A 45-year-old male presented with asymptomatic, well-defined, periarticular papules gradually evolving into nodules predominantly on palms, dorsum of hands, and extensor surface of bilateral elbows for the last five years (Figure 1 and 2). Examination revealed well-defined, firm, yellowish to erythematous nodules measuring 0.5 to 5 cm in diameter. On palms, lesions were crusted and ulcerated with surrounding erythema (Figure 2). He visited multiple medical centers for his complaint without improvement.

Complete blood count, liver function test, renal function test, thyroid function test, serum electrolytes, anti-nuclear antibody (ANA), Rheumatoid factor, Anti-cyclic citrullinated peptide (anti-CCP), uric acid, and serological tests including HIV were within normal limit. An X-ray of his hands revealed osteophytic changes without joint involvement. Excisional biopsy of the nodule at elbow revealed epidermis with compact orthokeratosis, dermis showing leukocytoclastic vasculitis of small blood vessels along with well-circumscribed dermal nodule composed of dense cellular infiltrates. (Figure 3). Reticular dermis shows storiform fibrosis (Figure 3). Diagnosis of EED was made and he was started on dapsone. A good response was seen in the first month but he lost to follow-up.