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PubMed1980

Disorder of cellular immunity in pemphigus vegetans.

In an untreated 26-year-old female patient suffering from Neumann's pemphigus vegetans for 8 months (with neither preceding thymoma nor myasthenia gravis), several in vivo and in vitro phenomena of immunodeficiency involving both T and B cell system were disclosed. A diagnosis of defective immune state was established on the basis of the low IgG-serum level (though pemphigus antibodies were present), reduced T cell count in blood, weak PHA stimulation of lymphocytes, lack of skin reactivity to recall antigens and to most other bacterial antigens tested. Impaired chemotaxis and a deficient intracellular killing of Candida albicans by PMNL were also found. The conclusion is drawn that a combined disorder of both the B and T cell system is involved in the etiology of pemphigus vegetans, even when thymoma is ruled out.

Adult↗
PubMed1984

Pemphigus vegetans. Neumann type and Hallopeau type.

Two cases of pemphigus vegetans are presented, one of the Neumann type and one of the Hallopeau type. The differences between the two subsets are clinical presentation and course. Patients with the Hallopeau type often have a relatively benign disease, require lower doses of systemic corticosteroids, and usually have a prolonged remission. Patients with the Neumann type have a course similar to pemphigus vulgaris, need higher doses of systemic corticosteroids, and have relapses and remissions. The histologic findings in the vegetating lesions are similar in both types. The immunopathologic features of both types are indistinguishable and similar to pemphigus vulgaris. IgM staining of eosinophilic microabscesses was observed in one case.

Abscess↗
PubMed1986

Charcot-Leyden crystals in pemphigus vegetans.

In the skin lesion of a patient with pemphigus vegetans, Charcot-Leyden crystals were observed in the eosinophilic abscess cavities of the epidermis. The finding is documented for the first time. In addition, some of the intraepidermal eosinophilic abscess cavities have been shown to be due to accumulation of eosinophils in dilated acrosyringia, but their formation from acantholysis and spongiosis can not be entirely excluded.

Acantholysis↗
PubMed1992

Captopril-induced pemphigus vegetans with Charcot-Leyden crystals.

A 78-year-old woman developed intertriginous vegetating plaques, mouth ulcers, and a cerebriform tongue after 11 months of captopril therapy. Findings of clinical, histologic, and immunofluorescence testing were consistent with a diagnosis of pemphigus vegetans. In addition, Charcot-Leyden crystals were observed within some of the intraepidermal, eosinophilic abscess cavities, which were bordered by granular cells undergoing keratinization. The skin lesions cleared rapidly after discontinuation of captopril. To the best of our knowledge, this report describes the first case of pemphigus vegetans induced by captopril.

Aged↗
PubMedDate not supplied

Pemphigus vegetans presenting as acrodermatitis continua suppurativa.

The authors present a 51-year-old patient with clinical signs of acrodermatitis continua suppurativa of the toes. Since histological findings failed to unambiguously support the diagnosis of acrodermatitis continua suppurativa, immunohistology was performed. The results showed intercellular epidermal fluorescence. The subsequent clinical picture (vegetating formation), the repeated histological examination (suprabasal cleft and eosinophilia), as well as the data of immunohistological findings supported the diagnosis of pemphigus vegetans Hallopeau. The patient was treated successfully with a combination of cyclosporine-A and low-dose steroid therapy. The case is of special interest, because there is only a single publication about phalangeal and nail involvement in Hallopeau's type pemphigus vegetans in the dermatological literature according to our knowledge.

Acrodermatitis↗
PubMed1998

Pemphigus vegetans involving the esophagus.

A 44-year-old man with pemphigus vegetans had severe odynophagia. He received an endoscopic examination for esophageal involvement. Many white plaque-like lesions with an erythematous base were seen on the esophageal mucosa. Biopsy from the mucosal epithelial layer showed rounded epidermal cells with large nuclei and numerous inflammatory cells including eosinophils.

Adult↗
PubMed1988

Epidermal antigens in pemphigus vegetans. Report of a case.

A patient is described who had typical pemphigus vegetans, according to clinical, histopathological and immunofluorescence criteria. To determine the specific molecules bound by antibodies in the serum of this patient, an immunoprecipitation analysis was performed with extracts of [14C]-amino acid labelled cultured normal human keratinocytes. The patient's serum precipitated the 130 kd and 85 kd polypeptides of the pemphigus vulgaris antigen, but also precipitated other polypeptides, the significance of which is not clear.

Antibody Specificity↗
PubMed2004

Paraneoplastic pemphigus secondary to fludarabine evolving into unusual oral pemphigus vegetans.

We report a patient with chronic lymphocytic leukaemia who developed paraneoplastic pemphigus (PNP) soon after the initiation of fludarabine therapy. He presented with severe oral and cutaneous erosions. Initially, he had high titres of circulating autoantibodies as detected by indirect immunofluorescence (IF) on multiple epithelial substrates (normal human skin, monkey oesophagus, and rat bladder) and by desmoglein 1 and 3 enzyme-linked immunosorbent assays (ELISAs). His oral erosions have subsequently progressed into unusual hyperplastic papillomatous lesions affecting the inner aspect of lips and buccal mucosae, histologically consistent with pemphigus vegetans. Desmoglein 1 antibodies and IF on rat bladder substrate have become negative after 18 months of therapy. Several agents had been initiated to bring the disease under control originally, but a partial remission was achieved and maintained with mycophenolate mofetil and low-dose prednisolone.

Aged↗
PubMed1977

Pemphigus vegetans of Hallopeau: immunofluorescent studies.

Immunofluorescent techniques were used in the study of tissue from a patient with pemphigus vegetans of Hallopeau. The findings were typical of pemphigus, confirming the propriety of classifying this entity as a benign variant of pemphigus, rather than a disease of purely microbial etiology. The question is raised whether other types of vegetating pyodermas actually are separate diseases or represent a spectrum of pemphigus with the clinical appearance dependent on the patient's immunity to his disease. Immunofluorescent studies are proposed as a basis for classification.

Aged↗
PubMed1985

[Pemphigus vegetans (Hallopeau) with eosinophilic spongiosis--successful retinoid therapy].

We present an unusual case of pemphigus vegetans (Hallopeau) with widespread, nonblistering gyrate erythematous lesions on the trunk and extremities in addition to typical vegetating lesions in the inguinocrural area. The histopathology which was characterized by eosinophilic spongiosis, as well as immunofluorescence and immunoelectron-microscopic studies indicated that the gyrate erythema (characterized by eosinophilic spongiosis) seen in our patient represented a manifestation of pemphigus. Treatment with retinoids resulted in the prompt disappearance of the gyrate erythemas, and the eosinophils returned to normal. A short-term, low-dose corticosteroid regimen also led to rapid healing of vegetating lesions.

Aged↗
PubMed1980

Pemphigus vegetans: its relationship to eosinophilic spongiosis and favorable response to dapsone.

Two cases of pemphigus vegetans are presented. One, an example of the Hallopeau type of the disease, was found to have early lesions that simulated eosinophilic spongiosis clinically and histologically. The other had lesions that were largely confined to the oral mucosa and was also considered to be the Hallopeau type of the disease in a limited form. The cases of these two patients were compared with a typical case of eosinophilic spongiosis. All were controlled with dapsone therapy.

Dapsone↗
PubMed1993

[Neumann type pemphigus vegetans with fatal outcome].

We report on a 45-year-old patient, who first developed pemphigus vegetans of the Neumann type 9 years ago and who presented with typical manifestations of the disease 6 years after the first exacerbation. On systemic therapy with corticosteroids progression of the disease ceased. Additional application of azathioprine, cyclophosphamide or gold thiomalate, however, did not make it possible to reduce the high corticosteroid dosage without exacerbation of the disease. The long-term corticosteroid therapy led to sepsis, thrombosis of the leg and pelvic veins and osteoporosis with crush fractures of the vertebrae; corticosteroid therapy was therefore discontinued and replaced by cyclophosphamide bolus therapy associated with gold thiomalate. The subsequent exacerbation of the pemphigus was treated with methotrexate and dapsone. The skin lesions progressed further, however, and the patient died of sepsis 39 weeks after admission to the clinic.

Drug Therapy, Combination↗
PubMed1993

Epidermal antigens and complement-binding anti-intercellular antibodies in pemphigus vegetans, Hallopeau type.

The serum of a 34-year-old woman with the Hallopeau type of pemphigus vegetans (PVg) contained antibodies against a 130-kDa polypeptide in human epidermal lysates, as revealed by Western blot analysis. The serum strongly fixed complement in vitro, and the PVg lesional skin contained a predominance of complement-fixing IgG2 and IgG4. Although the antigens reactive with sera from PVg and pemphigus vulgaris were the same, strong fixation of complement by PVg antibodies, due to the presence of complement-dependent IgG subclasses, and subsequent in situ activation of complement, might explain the marked infiltration of neutrophils and eosinophils in PVg.

Adult↗
PubMed1981

An unusual form of pemphigus vegetans.

Numerous vegetating plaques developed in a 63-year-old man followed up for the management of pemphigus vulgaris of 5 years' duration. These lesions occurred over the trunk and right upper arm with no involvement in the intertriginous area, and were controlled with systemic steroid and ODT. Direct immunofluorescence test showed heavy deposition of fibrinogen in the papillary dermis. To our knowledge, the occurrence of pemphigus vegetans in the non-intertriginous area is rare.

Fibrinogen↗
PubMed1989

Pemphigus vegetans presenting as scrotal tongue.

A case of scrotal tongue is described. Histological and immunofluorescent findings revealed that this case was pemphigus vegetans, which should therefore be added to the differential diagnosis of scrotal tongue.

Diagnosis, Differential↗
PubMed1983

Unusual immunofluorescence findings in pemphigus vegetans.

An 84-year-old man with massive verrucous lesions in the intertriginous areas is reported. The clinical, histological and electron microscopical findings indicated pemphigus vegetans. However, in addition to the intraepidermal findings direct immunofluorescence showed a linear deposit of IgG and C3 at the basement membrane.

Aged↗