Papular elastorrhexis: report of four cases and review of literature

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 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.

Immunopathological and ultrastructural features in a case of papular elastorrhexis

“…Thirty-one cases of PE have been reported [ 1 ] , but immunohistochemical and ultrastructural data is limited. Thirty-one cases of PE have been reported [ 1 ] , but immunohistochemical and ultrastructural data is limited.…”

“…In 1987, PE was initially considered a variant of nevus anelasticus [ 1 ] . Later, in 1994, it was proposed to be an abortive form of Buschke-Ollendorff syndrome (BOS) [ 2 ] .…”

“…However, recent fi ndings suggest that PE is not an incomplete variant of BOS, as there have been two cases of PE failing to show LEMD3 mutations [ 3 ] . Papular elastorrhexis is distinct from several elastic tissue disorders including nevus anelasticus, BOS, eruptive collagenoma, pseudoxanthoma elasticum-like papillary dermal elastolysis, and white fi brous papulosis of the neck [ 1 ] . Histopathologically, the prominent feature is fragmentation, thinning, or complete loss of dermal elastic fi bers.…”

Multiple yellowish white papules on the trunk and upper arms

“…Papular elastorrhexis is diagnosed by the nature of skin lesions, including their distribution and size, and histopathological findings. It is necessary to differentiate it from congenital collagenoma such as Buschke‐Ollendorff syndrome, pseudoxanthoma elasticum (PXE), pseudoxanthoma elasticum‐like papillary dermal elastolysis (PXE‐like PDE), and white fibrous papulosis of the neck . Congenital collagenoma ranges from soft to firm skin‐colored or yellowish nodules or plaques which are often larger lesions than papular elastorrhexis.…”

Small non‐follicular white papules on the back of the hands and the mandibular region

“…Ihre genaue Ätiologie ist nicht bekannt [2]. junge Erwachsene, von denen 65 % Frauen sind [2]. Läsionen auf den Handrücken und im Gesicht sind erst seit Kurzem bekannt [3].…”