Pemphigoid vegetans represents a bullous pemphigoid variant

Chan, Lawrence S.; Dorman, Michael A.; Agha, Amr; Suzuki, Tadashi; Cooper, Kevin D.; Hashimoto, Ken

doi:10.1016/0190-9622(93)70048-x

Cited by 27 publications

(9 citation statements)

References 10 publications

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“…The association of autoantibodies and complement does not always occur. In a case of pemphigous vegitans IgG4 (a comparatively ineffective complement activator) was localized to the BMZ in the absence of an C3 deposits [6]. It is noted that in this case of pemphigous vegetans a diffuse eosinophilia was present.…”

mentioning

confidence: 78%

“…It is clear that pemphigus and the pemphigoid diseases represent a complex array of conditions, and differentiating between the many bullous variants (termed pemphigoid as they are pemphigus-like syndromes) can present difficulties. However, conditions such as epidermolysis bullosa aquisita [5], cicatricial pemphigoid [2], and pemphigoid vegetans [6], have been distinguished. The occurrence in vivo of bound and circulating autoantibodies to basement membrane in these diseases prompts a number of questions.…”

mentioning

confidence: 99%

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Mechanisms of epithelial damage: are there parallels between bullous skin diseases and asthma?

Dhami

Shute

1994

Clin Experimental Allergy

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confidence: 78%

mentioning

confidence: 99%

Mechanisms of epithelial damage: are there parallels between bullous skin diseases and asthma?

Dhami

Shute

1994

Clin Experimental Allergy

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“…The age of presentation ranged from 9 to 89 years. 1 – 13 All patients presented with intertriginous vegetative lesions, mainly in the inguinal area ( Table 1 ). Some had mucosal lesions ( n = 8) and a few had vesicles or pustules ( n = 7 vs 6).…”

Section: Discussionmentioning

confidence: 99%

“…In our case, a linear IgA deposit at the dermo-epidermal junction was found by direct immunofluorescence, as reported by Ogasawara et al 11 Indirect immunofluorescence was positive in nine cases. 1 – 3 , 5 , 6 , 8 , 10 , 12 , 13 Immunoblotting analysis has been reported in five cases. 3 , 5 , 6 , 10 , 12 BP230 autoantibodies were positive in every case, and three cases had BP180 autoantibodies.…”

Section: Discussionmentioning

confidence: 99%

Management of pemphigoid vegetans with mycophenolate mofetil: A case report

Mathieu,

Chababi-Atallah,

Allard-Chamard

et al. 2024

SAGE Open Medical Case Reports

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Pemphigoid vegetans is a rare variant of bullous pemphigoid. Only 13 cases have been documented in the medical literature. The lesions clinically resemble pemphigus vegetans, but the histology is consistent with bullous pemphigoid. We present the case of a 63-year-old woman with painful vesicular and vegetative lesions progressing for 4 months. Histopathology showed a dermal-epidermal cleavage, epidermal acanthosis and an eosinophilic infiltrate. Direct immunofluorescence showed linear deposition of IgG and IgA at the junctional level. The diagnosis of pemphigoid vegetans was retained and successfully treated with oral corticosteroid, dapsone and mycophenolate mofetil.

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“…There are several distinct clinical presentations of BP, which have so far been described: generalized bullous form, vegetative form [19,20], vesicular form [21], generalized erythrodermic form [22,23], urticarial form, localized (usually pretibial) form [2,24] and nodular form [25,26,27,28]. …”

Section: Introductionmentioning

confidence: 99%

Ecthyma-Gangrenosum-Like Bullous Pemphigoid

et al. 2010

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Bullous pemphigoid (BP) is a chronic, autoimmune, subepidermal blistering skin disease with varied clinical presentations. Diagnosis is based on the clinical picture, histopathological findings, and direct and indirect immunofluorescence studies. In unclear cases, ELISA or Western blot analysis helps to establish a definite diagnosis by the detection of immunoglobulin G autoantibodies specific for the hemidesmosomal BP antigens BP230 and BP180. We report 3 cases of BP with an as yet not characterized, distinctive ecthyma-gangrenosum-like presentation. Patients were female, above 80 years of age, physically immobile, and skin lesions showed truncal localization and bacterial colonization. Factors contributing to physical immobility were a high body mass index, psychiatric disease, sedative medication and rheumatic disease. The clinical picture resembled ecthyma gangrenosum but lacked systemic infection with Pseudomonas aeruginosa. Lesional bacteriological studies revealed Staphylococcusaureus and/or P. aeruginosa. Diagnosis proved challenging in all cases. Suspicion has to be high, and repeated diagnostic procedures and additional laboratory studies may be necessary to establish a definitive diagnosis of BP. In summary, we propose this combination of truncal ecthyma-gangrenosum-like lesions with bacterial colonization in the context of older age and immobility as a clinically distinct presentation or variant of BP.

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Pemphigoid vegetans represents a bullous pemphigoid variant

Cited by 27 publications

References 10 publications

Mechanisms of epithelial damage: are there parallels between bullous skin diseases and asthma?

Mechanisms of epithelial damage: are there parallels between bullous skin diseases and asthma?

Management of pemphigoid vegetans with mycophenolate mofetil: A case report

Ecthyma-Gangrenosum-Like Bullous Pemphigoid

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