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

123

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.

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Papular elastorrhexis: report of four cases and review of literature.

Papular elastorrhexis is a rare cutaneous disorder; only 27 cases have been reported before the present review. We added four new cases, and reviewed ...
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