Epidemiologic analysis and clinical course of 84 consecutive cases of pemphigus in eastern Sicily

Micali, Giuseppe; Musumeci, M L; Nasca, Maria Rita

doi:10.1046/j.1365-4362.1998.00428.x

Cited by 83 publications

(98 citation statements)

References 6 publications

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“…There are several large series of PV patients in the literature, where the involvement of PV is limited only to the skin. The incidence of such patients has varied from 8 to 42% [34, 35, 36, 37, 38]. Furthermore, the clinical characteristics of all of our patients are similar to other patients described previously in the literature, as having dual features of BP and PV [9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19].…”

Section: Discussionsupporting

confidence: 74%

Diagnostic Features of Pemphigus vulgaris in Patients with Bullous Pemphigoid

Sami

Bhol²,

Beutner³

et al. 2002

Dermatology

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Background: The simultaneous presence of features of pemphigus vulgaris (PV) in patients with bullous pemphigoid (BP) has previously been reported in the literature. Objective: The purpose of this retrospective study is to present 13 patients with an initial diagnosis of BP, who subsequently demonstrated coexistent serological features of both BP and PV. Methods: The following information on each patient was documented, at the time of initial diagnosis: clinical profile on presentation, histology, direct immunofluorescence, indirect immunofluorescence (IIF) using monkey esophagus as substrate, salt-split skin (SSS) and an immunoblot assay. Since all 13 patients failed to respond to conventional systemic therapy, intravenous immunoglobulin (IVIg) was used as an alternative treatment modality. Prior to initiating IVIg therapy, in all 13 patients, serological studies were performed. In addition to IIF using monkey esophagus, an immunoblot assay and SSS, an enzyme-linked immunosorbent assay (ELISA) was performed to detect antibodies to desmogleins. These different assays were done to identify pathological autoantibodies typical of BP and PV. A control group of 25 healthy normal individuals, 37 patients with BP, 17 patients with PV and 12 patients with pemphigus foliaceus were used for comparison of serological studies. Results: At the time of initial presentation, histological and immunopathological studies confirmed the diagnosis of BP in all 13 patients. Prior to the initiation of IVIg therapy, results of IIF using monkey esophagus as substrate demonstrated high levels of anti-intercellular cement substance (anti-ICS) or antikeratinocyte cell surface antibody. Sera of all 13 patients on SSS bound to the epidermal side of the split. In an immunoblot, using bovine gingival lysate as substrate, sera of 6 patients bound to both a 230-kD (BP Ag1) and 180-kD protein (BP Ag2), while 7 sera bound to only a 230-kD protein. All 13 patients had high levels of antibodies to desmoglein 3 on ELISA. In a pilot experiment, the anti-ICS antibody in sera from 6 random patients was found to be predominantly of the IgG4 subclass. Use of IVIg resulted in an effective clinical response and the maintenance of a prolonged clinical remission. Conclusion: In patients with BP, who are nonresponsive to conventional therapy, the presence of two autoimmune diseases or a dual diagnosis should be considered.

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Section: Discussionsupporting

confidence: 74%

Diagnostic Features of Pemphigus vulgaris in Patients with Bullous Pemphigoid

Sami

Bhol²,

Beutner³

et al. 2002

Dermatology

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“…In the Mediterranean regions, pemphigus vulgaris, which accounts for more than 80% of all pemphigus cases, 21 is known to affect more women than men in a ratio of approximately 1:1.5. [24][25][26][27] In this aspect, Israel is similar to northern countries, where the incidence of the disease is equal between the sexes. This may be partly owing to the large population of new immigrants to Israel from the former Union of Soviet Socialist Republics.…”

Section: Commentmentioning

confidence: 99%

Pemphigus Variant Associated With Penicillin Use

Heymann¹,

Chodick²,

Kramer³

et al. 2007

Arch Dermatol

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“…Ein Über-wiegen des weiblichen Geschlechts wurde auch von anderen Autoren angegeben [5,15,20,23]. Einige Untersucher fanden ein geringfügig höheres Auftreten des PV bei Männern [14,25].…”

Section: Besprechungunclassified