Unilateral Congenital Linear Atrophoderma of the Leg

Abstract

We report an infant with depressed, hypopigmented, linear plaques of congenital onset on the lower extremity. The lesions were asymptomatic and the child was otherwise healthy. Despite the clinically obvious change in skin texture and color, histopathologic changes were subtle: a biopsy specimen showed hypopigmentation and a decrease in elastic fibers in the papillary and upper reticular dermis. Diagnoses considered included various congenital syndromes, idiopathic atrophoderma of Pasini and Pierini, and especially, linear atrophoderma of Moulin. However, because of the significant clinical and histopathologic differences when compared to the aforementioned entities, our patient appears to have a unique presentation of congenital linear atrophoderma.

Bilateral atrophoderma linearis: a relationship between atrophoderma linearis Moulin and atrophoderma Pasini–Pierini?

“…Review of the literature reveals two cases of congenital APP: a female case born with erythematous atrophic lesion on the right shoulder and a male case born with a plaque of APP on his lower right back . Ang et al . presented a child with congenital, linear, hypopigmented atrophoderma of the lower extremity, which shared some clinical features with ALM.…”

Linear Atrophoderma of Moulin:A Case Report and Review of the Literature

“…Hematoxylin and eosin staining usually shows hyperpigmentation only in basal epidermal layers without abnormal collagen or elastic fibers in the dermis or any obvious inflammation [1, 3, 4]. There may be some perivascular lymphocytic infiltration [2, 5–12], acanthosis [7, 8], epidermal atrophy [6], altered collagen in the dermis [2, 5–7, 9, 10, 13], and decreased or fragmented elastic tissue [14]. Once there is some alteration of the collagen component, the diagnosis should be atrophoderma of Pasini and Pierini (APP) [15].…”

“…Linear scleroderma and APP may show a segmental atrophic plaque similar to LAM. Abnormal collagen fibers in the histopathological findings and dermal tissue reduction in the ultrasound imaging are helpful for the diagnosis of linear scleroderma and APP, respectively [2, 14]. Considering the clinical manifestation of LAM, i.e.…”

Linear Atrophoderma of Moulin: Report of 4 Cases and 20th Anniversary Case Review

“…These variants include bilateral telangiectatic lesions, the presence of leukonychia, lentiginous variant and neck localization. Ang et al [25] reported a case of unilateral congenital atrophoderma of the leg and discussed a congenital form of LAM, but lesions were hypopigmented and they observed a decrease in elastic fibers in the papillary dermis with elastin stains. In our view, only cases with clearly pigmented and atrophic lesions arranged along Blaschko's lines should be considered as LAM.…”