Pemphigus herpetiformis - a case report of a rare form of pemphigus and review of the literature
Introduction. Pemphigus herpetiformis is the rare variant of pemphigus with characteristic clinical features, histopathological findings different from the convectional pemphigus, and immunological findings consistent with pemphigus. Case report. We presented a 65-year-old woman with initia...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Military Health Department, Ministry of Defance, Serbia
2016-01-01
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| Series: | Vojnosanitetski Pregled |
| Subjects: | |
| Online Access: | http://www.doiserbia.nb.rs/img/doi/0042-8450/2016/0042-84501600044S.pdf |
| _version_ | 1818997522089115648 |
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| author | Stojković-Filipović Jelena Lekić Branislav Milčić Danijela Milinković Mirjana V. |
| author_facet | Stojković-Filipović Jelena Lekić Branislav Milčić Danijela Milinković Mirjana V. |
| author_sort | Stojković-Filipović Jelena |
| collection | DOAJ |
| description | Introduction. Pemphigus herpetiformis is the rare variant of pemphigus with
characteristic clinical features, histopathological findings different from
the convectional pemphigus, and immunological findings consistent with
pemphigus. Case report. We presented a 65-year-old woman with initial
pruritus followed by pruritic urticarial papules and plaques, some with
annular rings of tense vesicles on the periphery, on the trunk and
extremities, with no mucous lesions. Histopathological examination
demonstrated spongiosis and intraepidermal vesicles in the mid or subcorneal
epidermis in some biopsy specimen, with neutrophil and eosinophil
infiltrate. Direct immunoflorescent microscopy revealed intercellular IgG
deposition, most prominent in the upper layers of epidermis. Indirect
immunoflorescent microscopy showed intercellular binding of IgG
autoantibodies in the patient’s sera. Initially the patient was threated
with systemic corticosteroids and azathioprine, but dapson provided complete
clinical remission. Conclusion. This entity was established 40 years ago,
and around 100 patients have been reported worldwide. It is important to be
aware of this particular form of pemphigus because clinical presentation,
course of the disease and therapeutic approach are different from
conventional forms of pemphigus. |
| first_indexed | 2024-12-20T21:46:58Z |
| format | Article |
| id | doaj.art-6e2b6cf913cb4444ac59581e177556cd |
| institution | Directory Open Access Journal |
| issn | 0042-8450 2406-0720 |
| language | English |
| last_indexed | 2024-12-20T21:46:58Z |
| publishDate | 2016-01-01 |
| publisher | Military Health Department, Ministry of Defance, Serbia |
| record_format | Article |
| series | Vojnosanitetski Pregled |
| spelling | doaj.art-6e2b6cf913cb4444ac59581e177556cd2022-12-21T19:25:39ZengMilitary Health Department, Ministry of Defance, SerbiaVojnosanitetski Pregled0042-84502406-07202016-01-01731096797210.2298/VSP150617044S0042-84501600044SPemphigus herpetiformis - a case report of a rare form of pemphigus and review of the literatureStojković-Filipović Jelena0Lekić Branislav1Milčić Danijela2Milinković Mirjana V.3Clinical Center of Serbia, Clinic of Dermatovenerology, Belgrade + Faculty of Medicine, BelgradeClinical Center of Serbia, Clinic of Dermatovenerology, BelgradeClinical Center of Serbia, Clinic of Dermatovenerology, Belgrade + Faculty of Medicine, BelgradeClinical Center of Serbia, Clinic of Dermatovenerology, Belgrade + Faculty of Medicine, BelgradeIntroduction. Pemphigus herpetiformis is the rare variant of pemphigus with characteristic clinical features, histopathological findings different from the convectional pemphigus, and immunological findings consistent with pemphigus. Case report. We presented a 65-year-old woman with initial pruritus followed by pruritic urticarial papules and plaques, some with annular rings of tense vesicles on the periphery, on the trunk and extremities, with no mucous lesions. Histopathological examination demonstrated spongiosis and intraepidermal vesicles in the mid or subcorneal epidermis in some biopsy specimen, with neutrophil and eosinophil infiltrate. Direct immunoflorescent microscopy revealed intercellular IgG deposition, most prominent in the upper layers of epidermis. Indirect immunoflorescent microscopy showed intercellular binding of IgG autoantibodies in the patient’s sera. Initially the patient was threated with systemic corticosteroids and azathioprine, but dapson provided complete clinical remission. Conclusion. This entity was established 40 years ago, and around 100 patients have been reported worldwide. It is important to be aware of this particular form of pemphigus because clinical presentation, course of the disease and therapeutic approach are different from conventional forms of pemphigus.http://www.doiserbia.nb.rs/img/doi/0042-8450/2016/0042-84501600044S.pdfpemphigusrare diseasesdiagnosisdrug therapytreatment outcome |
| spellingShingle | Stojković-Filipović Jelena Lekić Branislav Milčić Danijela Milinković Mirjana V. Pemphigus herpetiformis - a case report of a rare form of pemphigus and review of the literature Vojnosanitetski Pregled pemphigus rare diseases diagnosis drug therapy treatment outcome |
| title | Pemphigus herpetiformis - a case report of a rare form of pemphigus and review of the literature |
| title_full | Pemphigus herpetiformis - a case report of a rare form of pemphigus and review of the literature |
| title_fullStr | Pemphigus herpetiformis - a case report of a rare form of pemphigus and review of the literature |
| title_full_unstemmed | Pemphigus herpetiformis - a case report of a rare form of pemphigus and review of the literature |
| title_short | Pemphigus herpetiformis - a case report of a rare form of pemphigus and review of the literature |
| title_sort | pemphigus herpetiformis a case report of a rare form of pemphigus and review of the literature |
| topic | pemphigus rare diseases diagnosis drug therapy treatment outcome |
| url | http://www.doiserbia.nb.rs/img/doi/0042-8450/2016/0042-84501600044S.pdf |
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