Epidermolysis bullosa acquisita: a retrospective analysis of 12 patients evaluated in four tertiary hospitals in Spain.

BACKGROUND: Epidermolysis bullosa acquisita is an exceedingly rare subepidermal blistering disease caused by antibodies against type VII collagen. Studies summarizing the clinical and immunological features of this disease in large series of patients are scarce. OBJECTIVES: To analyse the clinical and immunopathological characteristics, treatment responses and outcomes of 12 patients with epidermolysis bullosa acquisita from four tertiary hospitals in Spain. METHODS: An extensive retrospective review of clinical charts. RESULTS: The mean age of onset was 48 years and the mean delay to diagnosis was 20·75 months. The classical phenotype occurred in 42% of cases, inflammatory in 42% and mixed in 17%. Mucosal involvement was present in 75%. Linear IgG deposition along the basement membrane zone was consistently present on direct immunofluorescence examination. Indirect immunofluorescence study was positive in 67% of the cases. Frequently associated diseases were neoplasms (25%), inflammatory bowel disease (25%), hepatitis C virus infection (17%) and thyroid dysfunction (17%). Therapeutic responses were variable. CONCLUSIONS: The prevalence of neoplasms was similar to that seen in inflammatory bowel disease. Multicentric prospective studies including larger numbers of patients are required for a better knowledge and management of this disease.

Progressive supravenous granulomatous nodular eruption in a human immunodeficiency virus-positive intravenous drug user treated with highly active antiretroviral therapy.

We describe a 41-year-old human immunodeficiency virus-infected woman with a previous history of intravenous drug abuse, who developed multiple linear nodules following the superficial veins on both arms. Histopathological examination disclosed a dermal histiocytic inflammatory reaction with sarcoid-like granuloma formation occasionally showing an intracytoplasmic refractile material in the histiocytic cells. Nodular lesions developed progressively after starting on highly active antiretroviral therapy (HAART) which increased her CD4 cell count and suppressed her viral load. The appearance of latent inflammatory or autoimmune disease following HAART is a well-recognized phenomenon. We consider that this peculiar 'progressive supravenous granulomatous nodular eruption' should be included within the spectrum of the so-called immune reconstitution inflammatory syndrome.

Primary anetoderma associated with primary Sjögren's syndrome.

We report the case of a woman with a primary Sjögren's syndrome who developed asymptomatic anetoderma lesions with no other pathology responsible. This dermatosis has been associated with many autoimmune disorders, in particular lupus erythematosus and lupus-like syndromes. Our literature review found only one previous description of primary anetoderma associated with primary Sjögren's syndrome.