fibrin and Dermatitis-Herpetiformis

Recently, high prevalence of cryofibrinogenaemia has been observed in plasma of untreated dermatitis herpetiformis (DH) patients, and the pathological IgA and TG3 deposits in the papillary dermis were found to co-localize with fibrin and fibrinogen.. To study the fibrinolytic potential in plasma of untreated, dapsone and or/gluten-free diet treated DH patients as well as the in vitro effect of dapsone on the fibrinolytic profile.. Plasma samples of 23 DH patients, 19 healthy subjects and 5 pemphigus vulgaris patients were investigated by a turbidimetric-clot lysis assay. Out of them 5 DH plasma samples representing different fibrinolytic parameters, and 3 healthy controls were selected for parallel fibrin clot preparation. The clot fibrin structure was examined by scanning electron microscopy (SEM), and the diameters of 900 fibrin fibres were determined in each clot.. A significantly prolonged clot lysis time was detected in untreated DH patients. The turbidity values of DH plasma clots indicated an altered fibrin structure that was also confirmed by SEM: significantly thicker fibrin fibers were observed in untreated, TG3 antibody positive DH patients compared to healthy controls, whereas the fiber diameters of dapsone-treated patients were similar or thinner than the control values. In line with the structural changes of fibrin, the fibrinolytic profile of 5 DH patients under dapsone treatment approached the control values.. This study revealed that the fibrinolytic potential was impaired in the plasma of untreated DH patients, whereas dapsone corrected the fibrinolytic defect. These data suggest a pathogenic role for plasma-derived factors in the development of skin symptoms and add a new aspect to the long-known beneficial, symptomatic effect of dapsone in active DH.

Topics: Adult; Aged; Blood Coagulation; Case-Control Studies; Cryoglobulinemia; Dapsone; Dermatitis Herpetiformis; Enzyme-Linked Immunosorbent Assay; Female; Fibrin; Fibrinogen; Fibrinolysis; Fluorescent Antibody Technique, Direct; Humans; Kinetics; Male; Microscopy, Electron, Scanning; Middle Aged; Nephelometry and Turbidimetry; Skin; Young Adult

Dermatitis herpetiformis has a characteristic histologic pattern consisting of subepidermal blisters often containing fibrin, infiltrates of neutrophils and nuclear dust at tips of dermal papillae, and papillary dermal edema. These are features of early and evolving lesions. We present two cases of clinically typical dermatitis herpetiformis with previously unreported histologic features that may provide a significant diagnostic clue. In each of these cases there were focal collections of nuclear dust in the cornified layer of the epidermis, a finding that may represent a resolving phase of dermatitis herpetiformis, beyond the usual papillary dermal neutrophilic microabscesses seen in early lesions. Biopsy material was available for immunofluorescent studies in one of the cases presented. In addition to the granular pattern of IgA positivity at the dermal-epidermal junction, which is diagnostic of dermatitis herpetiformis, this biopsy also showed similar IgA positivity in the intracorneal nuclear dust aggregates. In the second case, initial sections showed only intracorneal nuclear dust, but at deeper levels there were more typical diagnostic microabscesses at the tips of dermal papillae.

Topics: Abscess; Adult; Blister; Cell Nucleus; Dermatitis Herpetiformis; Edema; Epidermis; Female; Fibrin; Fibrinogen; Follow-Up Studies; Humans; Immunoglobulin A; Male; Middle Aged; Neutrophils; Skin

Skin lesions were produced by application of 50% potassium iodide to twelve patients with dermatitis herpetiformis (DH). Perivascular cellular infiltrates were found to be characteristic of developing lesions. The cells were mainly round cells; alpha-naphthyl acetate esterase staining revealed that in 24-h lesions the mean percentage of T-lymphocytes was 43%, that of mononuclear phagocytes 6% and that of non-T/non-M cells (mainly B-lymphocytes) 44%. The percentage of the latter was highest (mean 81%) in 6-h specimens, suggesting that these cells are participating in the early stages of lesion formation. The infiltrating cells in dermal papillae and within subepidermal vesicles were predominantly polymorphonuclear leukocytes (mean 86%) with some mononuclear phagocytes and non-T/non-M cells. Immunofluorescence examination confirmed that fibrin deposition is characteristic of the initial lesions of DH and showed that the same is true of fibronectin. Seven out of eight patients had fibronectin deposits in dermal papillae. IgA was found in all and C3 in most of the specimens and, with the exception of papillary vesicles and blister cavities, the intensity of IgA and C3 fluorescence showed no marked alterations during the development of lesions.

Topics: Complement C3; Dermatitis Herpetiformis; Female; Fibrin; Fibronectins; Fluorescent Antibody Technique; Humans; Immunoglobulin A; Leukocyte Count; Male; Potassium Iodide; Skin

Juvenile dermatitis herpetiformis occurred in a 20-month-old girl. She had granular lgA, C3, and fibrin bound to the basement membrane zone of the skin by direct immunofluorescence and negative serum antibodies against the skin on indirect immunofluorescence. The HLA typing of peripheral lymphocytes was A1, Aw30, B8, Bw51 without clinical evidence of malabsorption syndrome. A rapid improvement was observed on dapsone therapy. These findings strongly suggest that juvenile dermatitis hepetiformis is a disease entity different from chronic bullous dermatosis of childhood.

Topics: Basement Membrane; Complement C3; Dermatitis Herpetiformis; Female; Fibrin; Fluorescent Antibody Technique; HLA Antigens; Humans; Immunoglobulin A; Immunoglobulin G; Infant; Phenotype; Skin

Topics: Biopsy; Cytoplasm; Dermatitis Herpetiformis; Desmosomes; Erythema; Fibrin; Glycogen; Histocytochemistry; Humans; Immunoglobulins; Lipids; Microscopy, Electron; Skin

Topics: Basement Membrane; Biopsy; Blister; Dermatitis Herpetiformis; Fibrin; Humans; Microscopy, Electron

Topics: Blister; Dapsone; Dermatitis Herpetiformis; Diagnosis, Differential; Erythema Multiforme; Fibrin; Fluorescent Antibody Technique; Humans; Pemphigus; Skin; Staining and Labeling

Topics: Dermatitis Herpetiformis; Fibrin; Fluorescent Antibody Technique; Humans; Potassium Iodide; Staining and Labeling