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Trichophyton dermatophytosis--a disease easily confused with pemphigus erythematosus.

Trichophytosis is a rare diagnosis in dogs in Ontario, but recently 4 dogs with a scaling and crusting, alopecic, facial dermatitis have been so diagnosed. In all cases, the histopathological findings of severe epidermal and follicular interface dermatitis, accompanied by an acantholytic intraepidermal pustular dermatitis suggested an immune-mediated disease.

Animals↗

Use of tetracycline and niacinamide for treatment of autoimmune skin disease in 31 dogs.

A combination of niacinamide and tetracycline was used to treat 31 dogs with various autoimmune skin diseases (discoid lupus erythematosus, pemphigus foliaceus, pemphigus erythematosus, and bullous pemphigoid). Of the 20 dogs with discoid lupus erythematosus, 70% had excellent or good response to treatment. Serious side effects were not noticed in any dog.

Animals↗

Management of the immunobullous disorders. II. Pemphigus.

In the second of our reviews on the management of the immunobullous disorders, we review the therapy of pemphigus disorders, including pemphigus vulgaris, pemphigus vegetans, pemphigus foliaceus, pemphigus erythematosus, pemphigus herpetiformis, drug-induced pemphigus, IgA pemphigus and paraneoplastic pemphigus.

Adrenal Cortex Hormones↗

Investigation of antibodies to extractable nuclear antigens in dogs.

Determination of antibodies to specific nuclear antigens, termed extractable nuclear antigen (ENA), was investigated in healthy dogs and in dogs with autoimmune, inflammatory, and neoplastic diseases. Using a counterimmunoelectrophoresis method, the dogs' sera were tested for antibodies against the nuclear antigens single-stranded DNA, Sm, Ro, La, ribonucleoprotein, Scl, and proliferating cell nuclear antigen. Antibodies to the Ro antigen were found in 1 dog with discoid lupus erythematosus, in 1 dog with pemphigus erythematosus, and in 1 dog with facial pyoderma and chronic superficial keratitis. In 15 dogs, antibodies were detected to ENA, but the precipitin lines were too weak to identify the specific ENA. These antibodies were found in some dogs with systemic lupus erythematosus, discoid lupus erythematosus, pemphigus erythematosus, dermatomyositis, vitiligo, lymphoma; in the dog with facial pyoderma and chronic superficial keratitis; and in 1 healthy dog. The highest percentage of dogs with antibodies to ENA in a large series (greater than 8) of this study was in dogs with systemic lupus erythematosus (4 of 13; 31%).

Animals↗

[Comparative study of the development and prognosis of pemphigus vulgaris and seborrheic pemphigus].

Traditionally, the prognosis of pemphigus erythematosus is thought to be more favourable than that of pemphigus vulgaris. A retrospective study of the records of 10 patients with pemphigus erythematosus and 13 patients with pemphigus vulgaris was set up to compare the courses of the two diseases. This comparison, carried out in populations with similar age, sex ratio, pretreatment duration of the disease and treatment received, showed that relapses were more frequent in the course of pemphigus erythematosus, whereas remissions, mean duration of the disease and iatrogenic complications were the same in both groups. This study, therefore, throws some doubts on the dogma of relative mildness of pemphigus erythematosus, which goes back to a period long before systemic corticosteroid therapy was known. Mortality studies performed since the event of this treatment have shown that the prognosis had improved and tended to be the same in both diseases. The other data concerning the course of treated pemphigus erythematosus are little known and were never compared with those concerning pemphigus vulgaris. Our study shows that treatments similar to those of pemphigus vulgaris are necessary to obtain remissions in pemphigus erythematosus and that these diseases share the same evolutive profile. However, the question of the best therapeutic strategy to be used has not yet been answered.

Adult↗

230-kDa and 190-kDa proteins in addition to desmoglein 1 as immunological targets in a subset of pemphigus foliaceus with a combined cell-surface and basement membrane zone immune staining pattern.

Pemphigus erythematosus, initially described as a combination of pemphigus with lupus erythematosus, and pemphigus foliaceus are now frequently considered localized and generalized variants of superficial pemphigus. Yet diagnostic criteria for pemphigus erythematosus remain controversial. Distinct from pemphigus foliaceus, pemphigus erythematosus displays immune depositions at the dermal-epidermal junction, which suggests additional immunopathological mechanisms. We present three patients with clinical and histopathologic signs of superficial pemphigus, who all exhibited an immunomorphology characteristic of pemphigus erythematosus. Complement depositions in a granular-linear fashion were consistently found at the dermal-epidermal junction besides in vivo bound and circulating antikeratinocyte cell-surface autoantibodies. Histopathology showed subcorneal acantholysis, and all sera contained antidesmoglein 1 but not antidesmoglein 3 autoantibodies detected by enzyme-linked immunosorbent assays (ELISA). Additional autoantibodies against a 230-kDa protein and against a 190-kDa protein comigrating with bullous pemphigoid antigen 1 (BP230) and periplakin, respectively, were present in all the patients' sera. As two sera specifically reacted with BP230 by ELISA, the presence of BP230-specific autoantibodies could be associated with dermal-epidermal immune staining in these patients. In pemphigus erythematosus, dermal-epidermal immune staining is generally attributed to the deposition of immune complexes, while the presence of BP230-specific autoantibodies has not been reported in this disease previously. Perhaps, the unique autoantibody profile of the patients in the study permits discrimination between patients with superficial pemphigus that display additional dermal-epidermal immune staining from those with conventional pemphigus foliaceus on a molecular basis. Further studies will be required to substantiate the frequency of this occurrence and to unravel its pathogenic significance.

Adult↗

Pemphigus foliaceus: a case with serologic features of Senear-Usher syndrome and other autoimmune abnormalities.

The two variants of superficial pemphigus, pemphigus erythematosus (Senear-Usher syndrome) and pemphigus foliaceus, share common histopathologic and indirect immunofluorescence findings. They differ in that pemphigus erythematosus is limited in distribution and usually has concomitant basement membrane zone deposition of immunoglobulin and complement in lesional skin in addition to intercellular space staining in the epidermis, whereas pemphigus foliaceus is a generalized process that reputedly lacks basement membrane zone staining. A 49-year-old man with an array of immunologic abnormalities, including pernicious anemia and Sjögren's syndrome consistent with broad immunologic dysfunction, developed generalized superficial pemphigus (pemphigus foliaceus) with concomitant basement membrane zone immunoreactant deposition. The demonstration of basement zone immunofluorescent staining lends additional support to the basic viewpoint that pemphigus erythematosus and pemphigus foliaceus are variable phenotypic expressions of the same basic disease process.

Autoimmune Diseases↗

Visken as supplementary drug in the treatment of pemphigus.

Results of combined treatment with low doses of steroids (50-10-5 mg) and Visken (7.5-15 mg) in 5 cases of pemphigus erythematosus and 5 cases of pemphigus vulgaris are presented. Treatment lasted from 30 days to 1.5 years. Some of the patients had been previously treated with steroids and immunosuppressive drugs, but had active lesions or frequent recurrences. In pemphigus erythematosus, very good results were obtained in 3 of 5 cases, and failure in 2 cases was probably related to insufficient dosage of steroids, or no treatment with steroids at all. In pemphigus vulgaris results were not satisfactory. Visken can serve as a supplementary drug in the treatment of pemphigus erythematosus. No complications were observed, and the drug may be regarded as safe.

Adrenal Cortex Hormones↗

The men behind the eponym: Francis E. Senear, Barney Usher, and the Senear-Usher syndrome.

This historical review will summarize the report of Francis Senear and Barney Usher on a disease that they called pemphigus erythematodes and which later became pemphigus erythematosus (the Senear-Usher syndrome). It will then outline the lives of these two men. Finally, it will review the literature on that condition and relate the views of various authorities as to whether pemphigus erythematosus is merely pemphigus foliaceus, a variant of pemphigus foliaceus, a syndrome combining features of lupus erythematosus and pemphigus or whether pemphigus erythematosus (Senear-Usher syndrome) is a distinct and separate disease.

Dermatology↗

[Pemphigus. Loss of desmosomal cell-cell contact].

Pemphigus diseases comprise a group of autoimmune disorders which are characterized by intraepidermal blisters and autoantibodies to components of desmosomes. Desmosomes mediate adhesion between neighbouring keratinocytes. A common feature of pemphigus diseases are intercellular deposits of IgG or, less frequently, of IgA within the epidermis. The group of pemphigus diseases includes pemphigus vulgaris, pemphigus foliaceus, pemphigus vegetans, pemphigus herpetiformis, pemphigus erythematosus, paraneoplastic pemphigus, drug-induced pemphigus, and IgA pemphigus. Using molecular tools, some of the autoantigens in these diseases have been characterized. In pemphigus vulgaris, autoantibodies are directed to desmoglein 3 and in pemphigus foliaceus to desmoglein 1. Target antigens in IgA pemphigus are desmocollin 1 and desmoglein 3. In paraneoplastic pemphigus, autoantibodies react with a complex of various proteins, including desmoplakin 1 and 2, BP230, envoplakin, periplakin, plectin, desmoglein 3, and a yet uncharacterized 170 kD protein. This review summarizes new insights into the immunopathogenesis and diagnosis of pemphigus diseases.

Animals↗