another 1 had not mentioned if he had any associations. One developed Hodgkin’s lymphoma after the diagnosis of PE [20]. The described lesions presented as acquired, asymptomatic, white or skin-color papules which may mainly distribute in symmetry or asymmetry over trunk (27/31), limbs (14/31); and in rare instance, the lesions may also appear on shoulders (3/31), scalp (1/31), face (1/31), mandibula (1/31), retroauricular region (1/31), occipitocervical (1/31), neck (1/31), armpits (1/31), hips (1/31), thighs (1/31). The involved truncal areas included abdomen, back and chest. Upper limbs (13/14) were more common areas than lower limbs (6/14) to be involved. The reported lesional diameter was from 1- to 8-mm, and most of them were 2–5 mm. The lesions of 20 out of 31 patients were nonfollicle, while the rest had been not mentioned the condition. No lesions had been reported coalescing to form plaques.
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Histology of hematoxylin–eosin staining was available for 28 patients, the epidermis may be normal or hyperkeratotic, there may be perivascular lymphoid infiltrate in the superficial dermis in some patients. Twenty-three out of the 28 patients showed that the collagen may be increased, homogenized or condensed [5–8, 10, 13, 15, 20, 23, 25, 28, 29, 31, 33], and other 5 showed normal collagen fibers [11, 12, 24]. The elastic fibers had been tested in all the 31 patients which showed complete loss, decrease, fragmentation or thinning in focal area. Treatments were described in five patients only: one had poor response to intra-lesional injection of steroids and liquid nitrogen cryotherapy [10], one failed in responding to anti-acne therapies, including isotretinoin, antibiotics and benzoyl peroxide [25], and the present two cases had poor response to topical tretinoin gel; only one improved by intra-lesional injection of triamcinolone, but recurrence occurred after stopping the treatment [17].
Discussion Papular elastorrhexis is a rare disorder of elastic tissue. To our knowledge, 27 cases with biopsy have been reported before our review since its first description [1, 4–8, 10–13, 15, 17, 23, 25, 28, 29, 31, 33]. In the present review, we find that all the patients including the present cases presented only cutaneous involvement without any description of extracutaneous changes or systemic involvement. Almost the patients are asymptomatic, but it may be associated with atopic dermatitis [6] in rare instance. PE has a predilection for adolescent and for females. Twentynine out of 31 cases are sporadic, except for an isolated instance of familial cluster [24]. The lesions were acquired, a few to multiple in numbers, mostly 1- to 5-mm in size, whitish or skin-colored, nonfollicular (at least mostly) and indurated papules, with symmetrical or asymmetrical distribution over the trunk and extremities; and in rare instance, the lesions may involve the mandibula, the occipitocervical, the face, the scalp, the retroauricular region, etc. Histopathologically, the most characteristic change is fragmentation, thin and/or loss of elastic fibers in the dermis, the collagen may present as focally homogenized or condensed, or normal in the dermis. Based on the present data, nonfollicular papules may include the diagnostic criteria except the definitions mentioned above. Pajot et al. [20] reported a case whose lesions ranged from few millimeters to 2 cm in diameter with the oldest lesion being 15 cm wide, and showed histopathological features mimicking PE. Although the patient was diagnosed as PE by the authors, considering the lesional diameter, we are suspicious of their diagnosis and have excluded the case from the present review.
Age/ duration (years)
17/3
21/7
21/3
17/5 wks
20/a few years
4/1
18/4
9/1
12/4 mo
No.
1
2
3
4
5
6
7
8
9
F
F
F
M
M
F
F
F
M
Sex
Trunk
Upper back and abdomen
Trunk
Chest and back
Trunk and extremities
Back and extremities
Chest
Back and shoulders
Abdomen, back and chest
Lesion site
Symptomless
Symptomless
Symptomless
NM
Symptomless
Symptomless
Symptomless
Symptomless
None
Association
Multiple white firm papules
Multiple white papules, 4–5 mm in diameter
Multiple, discrete, 1–3 mm, white papules
Multiple, hard, whitish papules
2- to 3-mm white papules
3-mm, white papules
1–3 mm whitish firm papules
1–3 mm, white, firm papules
Flat, firm, yellowish, isolated, painless, 2-5 mm in diameter papules
Evolution of the lesions
Table 1 Summary of the previously reported cases of papular elastorrhexis
NM
NM
No treatment
Improved by intralesional injections of triamcinolone, but recurred
NM
NM
No treatment
Failed in anti-acne therapies, including isotretinoin, antibiotics and benzoyl peroxide
NM
Therapy
Significant decrease, fragmentation or a virtually complete loss of elastic tissue
Decrease and fragmentation
Decreased elastic fibers and thin and fragmentation for the residual fibers
Focal loss of elastic fibers in the dermis
Decrease and fragmentation in the first third dermis
Decrease and fragmentation in dermis
Decrease and partial fragmentation
Decrease in lesion, thin and fragmentation for residual elastic fibers
Decrease, intense fragmentation in middle and deep dermis
Elastic fibers
Thickening and homogenization of collagen bundles
Focal area of homogenized and condensed collagen in the upper dermis
Focal area of homogenized and condensed collagen in the reticular dermis
NM
Normal
Normal collagen
Focal homogenized, condensed collagen in the mid dermis
Collagen homogenization in reticular dermis
NM
Collagen bundles in dermis
Buechner et al. [5]
Nonfollicle
NM
Choonhakarn et al. [8]
Lee et al. [17]
Schirren et al. [24]
Sear et al. [25]
Bordas et al. [4]
References
Nonfollicle
NM
Nonfollicle
Nonfollicle
Nonfollicle
Nonfollicle
Nonfollicle
Follicles
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Age/ duration (years)
12/6 mo
11/2
11/1
34/4
22/2
32/3 wks
37/NM
22/NM
24/NM
12/9
No.
10
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11
12
13
14
15
16
17
18
19
F
M
F
F
F
M
M
F
M
F
Sex
Table 1 continued
Extremities and trunk
The trunk and proximal limbs
Trunk
Trunk and upper arms
Trunk and upper limbs Mandibula and occipitocervical
Back
Arms, thighs and back
Trunk and legs
Trunk and upper arms
Lesion site
Symptomless
Atopic eczema
Health
Symptomless
Symptomless
Symptomless
Symptomless, but developed Hodgkin’s lymphoma
Symptomless
Symptomless
No associations
Association
Multiple yellowish to skin-colored, nontender papules Multiple white, firm papules
Multiple skincolored papules
White papules
Sparse fleshcolored papules 2–4 mm white papules
Papules
Fifteen tender round 5-mm white papules
Twelve slightly indurated papules
White papules
Evolution of the lesions
No treatment
NM
NM
No treatment
No treatment
No treatment
NM
NM
NM
NM
Therapy
Decrease and fragmentation of elastic fibers in the upper dermis
Localized thin and fragmented elastic fibers in the reticular dermis
Localized thin and fragmented elastic fibers in the reticular dermis.
Decrease and significant fragmentation
Focal decrease and fragmentation Decrease and fragmentation
Almost complete loss
Significant decrease, fragmentation or a virtually complete loss
Decrease and fragmented elastic fiber Significant decrease, fragmentation or a virtually complete loss of elastic tissue
Elastic fibers
Hyperkeratotic epidermis and perivascular lymphoid infiltrate in the superficial dermis, Increase of collagen fibers
Slight fibrosis in the middermis, and homogenized and condensed collagen
Discrete hyperkeratosis, slight fibrosis in the middermis, and homogenized and condensed collagen
Focal area of homogenized collagen in the reticular dermis
Slight increase of collagen
Normal
Thickened collagen bundles
Thickening homogenization collagen bundles
Thickening and homogenization of collagen bundles Thickening and homogenization of collagen bundles
Collagen bundles in dermis
Nonfollicle
NM
NM
Nonfollicle
Nonfollicle
NM
NM
NM
NM
NM
Follicles
Choi et al. [7]
Canueto et al. [6]
Flores et al. [13]
Del Pozo et al. [11] Tan et al. [28]
Pajot et al. [20]
References
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21/NM
40/20
7/0.5
8/2
24/10
22/3 wks
26/1
30/1
7/1
15/5
36/6 mo
12/1
20
21
22
23
24
25
26
27
28
29
30
31
M
M
F
M
M
F
F
F
F
F
F
F
Sex
Trunk
Shoulders, neck, trunk, upper arms and hips.
Trunk and upper limbs
Trunk
Face, scalp and retroauricular region
Lower portion of chest
Trunk
Upper arms
Chest and back
Trunk and limbs
Trunk, armpits, neck and proximal upper limbs
Upper trunk and shoulders
Lesion site
Symptomless
Symptomless
Symptomless
Symptomless
Symptomless
Symptomless
Symptomless
Symptomless
Symptomless
Symptomless
Symptomless
Symptomless
Association
2–5 mm white papules
2–5 mm white papules
2–3 mm white papules
3–5 mm white macule and papules
NM
1–5 mm white papules
2 mm papules
3.5–5.5 mm white firm papules
2–3 mm buff papules
5 mm white firm papules
Multiple, slightly raised, whitish, 1–4 mm Various size papules (less than 8 mm in diameter)
Evolution of the lesions
F female, M male, mo month, NM not mentioned, No. number, wks weeks
Cases 4 and 5 are from a family. Cases 22–25 are from Chinese literature
Age/ duration (years)
No.
Table 1 continued
No treatment
Poor response to topical tretinoin gel
No treatment
Poor response to topical tretinoin gel
NM
NM
No treatment
Failed in responding to intra-lesional injection of steroids and liquid nitrogen cryotherapy
NM
NM
No treatment
NM
Therapy
Decrease and fragmentation
Loss, decrease and fragmentation
Decrease and fragmentation
Localized fragmentation in lesional area
Decrease and prominent fragmentation
Diminution and fragmentation
Fragmentation, decrease or disappear
Decrease and fragmentation
Decrease
Decrease and fragmentation
Rarefaction and fragmentation of elastic fibers in the reticular dermis
Reduction, thinning, and fragmentation
Elastic fibers
Moderate fibrosis
Nonfollicule
Nonfollicle
Nonfollicle
Condensed collagen No change
Nonfollicle
Nonfollicle
NM
Nonfollicle
Nonfollicle
NM
Nonfollicle
Nonfollicle
Nonfollicle
Follicles
Mild condensed collagen
Slight fibrosis
Normal
Increase collagen
Increased collagen and fragmentation
Homogenized collagen
Condense collagen
Homogenized collagen
NM
Collagen bundles in dermis
Present
Present
Present
Present
Sabin et al. [23]
Emre et al. [12]
Wang et al. [31]
Dang et al. [10]
Hao et al. [15]
Zhou et al. [33]
Almaza´nFerna´ndez et al. [1] Thome et al. [29]
References
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Fig. 4 a, b Homogenized and condensed collagen in the upper dermis, the epidermis is normal (hematoxylin–eosin). c, d Decrease of elastic fibers in the superficial dermis, and fragmentation and thin
of remaining elastic fibers elastic (Aldehyde-Fuchsin stain) (the length of the white bar is 1 mm for a and c; and 200 lm for b and d, respectively)
The clinical diagnosis of PE is often a challenge for physicians because of its rarity. Based on the clinical manifestations and histopathology examinations, there are several elastic tissue disorders mimicking those of PE, which include, NA, Buschke–Ollendorff syndrome, white fibrous papulosis of the neck (WFPN), PXE-PDE, middermis elastolysis, late-onset focal dermal elastosis, eruptive collagenoma, as well as papular acne scars. NA, first described by Lewandowsky in 1921 [19], was termed by Staricco and Mehregan in 1961 [27] to distinguish the connective tissue nevi in which elastic tissue is increased (nevus elasticus or elastoma) from that in which elastic fibers are lost (NA). It is an exceptionally rare disorder characterized by perifollicular papules and loss of elastic tissue, or fragmentation of the residual elastic fibers in the upper and mid dermis [9, 19]. To our knowledge, only four cases have been reported until present [9, 19; De Simone P, Catricala` C, Mariani G, Muscardin L, Silipo V (2009) Connective tissue nevus: report and discussion about one case, unpublished; Park
YL, Lee JS, Whang KU, Lee SH (2011) A case of nevus anelasticus, unpublished]. PXE-PDE, described by Rongioletti and Rebora [21] in 1992, is an acquired elastolytic disease and affects more often elderly women without systemic involvement [2, 19, 21, 30]. It always presents as asymptomatic, bilateral and symmetrical yellowish papules that coalesce into cobblestone plaques predominantly in the neck and supraclavicular regions [19]. PXE-PDE always reveals atrophic epidermis [19], absence or marked loss of elastic fibers on the papillary dermis, and absence of calcifications or fragmentation of the elastic fibers [2, 19, 21, 30]. WFPN has clinical features of multiple, asymptomatic, symmetric, isolated, 2- to 3-mm diameter, nonfollicular, white or pale papules distributed over the upper sternal and neck areas [19, 30]. The typical pathologic changes are thickened collagen bundles in the papillary and mid-dermis, and loss of elastic tissue in the papillary and reticular dermis as well [19]. Mid-dermis elastolysis is characterized by patches and plaques of finely wrinkled skin with focal
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loss of elastic tissue in the midreticular dermis [14, 19]. Late-onset focal dermal elastosis is characterized by slowly progressive, asymptomatic, 1–3 mm diameter, yellow papules which mimic the eruption of pseudoxanthoma elasticum [19]. Its histopathology shows a focal increase in normal-appearing elastic tissue. Upper dermal elastolysis presents as 2- to 5-mm yellowish papules on the neck, shoulders, upper chest and upper back with complete loss of elastic fibers in the upper dermis including papillary dermis and intact fibers in the dermis [16]. Papillary dermal elastosis presents as scattered, white-to-yellow, 1–2 mm in diameter papules with normal surface on the upper back and neck region, its histologic features reveal foci of clumped, granular and curled elastic fibers replacing oxytalan and elaunin, alternating with focal decrease of normal-appearing elastic fibers within the papillary dermis [30]. Eruptive collagenoma, first described by Colomb in 1955, is a rare type of collagenoma presenting as multiple whitish papules measuring 2–5 mm in diameter, which distribute on the trunk during adolescence. Histological examination is characterized by an excessive accumulation of homogenization and thickening of collagen fibers in the dermis and a reduction of elastic fibers [22, 26]. Papular acne scars, first described by Wilson et al. [32] present as small, asymptomatic, hypopigmented, follicular papules distributed on the upper part of the trunk. Most of the patients have a history of truncal acne. Histologic examination reveals circumscribed, perifollicular or parafollicular lesions with a marked decrease in elastic and collagen fibers in the dermis, which is quite different from PE. The pathogenesis of PE remains unclear. As it is an exceptionally rare disorder and is always assessed on a caseby-case basis, it is hard to have further study at present. There is no reliable cure for most of the elastic tissue diseases including NA and PE [19]. Based on the present materials, it seems that no suggestive therapeutics is recommended for PE so far. Two of our patients had poor response to short-term use of topical tretinoin, though it was reported that retinaldehyde cream could repair UVAinduced elastic fiber damage [3] and 3 months therapy of topical tretinoin 0.1 % gel resulted in decreased prominence of the lesions in papillary dermal elastosis [30], we suggested that a long-term observation for the response should be studied if possible. As the disease is only a cosmetic concern without other associations, hence, we considered that a ‘‘wait-and-see’’ approach may be optional at present. As NA, PE, WFPN, upper dermal elastolysis, and papillary dermal elastosis present with papules and loss or decrease of elastic tissue in dermis, we consider that these disorders may belong to the same spectrum, and their different clinical and pathological features are because of different stages and locations.
Because of symptomless, PE may be neglected by the patients, the doctors, or even the both. Consequently, its true prevalence may have been underestimated. Of course, PE may be much more frequent if correctly recognized. Conflict of interest
All the authors have no conflicts of interest.
References 1. Almaza´n-Ferna´ndez FM, Clemente-Ruiz de Almiro´n A, SanzTrelles A, Ruiz-Carrascosa JC, Naranjo-Sintes R (2011) Papular elastorrhexis: a case report and principal differential diagnoses. Actas Dermosifiliogr 102:549–550 2. Alves R, Ferreira L, Vale E, Bordalo O (2010) Pseudoxanthoma elasticum papillary dermal elastolysis: a case report. Dermatol Res Pract Doi:pii: 352724. 10.1155/2010/352724 3. Boisnic S, Branchet-Gumila MC, Le Charpentier Y, Segard C (1999) Repair of UVA-induced elastic fiber and collagen damage by 0.05 % retinaldehyde cream in an ex vivo human skin model. Dermatology 199(Suppl 1):43–48 4. Bordas X, Ferra´ndiz C, Ribera M, Galofre´ E (1987) Papular elastorrhexis: a variety of nevus anelasticus? Arch Dermatol 123:433–434 5. Buechner SA, Itin P (2002) Papular elastorrhexis. Report of five cases. Dermatology 205:198–200 ´ , Ciria S, Gonza´lez R, Una6. Can˜ueto J, Roma´n C, Santos-Briz A muno P (2011) Papular elastorrhexis and Buschke-Ollendorff syndrome are different entities. J Am Acad Dermatol 65:e7–e9 7. Choi Y, Jin SY, Lee JH, Kwon HB, Lee AY, Lee SH (2011) Papular elastorrhexis: a case and differential diagnosis. Ann Dermatol 23(Suppl 1):S53–S56 8. Choonhakarn C, Jirarattanapochai K (2002) Papular elastorrhexis: a distinct variant of connective tissue nevi or an incomplete form of Buschke–Ollendorff syndrome? Clin Exp Dermatol 27:454–457 9. Crivellato E (1986) Disseminated nevus anelasticus. Int J Dermatol 25:171–173 10. Dang Y-P, Zhao G, Ma H-J (2007) A case of papular elastorrhexis. J Clin Dermatol 36:565–566 (Chinese) 11. Del Pozo J, Martı´nez W, Sacrista´n F, Ferna´ndez-Jorge B, Fonseca E (2008) Papular elastorrhexis, a distinctive entity? Am J Dermatopathol 30:188–190 12. Emre S, Metin A, Demir-Pektas¸ S, Kılıc¸arslan A (2013) Papular elastorrhexis. Cutis 92(1):E4–E5 13. Flores PB, Cuevas J, Sa´nchez C, De Eusebio E, Vergara A (2010) Papular elastorrhexis: an acquired disorder of elastic tissue. Eur J Dermatol 20:525–526 14. Gambichler T (2010) Mid-dermal elastolysis revisited. Arch Dermatol Res 302:85–93 15. Hao H-S, Zhang X-F, Liu Y, Wang J (2010) A case of papular elastorrhexis. Chin J Derm Venereol 24:652–653 (Chinese) 16. Hashimoto K, Tye MJ (1994) Upper dermal elastolysis: a comparative study with mid-dermal elastolysis. J Cutan Patbol 21:533–540 17. Lee SH, Park SH, Song KY, Yoon TJ, Kim TH (2001) Papular elastorrhexis in childhood improved by intralesional injections of triamcinolone. J Dermatol 28:569–571 18. Lewis KG, Bercovitch L, Dill SW, Robinson-Bostom L (2004) Acquired disorders of elastic tissue: part I. Increased elastic tissue and solar elastotic syndromes. J Am Acad Dermatol 51:1–21 19. Lewis KG, Bercovitch L, Dill SW, Robinson-Bostom L (2004) Acquired disorders of elastic tissue: part II. Decreased elastic tissue. J Am Acad Dermatol 51:165–185
123
Arch Dermatol Res 20. Pajot C, Le Clec’h C, Hoareau F, Croue A, Verret JL (2008) Two cases of papular elastorrhexis. Ann Dermatol Venereol 135: 757–761 21. Rongioletti F, Rebora A (1992) Pseudoxanthoma elasticum-like papillary dermal elastolysis. J Am Acad Dermatol 26:648–650 22. Ryder HF, Antaya RJ (2005) Nevus anelasticus, papular elastorrhexis, and eruptive collagenoma: clinically similar entities with focal absence of elastic fibers in childhood. Pediatr Dermatol 22:153–157 ¨ , Sezer E, Cetin ED (2013) Eruptive papular 23. Sahin S, Durmaz EO elastorrhexis of the face and scalp. J Am Acad Dermatol 69:e251–e252 24. Schirren H, Schirren CG, Stolz W, Kind P, Plewig G (1994) Papular elastorrhexis: a variant of dermatofibrosis lenticularis disseminata (Buschke–Ollendorff syndrome)? Dermatology 189:368–372 25. Sears JK, Stone MS, Argenyi Z (1988) Papular elastorrhexis: a variant of connective tissue nevus. Case reports and review of the literature. J Am Acad Dermatol 19((2 Pt 2)):409–414 26. Sharma R, Verma P, Singal A, Sharma S (2013) Eruptive collagenoma. Indian J Dermatol Venereol Leprol 79:256–258
123
27. Staricco RG, Mehregan AH (1961) Nevus elasticus and nevus elasticus vascularis. Arch Dermatol 84:943–947 28. Tan C, Zhu WY, Min ZS (2009) Papular elastorrhexis located on occipito-cervical and mandibular regions. Eur J Dermatol 19:399–400 29. Thome´ EP, Steglich RB, Meotti CD, Schwartz J, Boff AL (2012) Case for diagnosis. Papular elastorrhexis. An Bras Dermatol 87:651–653 30. Wang AR, Lewis K, Lewis M, Robinson-Bostom L (2009) Papillary dermal elastosis: a unique elastic tissue disorder or an unusual manifestation of pseudoxanthoma elasticum-like papillary dermal elastolysis? J Cutan Pathol 36:1010–1013 31. Wang H-M, Xiao Y, Ji H-A, Chen D, Wang Q-W, Xue C-W (2012) One case of papular elastorrhexis. Chin J Derm Venereol 26:434–435 (Chinese) 32. Wilson BB, Dent CH, Cooper PH (1990) Papular acne scars. A common cutaneous finding. Arch Dermatol 126:797–800 33. Zhou X, Tian X, Wang J-M, Dong Y-H (2010) A case of papular elastorrhexis. Chin J Derm Venereol 24:190 (Chinese)