Usefulness of a Simple Immunohistochemical Staining Technique to Differentiate Anti-p200 Pemphigoid From Other Autoimmune Blistering Diseases: A Report of 2 Cases. / Descripción de 2 casos de penfigoide anti-p200. Utilidad de una técnica inmunohistoquímica sencilla en el diagnóstico diferencial con otras enfermedades ampollosas autoinmunes.

Anti-p200 pemphigoid is a rare autoimmune subepidermal blistering disease characterized by the presence of circulating immunoglobulin G antibodies directed against laminin gamma-1, a 200-kDa protein located in the lamina lucida of the basement membrane. We review the clinical, histopathological and immunological characteristics of the first 2 cases described in Spain. Anti-p200 pemphigoid shares histopathological and immunopathological findings with epidermolysis bullosa acquisita, the main entity in the differential diagnosis. However, its management follows the same guidelines as those used for bullous pemphigoid. The diagnosis is confirmed by immunoblotting, which is a complex technique available in few centers. We propose the immunohistochemical detection of collagen type IV on the floor of the blister, combined with standard immunofluorescence techniques, as a simple, accessible alternative to differentiate anti-p200 pemphigoid from epidermolysis bullosa acquisita.

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.

[Clinical guidelines for the diagnosis and treatment of dermatitis herpetiformis]. / Guía clínica de diagnóstico y tratamiento de la dermatitis herpetiforme.

Dermatitis herpetiformis is an autoimmune blistering disease that appears as a cutaneous manifestation of gluten intolerance. It is one of a group of disorders that have gluten sensitivity in common, including celiac disease and gluten ataxia. Patients with dermatitis herpetiformis present with a pruritic papulovesicular rash on extensor surfaces and on the buttocks. Immunological studies demonstrate the presence of specific immunoglobulin (Ig) A anti-endomysial and anti-transglutaminase antibodies. The finding of granular deposits of IgA along the dermal-epidermal junction is pathognomonic of dermatitis herpetiformis. Treatment of dermatitis herpetiformis is based on a life-long, strict gluten-free diet, which improves all clinical aspects of gluten sensitivity, and dapsone, a drug that is only effective for the skin manifestations.

[Practical management of the most common autoimmune bullous diseases]. / Manejo práctico de las enfermedades ampollosas autoinmunes más frecuentes.

Autoimmune bullous diseases are relatively uncommon and their treatment -- although generally similar -- may vary depending on the dermatologist. Within this group of diseases, the most common are pemphigus vulgaris and pemphigus foliaceus, bullous and mucosal pemphigoid, linear immunoglobulin A disease, and dermatitis herpetiformis. In recent years, the therapeutic arsenal has been extended by new drugs, some of which have changed the prognosis of these diseases. This article describes current management protocols for these processes as indicated in the literature and derived from the experience of specialized clinics for bullous diseases. We also present the findings from an Internet survey on therapeutic approaches in pemphigus vulgaris answered by more than 40 dermatologists who work primarily in Spanish hospital clinics.

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.

Successful adjuvant treatment of severe bullous pemphigoid by tryptophan immunoadsorption.

Bullous pemphigoid (BP) is an autoimmune blistering skin disease associated with circulating autoantibodies to the hemidesmosomal antigens BP180 and BP230. We report two cases of therapy-refractory BP adjuvantly treated by tryptophan immunoadsorption. In both patients, this treatment was associated with rapid clinical improvement and reduction in the required corticosteroid dosage. In addition, levels of circulating anti-BP180 autoantibodies decreased markedly. Antibodies that were eluted from the tryptophan matrix bound to BP180 and induced dermal-epidermal separation in cryosections of human skin. Our observations suggest that immunoadsorption may be a helpful adjuvant treatment in severe BP.

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.