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Cutaneous neosporosis during treatment of pemphigus foliaceus in a dog.

A 4-year-old, intact male rottweiler was presented with a 10-day history of papulonodular dermatitis. At the time of presentation, the dog was receiving prednisone and azathioprine to treat pemphigus foliaceus. Cutaneous neosporosis was diagnosed by immunohistochemistry on skin biopsy specimens and a high serum antibody titer to Neospora caninum by Neospora agglutination test. Electron microscopy examination of skin specimens further supported the diagnosis. Clindamycin therapy, together with withdrawal of immunosuppressive medication, resulted in prolonged clinical remission. This report documents cutaneous neosporosis in an adult dog and suggests that immunosuppressive therapy might be a predisposing factor.

Animals↗

Pemphigus foliaceus and oral lichen planus in a patient with systemic lupus erythematosus and thymoma.

A 49-year-old woman with systemic lupus erythematosus, and a history of thymoma and chronic white plaques of the oral mucosa, developed a blistering eruption which was diagnosed as pemphigus foliaceus based on clinical, histological and direct immunofluorescence findings. However, the white plaques had both the clinical and histological features of lichen planus, as well as intercellular antibodies suggestive of pemphigus. Such conditions in combination have been documented previously, but this is believed to be the first report of the coexistence of all four in one individual. The common mechanism underlying these four conditions may well be an immunological disturbance.

Female↗

IgG subclasses of intercellular antibodies in Brazilian pemphigus foliaceus--the relationship to complement fixing capability.

Human IgG possesses four main subclasses, namely IgG1, IgG2, IgG3, and IgG4, of these IgG1-IgG3 fix complement, but IgG4 does not. We have studied the IgG subclasses of intercellular antibodies in the sera from 20 patients with Brazilian pemphigus foliaceus by immunofluorescent staining using mouse monoclonal antibodies against human IgG1-IgG4. At the same time, the complement fixing capability of each antibody was examined by complement immunofluorescence. All of four subclasses were frequently detected in most cases with varying distributions. However, no specific pattern was observed. Complement fixing antibodies were found in four patients. However, the distribution of IgG subclasses was incompatible with their known characteristics in terms of complement activation. This discrepancy increases the controversy over the importance of the complement system in blister formation in pemphigus.

Brazil↗

A case of drug-induced hypersensitivity syndrome showing transient immunosuppression before viral reactivation during treatment for pemphigus foliaceus.

Drug-induced hypersensitivity syndrome (DIHS) is one of the most severe drug adverse reactions, with characteristic biphasic symptoms. Reactivation of human herpesvirus-6 (HHV-6) is frequently observed, although the cause of DIHS is still unknown. A patient developed DIHS during treatment with diaminodiphenylsulphone for pemphigus foliaceus. The number of lymphocytes in his peripheral blood, and titres of serum total IgG and IgM and anti-desmoglein1 antibody transiently decreased just before reactivation of HHV-6, cytomegalovirus and Epstein-Barr virus. This observation suggests that transient suppression of both cellular and humoral immunity may trigger viral reactivation, which leads to the development of the second phase of DIHS.

Administration, Oral↗

Two patients with unusual skin lesions and circulating antikeratinocyte cell surface antibodies: detection of antibodies to the intracellular domain of the pemphigus foliaceus antigen (desmoglein) by studies using fusion proteins.

We report two patients with unusual skin lesions, who had antikeratinocyte cell surface IgG autoantibodies in their sera. Immunoblot analysis of both a human epidermal extract and a bovine desmosome preparation revealed that the sera of both patients reacted exclusively with the 160-kDa pemphigus foliaceus antigen (desmoglein). We further investigated the antigen molecule using bacterial fusion proteins produced by using bovine desmoglein cDNA, and found that both the sera reacted strongly and exclusively with the intracellular domain of the desmoglein. These results suggest that production of antidesmoglein autoantibodies in the present cases may be an epiphenomenon associated with damage to the keratinocyte cell membrane.

Aged↗

Pemphigus foliaceus resembling eruptive seborrheic keratoses.

A 78-year-old man had an acute eruption of lesions that clinically resembled seborrheic keratoses. Microscopic examination of the lesions indicated acantholysis and dyskeratosis primarily in the granular layer. The patient's serum contained antibodies to intercellular substance. Direct immunofluorescence microscopy of a lesion demonstrated immunoglobulin deposition in the intercellular areas. Treatment with oral prednisone produced rapid resolution of the lesions. Patients in whom eruptive seborrheic keratoses (Leser-Trélat sign) develop should have histological examination of their lesions to rule out the diagnosis of pemphigus foliaceus.

Aged↗

Pemphigus foliaceus of the footpads in three dogs.

Severe hyperkeratinization and villous hypertrophy of the footpads were seen in 3 middle-aged dogs. Peeling, fissuring, swelling, and ulcerations were noted on the margins of severely affected pads. Pain was evident in palpation and ambulation. Lesions were compatible with the traditional diagnosis of "hard pad disease". Histopathologic findings were diagnostic for canine pemphigus foliaceus in all 3 dogs, and direct immunofluorescence in an intercellular pattern was seen in both dogs that were tested. All 3 dogs responded to immunosuppressive dosages of corticosteroids.

Animals↗

Pemphigus foliaceus with typical histological and immunohistological findings in a dog.

A seven-year-old female mongrel dog showed incurable skin lesions for about a year. The lesions were at nasal bridge and periocular area, and were composed of crust formation and scaling. Biopsy specimens from the skin lesions possessed with multiple intraepidermal pustules containing many acatholytic keratinocytes. Direct immunoperoxidase stain using paraffin-embedded section showed IgG deposition at the intercellular area of upper epidermis and follicular infundibula. IgG was not detected at basement membrane zone. Clinical and pathological findings of the present case were identical to those of pemphigus foliaceus, an uncommon pustular autoimmune skin disease.

Animals↗

Structure of DSG1, the bovine desmosomal cadherin gene encoding the pemphigus foliaceus antigen. Evidence of polymorphism.

The cadherin superfamily of calcium-dependent cell-cell adhesion and recognition proteins can be categorized into a number of subsets on the basis of the distinct cytoplasmic sequences of their members. Currently these families include classical cadherins, desmogleins, desmocollins, protocadherins, and the products of the Drosophila genes FAT and Dachsous. Dsg1, the prototype of the desmoglein family, is a major component of epidermal desmosomes and the antigenic target of antibodies found in the sera of patients with the blistering disease, pemphigus foliaceus. In this study, we determined the organization of the bovine DSG1 gene. This gene consists of 15 exons distributed over > 37.5 kilobases of genomic DNA. A comparison of DSG1 with genes encoding classical cadherins revealed a striking conservation of exon boundaries in regions encoding the ectodomain and to a more limited extent among those encoding the cytoplasmic domain. Polymorphism was found in a sequence of DSG1 encoding protein proximal to the external face of the plasma membrane. This region is topologically equivalent to a domain of classical cadherins that harbors epitopes recognized by adhesion-disrupting antibodies. We discuss these results with regard to the evolution of the cadherin superfamily and their implications for the definition of pemphigus epitopes.

Animals↗

Pemphigus foliaceus in a goat.

A 7-year-old-female goat was referred with a 3-month history of chronic dermatitis, which partially responded to combined corticosteroid and antibiotic therapy. At dermatological examination diffuse alopecia, pustules and crusts were observed on the head, neck, dorsum and perianal area. Dermatophyte culture and skin scrapings were negative. Trichoscopic examination revealed a concurrent infestation with Damalinia caprae, which was successfully treated with ivermectin. Cytological examination of pus from intact pustules revealed nondegenerate neutrophils, absence of bacteria and numerous nucleated, irregularly shaped keratinocytes. Histopathological examination of lesional skin revealed intracorneal pustules containing neutrophils and acantholytic cells, and a mixed cell superficial perivascular dermatitis. Immunohistochemical stains of lesional skin showed intercellular IgG deposits in the spinous layer. Remission of the dermatitis was obtained with injectable dexamethasone-21-isonicotinate, every two months for one year. This is the first report to describe the cytological appearance of impression smears from intact pustules of pemphigus foliaceus in a goat and to document the presence of IgG deposits in lesional skin by means of immunohistochemistry.

Alopecia↗