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G Kirtschig

Publications and source records attributed to G Kirtschig.

44 records · Page 3Linked to original sources
PubMed1994

Molecular overlap of the IgA target antigens in the subepidermal blistering diseases.

Linear IgA disease (LAD) has circulating IgA auto-antibodies which have been found to be associated with epidermal- and dermal-derived antigens. The localization and molecular weight(s) of the IgA target antigen(s) implicated in LAD and their relationship to the target antigens for the IgA antibodies in bullous pemphigoid (BP) and cicatricial pemphigoid (CP) are unknown. Sera from patients with all 3 diseases who had circulating IgA antibodies were studied by immunoblotting with both epidermal and dermal extracts. Our findings indicate that there is heterogeneity in the localization and molecular weights of the target antigen(s), with multiple bands demonstrated with some sera. Three principal target antigens were identified with molecular weights of 110-120, 160-180 and one approximately 285 kD. These antigens were common to all 3 diseases and were found in 25 patients. These results suggest that the target antigens for IgA basement membrane zone antibodies in LAD, BP and CP are multiple, complex in composition and may differ from the conventional BP antigens.

Autoantibodies↗
PubMed1993

Bullous eruption of systemic lupus erythematosus: a clinicopathological study of four cases.

We describe the clinical, histological and immunopathological features of four female patients with the bullous eruption of systemic lupus erythematosus (bullous SLE). Three patients had circulating anti-basement membrane zone (BMZ) antibodies, and Western blot analysis in two cases revealed binding to type VII collagen. Immunoelectron microscopy in one of these patients demonstrated deposition of antibody in the lamina densa and sublamina densa regions, thus sharing immunopathological features with epidermolysis bullosa acquisita (EBA). The vesiculobullous lesions developed 8 months to 6 years after the initial symptoms of SLE, and cleared promptly with dapsone treatment.

Adult↗
PubMed1992

The distribution of alpha 6 beta 4 integrins in lesional and non-lesional skin in bullous pemphigoid.

The alpha 6 beta 4 integrin is associated ultrastructurally with the hemidesmosomes of the basal keratinocytes and with the bullous pemphigoid antigen (BPA), suggesting an important role in adhesion of epidermal cells to the basement membrane. Using an immunofluorescence technique with chain-specific monoclonal antibodies to the alpha and beta subunits we have investigated the distribution of the alpha 6 beta 4 integrin in normal skin (n = 3) and in BP skin (uninvolved, perilesional and lesional) [n = 11]). The findings have been compared with other types of subepidermal blisters and with normal skin split by chemical means (n = 2) and by suction (n = 2). The distribution of alpha 6 beta 4 integrin was compared with that of bullous pemphigoid antigen (BPA) and with other basement membrane zone (BMZ) macromolecules, laminin, collagen type IV, collagen type VII and the BM600 antigen. In uninvolved, perilesional and early pre-blistered lesional BP skin the distribution of both the alpha 6 and beta 4 integrin subunits, BPA laminin, collagen types IV and VII and the BM600 antigen was identical to normal skin, i.e. a linear band in the BMZ. Within BP blisters, both alpha 6 and beta 4 integrin subunits and BPA were absent, except in two blisters in which the integrin expression was retained in the blister roof, despite loss of BPA. The other BMZ components were expressed on the blister floor.(ABSTRACT TRUNCATED AT 250 WORDS)

Antibodies, Monoclonal↗
PubMed1992

[Leukonychia longitudinalis as the primary symptom of Hailey-Hailey disease].

Longitudinal white bands on the fingernails were observed in a 58-year-old male patient with perianal intertrigo. His nail changes were a first clue to the diagnosis of Hailey-Hailey disease, which was later confirmed histopathologically. Similar nail abnormalities were found in the patient's son who was also affected with Hailey-Hailey disease. These findings support the concept that those nail changes, first described by Burge in 1991, constitute a useful diagnostic marker in Hailey-Hailey disease.

Biopsy↗
PubMedDate not supplied

Bullous pemphigoid and epidermolysis bullosa acquisita: presentation, prognosis, and immunopathology in 11 children.

The immunobullous diseases bullous pemphigoid (BP) and epidermolysis bullosa acquisita (EBA) are very rare in childhood. Although case studies have been detailed, there are no reports of a large series of patients documenting the effectiveness of treatment and long-term prognosis. We report the clinical presentation, immunopathologic features, disease course, and long-term prognosis of BP and EBA in a series of 11 children. The initial diagnoses based on clinical features were BP (5), EBA (3), and chronic bullous disease of childhood (CBDC) (3). These were subsequently revised from BP to EBA (2), CBDC to BP (2), and CBDC to BP or EBA (1) following the results of direct and indirect immunofluorescence and immunoblotting. Analysis of IgG subclasses in eight cases showed that the predominant subclasses were IgG1 (8) and IgG4 (6). The clinical features appeared to be highly variable, and in patients presenting with inflammatory blistering, laboratory studies were required in order to differentiate between BP and EBA. All patients improved on treatment with corticosteroids and/or sulfones, although treatment regimens showed wide variation. Their diseases tended to remit within 2 years, and their long-term prognosis was good.

Adolescent↗