Linear atrophoderma of Moulin: an underrecognized entity

Abstract

Linear atrophoderma of Moulin (LAM) is an acquired skin condition that manifests in early childhood and adolescence. It likely represents a form of cutaneous mosaicism that presents with linear, hyperpigmented and atrophic lesions appearing on the trunk and limbs. Its clinical appearance varies and may closely resemble that of atrophoderma of Pasini and Pierini (APP) and linear scleroderma. LAM usually follows a benign course and no effective treatment options exist. We present a case of a young and healthy patient that developed such lesions on her upper and lower extremities over 5 years. The initial clinical impression of linear scleroderma was reviewed in favor of LAM following histological examination of the lesions which revealed no significant inflammatory changes. LAM remains a rare and possibly under recognized entity with reports confined only to the dermatologic literature. This case highlights the importance of recognizing LAM and distinguishing it from linear scleroderma given the significant differences in management and prognosis.

Linear perioral atrophoderma along blaschko's lines‐ a retrospective study describing 14 cases of atrophia maculosa varioliformis cutis

“…It usually presents as asymptomatic, solitary or few, hyperpigmented atrophic plaques that resemble linear scleroderma, a subtype of morphoea. However, the preceding inflammation, sclerosis and induration that accompany linear scleroderma is usually absent in LAM 12 …”

A boy with linear hyperpigmented atrophic plaques

“…Differential diagnosis of LAM includes atrophoderma of Pasini and Pierini (APP) and linear morphea. Zahedi Niaki even hypothesized that LAM, APP, and linear morphea may exist on a spectrum rather than being distinct disorders 9 . Other linear dermatoses such as ILVEN, lichen striatus, hyperpigmented mosaicism, linear lupus erythematosus, or dominant X‐linked dermatoses such as incontinentia pigmenti or focal dermal hypoplasia have to be considered as well.…”

Linear atrophoderma of moulin over face: An exceedingly rare entity

“…Atrophoderma of Pasini and Pierini clinically and histologically resembles LAM except that it does not follow Blaschko's lines. [5] Porokeratosis was excluded clinically and histopathologically by the absence of keratotic ridge with central groove and cornoid lamella, respectively. [2]…”