Direct immunofluorescence analysis of oral Tzanck smears for pemphigus vulgaris: A diagnostic test

2021 ◽  
Author(s):  
Wenxiu He ◽  
Kaiyi Li ◽  
Xiaosheng Hu ◽  
Hong Hua ◽  
Pan Wei
Keyword(s):  
Diagnostic Test ◽  
Pemphigus Vulgaris ◽  
Direct Immunofluorescence ◽  
Immunofluorescence Analysis

scholarly journals A relapse of pemphigus vulgaris in pemphigus herpetiformis or a phenotypic “switch”

2022 ◽  
Vol 13 (1) ◽  
pp. 107-108
Author(s):  
Siham Belmourida ◽  
Meriame Meziane ◽  
Nadia Ismaili ◽  
Laila Benzekri ◽  
Badreddine Hassam ◽  
...  
Keyword(s):  
Corticosteroid Therapy ◽  
Transition Period ◽  
Pemphigus Vulgaris ◽  
Skin Lesions ◽  
Exceptional Case ◽  
The Body ◽  
Direct Immunofluorescence ◽  
Phenotypic Switch ◽  
Dermatological Examination ◽  
Phenotypic Transition

Sir, Pemphigus herpetiformis (PH) was originally described by Jablonska et al. in 1975. Clinically, PH presents itself as a herpetiform dermatitis with immunopathological characteristics of pemphigus [1,2]. We report an exceptional case of typical pemphigus vulgaris (PV) relapsing after 36 years in PH. A 65-year-old patient, followed for PV for 36 years and treated with corticosteroid therapy with a remission for more than thirty years, consulted for pruriginous lesions evolving for the previous eight months. A dermatological examination revealed urticariform pruriginous ring lesions surmounted by small peripheral vesicles spread throughout the body (Fig. 1), sparing the mucous membranes, and without Nikolsky’s sign. After two non-specific skin biopsies, the histological examination revealed an intraepidermal bubble with acantholytic cells and eosinophilic spongiosis (Figs. 2a and 2b). Direct immunofluorescence confirmed the diagnosis of pemphigus and indirect immunofluorescence was at the upper limit. The diagnosis of a PV relapse in PH was retained and a dapsone-based treatment was initiated at a dose of 150 mg/day and stopped seven days later when met with hemolytic anemia. Oral corticosteroid therapy involving prednisone at a dose of 1 mg/kg/day was initiated but, given the persistence of the pruritus, the decision was to combine methotrexate at a dose of 12.5 mg/week. A good evolution and a decline within eight months were observed. An improved pruritus and the disappearance of the skin lesions were achieved after one month of treatment. PV and PH are two different anatomical and clinical entities of the autoimmune disease pemphigus, with distinct clinical, histopathological, and immunopathological characteristics [1,2]. Our observation documents a complete phenotypic “switch” of pemphigus with a transition from PV to PH both clinically, histologically, and immunologically. Several rare cases of PV switching to superficial pemphigus (SP) (“phenotypic switch”) have, since 1991, been reported, with a higher frequency this direction than otherwise; the transition period varies from six months to twenty years [3]. To the best of our knowledge, no case has been described of a progression from PV to PH. Having observed one firsthand, we are first to describe the case of a complete phenotypic switch from PV to PH. The mechanism of such a transition remains poorly understood and is often observed during a relapse. Some authors suggest that the effect of immunosuppressants on the desmoglein DSG3 more marked than on DSG1 could explain the relapse of PS in PH [3,4]. Future studies on the immunological factors and predictors of PV relapses after the discontinuation of treatment would be useful to better understand the mechanisms of a relapse in pemphigus, with or without a phenotypic transition.

scholarly journals Present Tense: A Peculiar Case of Pemphigus Vulgaris Presenting with Tense Blisters during the COVID-19 Pandemic

2021 ◽  
Vol 55 (5) ◽  
Author(s):  
Dianne Katherine R. Salazar-Paras ◽  
Mae N. Ramirez-Quizon
Keyword(s):  
Pemphigus Vulgaris ◽  
Direct Immunofluorescence ◽  
Bullous Dermatosis ◽  
Present Tense ◽  
Peculiar Case

Pemphigus vulgaris is an autoimmune bullous dermatosis presenting with flaccid blisters and erosions. The morphology of pemphigus reflects the more superficial intraepidermal level of split seen histologically in contrast with pemphigoid, where the level of split is deep below the epidermis. This is a case of a 58-year-old male clinically presenting with arcuate tense bullae, which are more characteristic of the pemphigoid group of disorders, which revealed an intraepidermal split and tombstoning pattern of the basal epidermis on histopathology. Direct immunofluorescence revealed intercellular IgG and C3 distribution. Although this patient presented clinically with tense bullae, the histopathology and direct immunofluorescence results were consistent with pemphigus vulgaris.

scholarly journals The Significance of Scalp Involvement in Pemphigus: A Literature Review

2018 ◽  
Vol 2018 ◽  
pp. 1-8 ◽  
Author(s):  
Marta Sar-Pomian ◽  
Lidia Rudnicka ◽  
Malgorzata Olszewska
Keyword(s):  
Web Of Science ◽  
Clinical Manifestations ◽  
Pemphigus Vulgaris ◽  
Hair Follicles ◽  
Response To Treatment ◽  
Direct Immunofluorescence ◽  
Pemphigus Foliaceus ◽  
Medical Databases ◽  
Scalp Lesions ◽  
Reliable Diagnostic Method

Scalp is a unique location for pemphigus because of the abundance of desmogleins localized in hair follicles. Scalp involvement is observed in up to 60% of patients in the course of pemphigus. The lesions may occasionally lead to alopecia. Unforced removal of anagen hairs in a pull test is a sign of high disease activity. Direct immunofluorescence of plucked hair bulbs is considered a reliable diagnostic method in patients with pemphigus. Follicular acantholysis is a characteristic histopathological feature of pemphigus lesions localized on the scalp. Trichoscopy may serve as a supplementary method in the diagnosis of pemphigus. This review summarizes the most recent data concerning scalp involvement in pemphigus vulgaris and pemphigus foliaceus. A systematic literature search was conducted in three medical databases: PubMed, Embase, and Web of Science. The analysis included literature data about desmoglein distribution in hair follicles, as well as information about clinical manifestations, histopathology, immunopathology, and trichoscopy of scalp lesions in pemphigus and their response to treatment.

scholarly journals Pemphigus Vulgaris: A Clinical Study of 31 Cases (2004–2014) in Morocco

2020 ◽  
Vol 2020 ◽  
pp. 1-7
Author(s):  
Titou Hicham ◽  
Fatima Zahra Chahnoun ◽  
Tarik Hanafi ◽  
Naoufal Hjira ◽  
Boui Mohammed
Keyword(s):  
Oral Cavity ◽  
Age At Onset ◽  
Pemphigus Vulgaris ◽  
Direct Immunofluorescence ◽  
Long Term Outcomes ◽  
Prognosis Factors ◽  
Mucosal Involvement ◽  
Multiple Relapses

Background. Pemphigus vulgaris is a rare bullous autoimmune dermatosis whose evolution and prognosis are unpredictable. Aim. The objective was to analyze long-term outcomes in patients with pemphigus vulgaris by identifying the factors that are able to influence prognosis, in particular the phenotype of pemphigus vulgaris, age at onset, multiplicity of mucosal involvement, relapse and remission rates, and survival functions. Methods. A retrospective analysis of a cohort of 31 patients followed for pemphigus vulgaris during the period from January 2004 to January 2014. Inclusion criteria were a diagnosis of pemphigus vulgaris confirmed by histopathology and direct immunofluorescence (DIF) and a period of follow-up of at least five years from the diagnosis. The following information was collected by a single investigator. Results. In total, 67.7% of patients presented a mucocutaneous pemphigus vulgaris. Male-female sex ratio was 2.4. The median duration of patient’s follow-up was estimated at 7 (6–9) years. Multiple mucosal involvement in the oral cavity and at other mucosal sites was significantly associated with severe mucocutaneous pemphigus vulgaris (p=0.01). Multiple relapses were significantly associated with the disease severity (p=0.04). Conclusion. Poor prognosis factors were severe mucocutaneous type of pemphigus vulgaris and multiple mucosal involvement in the oral cavity and at other mucosal sites.

Value of direct immunofluorescence in predicting remission in pemphigus vulgaris

2006 ◽  
Vol 45 (11) ◽  
pp. 1308-1311 ◽  
Author(s):  
Kamran Balighi ◽  
Arash Taheri ◽  
Parisa Mansoori ◽  
Cheida Chams
Keyword(s):  
Pemphigus Vulgaris ◽  
Direct Immunofluorescence
Sign in / Sign up

Export Citation Format

Share Document