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Case ReportsAbstract
A 68-year-old male presented with non-painful right foot and toe lesions of 5 years’ duration, refractory to hydroxychloroquine (9 months), methotrexate (8 months), and adalimumab (12 weeks). Examination revealed a swollen, indurated right third toe with overlying violaceous scaly plaques, and erythematous papules on the right dorsal foot. Of note, he had a 11-year history of hilar lymphadenopathy and a 1-year history of biopsy-proven intranasal cutaneous sarcoidosis (lupus pernio), responsive to intralesional triamcinolone. Foot radiographs demonstrated cortical irregularity of the right third distal phalanx tuft with surrounding soft tissue swelling, compatible with inflammatory-related acro-osteolysis. Three skin biopsies from the toe and foot demonstrated superficial and deep dermal non-necrotizing epithelioid granulomatous inflammation; GMS, PAS-D, and Fite stains and pan-tissue cultures were negative. Findings were consistent with cutaneous sarcoidosis and associated acro-osteolysis. Multidisciplinary evaluation, including laboratory and imaging studies, found no systemic involvement. Given the refractory nature, intralesional steroid injections or treatment with JAK inhibitors were considered. This case is notable for its unusual distribution, with lupus pernio-like lesions of the toes and feet preceding nasal disease, highlighting an atypical initial presentation of cutaneous sarcoidosis. Osseous involvement occurs in up to 15% of cases, often affecting the phalanges of the hands and feet in an osteolytic pattern, and is frequently associated with lupus pernio, as seen in our patient. The heterogenous nature of sarcoidosis can make diagnosis challenging; therefore, dermatopathologists should consider it in the differential when non-necrotizing granulomas are present, even in unusual sites.