A New Variant of Connective Tissue Nevus with Elastorrhexis and Predilection for the Upper Chest

Abstract

Localized changes in cutaneous elastic tissue often manifest with flesh-colored, hypopigmented, or yellow papules, plaques, and nodules. We present five children with clinically similar cobblestone plaques composed of multiple hypopigmented, nonfollicular, pinpoint papules located unilaterally over the upper chest. All lesions first appeared at birth or during early infancy. No associated extracutaneous abnormalities have been identified. Histopathology was remarkable for many, thick elastic fibers with elastorrhexis. We believe that these cases represent a distinct and unique variant of connective tissue nevi.

Papular elastorrhexis: a rare and benign disease

“…The diagnosis of papular elastorrhexis is based on clinical and histopathological findings. 6 The histopathological assessment of the lesions reveals characteristic rarefaction and important fragmentation of the elastic fibers of the reticular dermis. 4 , 5 There can be a perivascular lympho-histiocytic infiltrate.…”

Papular Elastorrhexis: A Case Report of Rare Connective Tissue Naevus

“…1 It is diagnosed with good clinical acumen and histologically with fragmented or decreased dermal elastic fibers, with or without changes in collagen bundles in the dermis. 4 PE is difficult for the clinicians to diagnose because of the rarity of the disease and non-specific presentation. 1 Here we report a rare case of PE.…”