Total hemiatrophy. Association with localized scleroderma, Schönlein-Henoch nephritis, and paroxysmal nocturnal hemoglobinuria.
In the clinical course of a patient with progressive facial hemiatrophy associated with ipsilateral body atrophy (total hemiatrophy), signs and symptoms of localized scleroderma were noted. The patient subsequently was found to have Schönlein-Henoch purpura with renal involvement and, later, paroxysmal nocturnal hemoglobinuria. To our knowledge, such an association has not been reported before.
D000328:Adult; D001284:Atrophy / Q000150:complications; D005150:Facial Hemiatrophy / Q000150:complications*; D005260:Female; D005921:Glomerulonephritis / Q000150:complications* / Q000473:pathology; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications*; D006801:Humans; D011695:IgA Vasculitis / Q000150:complications*; D012594:Scleroderma, Localized / Q000150:complications*