Degos Disease (Malignant Atrophic Papulosis) With Granular I
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Degos disease is a rare vasculopathy characterized by skin papules with central porcelain white atrophy and a surrounding telangiectatic rim.

A 45-year-old female who presented with numerous red papules with sclerotic white centers and minimal systemic symptoms. Laboratory workup was notable for a negative autoimmune panel and hypercoagulation panel.

Histopathology revealed epidermal atrophy, abundant dermal mucin, a perivascular lymphocytic infiltrate, interface inflammation, papillary dermal hemorrhage, and several small thrombi in the mid-to-superficial vessels.

Direct immunofluorescence (DIF) showed strong granular immunoglobulin M (IgM) deposition at the dermal-epidermal junction.

Based on the pathognomonic skin findings, persistently negative antinuclear antibody, lack of systemic signs of systemic lupus erythematosus, and characteristic hematoxylin and eosin findings, a diagnosis of Degos disease was rendered.

Source: https://www.cureus.com/articles/39989-degos-disease-malignant-atrophic-papulosis-with-granular-igm-on-direct-immunofluorescence
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