[Chronic benign familial pemphigus. Clinical, histological and immunological study of 7 cases]. / Pénfigo familiar benigno crónico. Estudio clínico, histológico e inmunológico de siete casos.

A clinical, histopathological and immunological study was carried on a series of seven patients of Familial Benign Chronic Pemphigus (FBCP). This condition is characterized by recurrent small blisters, mainly, on intertriginous areas and on the sides of the neck, that become wet and crusted rapidly. They are generally sharply marginated and Nikolsky's sign is often positive. The lesions appear spontaneously and may be precipitated by warm, humid environment, mechanical trauma, radiations, bacterial or mycotic infection. Healing occurs with residual non scarring hyperpigmentation. Histopathologically, the epidermal alteration respond to a primary acantholytic mechanism. Ultramicroscopic studies have suggested an alteration on the desmosome-tonofilament complex. Comparatively with Pemphigus, another acantholytic disease in which immunological pathogenesis is strongly suspected, only few reports are referred to immunological studies in FBCP. In the present paper, a direct immunofluorescent study on spontaneous and provoked blisters was made in order to investigate deposits of immunoglobulins and complement. Indirect IF was performed with sera of teh patients for detection of circulating antibodies. Two cases were also sensitized with erythrocytic antigen. The immunological response to this substance was evaluated. The clinical and histopathological findings of the present series, are similar with previous descriptions. The immunofluorescent studies do not provide evidence of antibodies to epidermal intercellular space, like Pemphigus.(ABSTRACT TRUNCATED AT 250 WORDS)

[Kaposi's angiosarcoma developing in a patient with a renal transplant]. / Angiosarcoma de Kaposi desarrollado en una paciente con transplante renal.

A patient with renal transplant developed muco-cutaneous Kaposi's sarcoma. The tumors appeared within a mont after surgery, while receiving daily 240 mgr . of methylprednisone and 150 mgr . of azathioprine. She received superficial radiotherapy ( Cesio 137) on her cutaneous lesions and the nasal tumor was removed with Laser radiation CO2 obtaining a favorable a response. Possible etiopathogenic mechanisms are mentioned. Clinical, epidemiologic, therapeutic and prognosis features of these transplanted subjects are discussed. Publications of other similar cases are reviewed.

Dermatosis ampollar cronica benigna del nino. Estudio clinico e inmunofluorescente de 6 casos. / Chronic benign bullous dermatosis of children Clinical and immunofluorescent study in 6 cases

La dermatosis ampollar cronica benigna del nino es una entidad caracterizada por la presencia de ampollas tensas, aisladas o agrupadas en forma de "roseta", que se localiza preferentemente en la zona pelviana pudiendo afectar otros sectores. La evolucion es benigna, con curacion en 2 o 3 anos. Fueron estudiados 6 casos en los cuales se destaca el predominio del sexo masculino (5/1) y algunas localizaciones poco frecuentes, como cuero cabelludo, orejas, manos, pies y mucosa lingual. La evolucion se efectuo por brotes, curando en el lapso de 3 a 41 meses, coincidiendo con la mayoria de los casos relatados.Desde el punto de vista histopatologico se observo una formacion ampollar dermoepidermica semejante a penfigoide ampollar en 3 casos y compatible com dermatitis herpetiformes en 2 de ellos. Los hallazgos de inmunofluorescencia directa no fueron constantes; en 2 casos se demostro deposito de IgA lineal en la zona de la union dermoepidermica, en otros 3 los resultados fueron negativos. En ninguno se pudo evidenciar anticuerpos circulantes con inmunofluorescencia

Dermatosis ampollar cronica benigna del nino. Estudio clinico e inmunofluorescente de 6 casos. / Chronic benign bullous dermatosis of children Clinical and immunofluorescent study in 6 cases

La dermatosis ampollar cronica benigna del nino es una entidad caracterizada por la presencia de ampollas tensas, aisladas o agrupadas en forma de "roseta", que se localiza preferentemente en la zona pelviana pudiendo afectar otros sectores. La evolucion es benigna, con curacion en 2 o 3 anos. Fueron estudiados 6 casos en los cuales se destaca el predominio del sexo masculino (5/1) y algunas localizaciones poco frecuentes, como cuero cabelludo, orejas, manos, pies y mucosa lingual. La evolucion se efectuo por brotes, curando en el lapso de 3 a 41 meses, coincidiendo con la mayoria de los casos relatados.Desde el punto de vista histopatologico se observo una formacion ampollar dermoepidermica semejante a penfigoide ampollar en 3 casos y compatible com dermatitis herpetiformes en 2 de ellos. Los hallazgos de inmunofluorescencia directa no fueron constantes; en 2 casos se demostro deposito de IgA lineal en la zona de la union dermoepidermica, en otros 3 los resultados fueron negativos. En ninguno se pudo evidenciar anticuerpos circulantes con inmunofluorescencia

Hereditary complement (C2) deficiency with discoid lupus erythematosus and idiopathic atrophoderma.

A family with hereditary deficiency of the second component of complement was studied. Three siblings were homozygous for C2 deficiency and two of them had associated skin diseases. One sister presented with idiopathic atrophoderma and the other had clinical and pathological manifestations of discoid lupus erythematosus. This is the first description of an association between idiopathic atrophoderma and C2 deficient state.

Immunologic response to A and B erythrocytic antigen.

Immunologic response to A and B erythrocytic antigen stimulation was studied in patients with pemphigus, in patients with systemic lupus erythematosus (SLE), and in normal subjects. Patients with pemphigus and normal subjects demonstrated a similar specific response (isohemagglutinins). A comparison between patients with pemphigus and SLE showed higher titers in the latter. Intercellular antibody titers increased after stimulation; corresponding titers were unaffected by isohemagglutinin absorption. Direct immunofluorescent studies showed IgG deposits in the intercellular spaces of the skin of the patients with pemphigus. However, C3 was found in the same sites in only three cases of pemphigus erythematosus. Immunofluorescent studies for IgG at the basal membrane were positive in only two cases of pemphigus erythematosus. Immunologic response of patients with pemphigus was similar to that of controls; however, it differed from that of patients with SLE.