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Case Reports

Abstract

A 74-year-old woman with no pertinent past medical history presented with a mildly pruritic eruption of 1.5 years duration. On examination, discrete skin-colored grouped papules were appreciated along the intermammary chest, mid-back, axillary folds, and posterior scalp. The lesions were mildly pruritic with sweating. A previous biopsy by an outside dermatologist was interpreted as chronic perivascular dermatitis. Repeat punch biopsies of the axilla and chest demonstrated normal H&E findings, however significantly decreased papillary dermal elastic fibers on Verhoeff–van Gieson stain. Given the clinical presentation and histologic findings, the patient was diagnosed with pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE).

PXE-PDE is an under-reported disorder characterized by loss or markedly reduced elastic fibers within the papillary dermis. Clinically, it is characterized by soft, yellow to skin-colored papules that coalesce into cobblestone plaques commonly involving the neck, scalp, axilla, and supraclavicular areas, often seen in postmenopausal women. Lesions are typically asymptomatic and systemic symptoms are lacking. It is considered a subtype of fibroelastolytic papulosis, a term proposed to encompass a spectrum of cutaneous elastic tissue fiber diseases alongside white fibrous papulosis of the neck and papillary dermal elastosis.  The histologic findings of PXE-PDE show complete or significantly decreased elastic fibers of the papillary dermis highlighted by elastic tissue special stains. Melanophages in the absence of interface inflammation is a frequent finding. Epidermal atrophy and a nonspecific perivascular lymphocytic infiltrate are occasionally observed. Given the subtle histologic findings on H&E, correlation with clinical examination findings is important and necessary for the diagnosis.