Panatrophy of Gowers associated with primary antiphospholipid syndrome: Description of an unusual case [Panatrofia di Gowers associata a sindrome da anticorpi antifosfolipidi primitiva: descrizione di un caso singolare]

An unusual case of Panatrophy of Gowers (PG) is here reported. PG is a rare disorder of skin and subcutaneous tissue, characterized by areas of atrophy in different parts of the integument, without any preceding inflammation. The etiopathogenesis is unknown, but the association of PG with multiple autoimmune diseases suggests an immunologic mechanism. A 49 years old woman, affected by PG since 1990, was admitted to our Institute in december 1993, because of a progressive development of arthralgias and myalgias, dyspnoea, vertigo, derangement and thrombophlebitis. The clinical features, the instrumental and biochemical investigations, in particular the positivity of antiphospholipid antibodies, allowed the diagnosis of primary antiphospholipid syndrome (APS). The term of primary APS has been introduced as a means of categorizing and studying these patients who do not have any feature of systemic lupus erythematosus or any other well defined disease. The feature of the antiphospholipid syndrome - arterial and venous thrombosis, livedo, strokes, migraine, recurrent abortions, occasional thrombocytopenia - have gradually expanded to embrance many specialities: nephrology (glomerular thrombosis), psychiatry (multi-infarct dementia), orthopedics (avascular necrosis), gastroenterology (Budd-Chiari Syndrome), endocrinology (Addison's disease), cardiology (valvular disease, pulmonary hypertension). The association of APS and PG is not reported in literature; so this case appears particulary interesting and suggests the possibility that PG can represent a clinical manifestation of a systemic vasculitis.