Schamberg’s disease affecting both upper and lower limbs

A 59-year-old male, presented with asymptomatic reddish-brown lesions over bilateral lower limbs for three years. Two months following the initial lesions, reddish-colored macules also developed over the bilateral forearms. A history of prolonged standing (eight to ten hours/day) was present.

On cutaneous examination, multiple discrete reddish-brown macules with ‘cayenne pepper spots’ were present over the bilateral ankles extending up to one-third of the lower legs [Figure 1a]. Bilateral forearms showed numerous discrete nonpalpable, nonblanchable petechiae [Figure 1b]. Dilated tortuous veins were visible in the legs.

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Figure 1:

(a) Multiple discrete reddish – brown macules giving a ‘cayenne pepper appearance’ diffusely present over the bilateral ankles, predominantly around medial malleolus extending up to one-third of the legs. Numerous dilated tortuous veins visible over the bilateral shins. (b) Numerous pinhead size non palpable and non blanchable petechiae present over the extensors of bilateral forearms.

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Biopsy taken from leg and forearm showed similar findings of increased vascularity in papillary dermis with edema, RBCs extravasation, perivascular, and periadnexal lymphocytic infiltrate. No leukocytoclasia or plump endothelial cells are seen ruling out leukocytoclastic vasculitis. Based on the clinical characteristics and histopathological findings, the patient was diagnosed with a case of Schamberg’s disease affecting both upper and lower limbs. Schamberg’s disease is a form of pigmented purpuric dermatoses which presents as asymptomatic reddish-brown macules resembling cayenne pepper appearance commonly presenting over the lower limbs.^[1]