An unusual duo: Immunodeficiency disorder and scleroderma.

A 45-year-old woman on treatment for HIV infection with highly active antiretroviral therapy for the past 10 years presented to us with a history of Raynaud's phenomenon and hyperpigmentation of the skin for 2 years. She was diagnosed to have pulmonary arterial hypertension 8 months ago. On examination, she had salt-and-pepper pigmentation and sclerodactyly. Her biochemical workup was normal. She had positive antinuclear antibody by indirect immunofluorescence method. Skin biopsy was consistent with systemic sclerosis. HIV has its own musculoskeletal manifestations. The paradox of autoimmunity in the background of immunodeficiency was intriguing. Treating autoimmunity in the presence of immunodeficiency was challenging. The attribution and differentiation of pulmonary hypertension were difficult. There has been a homology identified between human immunodeficiency virus 1 (HIV 1) and centromere B protein (CENP B). This case is reported because of the unusual occurrence of systemic sclerosis in an HIV patient.