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 HLA-DRB1 and DQB1 genetic susceptibility to pemphigus vulgaris and pemphigus foliaceus in Vietnamese patients

2021 ◽  
Author(s):  
The Bich Thanh Vuong ◽  
Duc Minh Do ◽  
Phuc Thinh Ong ◽  
Thai Van Thanh Le
Keyword(s):  
Clinical Manifestations ◽  
Pemphigus Vulgaris ◽  
Class Ii ◽  
Hla Class Ii ◽  
Control Group ◽  
Direct Immunofluorescence ◽  
Pemphigus Foliaceus ◽  
High Morbidity ◽  
Increased Risk ◽  
Life Threatening

Background: Pemphigus is a group of rare, life-threatening bullous autoimmune diseases that affect the skin and mucous membranes and are associated with high morbidity and morbidity. HLA class II genes, particularly HLA-DRB1 and HLA-DQB1, play roles in pemphigus. Objectives: To investigate the susceptibility of HLA class II DRB1 and DQB1 alleles in Vietnamese patients with pemphigus vulgaris (PV) or pemphigus foliaceus (PF). Methods: The study enrolled 31 participants (22 with PV, 9 with PF) with diagnoses confirmed by clinical manifestations, histopathology, and direct immunofluorescence from November 2019 to June 2020. The HLA polymorphisms were determined by Sanger sequencing. The HLA-DRB1 and HLA-DQB1 profiles of the 101 healthy individuals in the control group have been published previously. Results: The frequencies of HLA-DRB1*14, DRB1*13:07, DRB1*04:04, DRB1*03:02, DQB1*02:02, and DQB1*05:03 were significantly higher, whereas those of DRB1*09:01, DRB1*12:02, DQB1*03:03, DQB1*05:01, and DQB1*06:01 were significantly lower, in the PV group than in the controls. The frequencies of DRB1*14:54, DRB1*13:07, and HLA-DQB1*03:02 were significantly higher in the PF group than in the controls. Conclusions: Alleles HLA-DRB1*14:54, DRB1*14:04, DRB1*14:03, DRB1*14:01, DRB1*14:12, DRB1*13:07, DRB1*04:04, DRB1*03:02, DQB1*02:02, and DQB1*05:03 were associated with an increased risk of PV, whereas alleles DRB1*09:01, DRB1*12:02, DQB1*03:03, DQB1*05:01, and DQB1*06:01 might protect against PV. In PF, DRB1*14:54, DRB1*13:07, and HLADQB1* 03:02 are promising susceptibility alleles.

scholarly journals An Updated Review of Pemphigus Diseases

Medicina ◽  
2021 ◽  
Vol 57 (10) ◽  
pp. 1080
Author(s):  
Ali M. Malik ◽  
Sarah Tupchong ◽  
Simo Huang ◽  
Abhirup Are ◽  
Sylvia Hsu ◽  
...  
Keyword(s):  
Clinical Features ◽  
Clinical Assessment ◽  
Treatment Options ◽  
Delayed Diagnosis ◽  
Pemphigus Vulgaris ◽  
Enzyme Linked Immunosorbent Assay ◽  
Direct Immunofluorescence ◽  
Pemphigus Foliaceus ◽  
Iga Pemphigus ◽  
Anti Cd20

Clinicians may encounter a variety of skin conditions that present with vesiculobullous lesions in their everyday practice. Pemphigus vulgaris, pemphigus foliaceus, IgA pemphigus, and paraneoplastic pemphigus represent the spectrum of autoimmune bullous dermatoses of the pemphigus family. The pemphigus family of diseases is characterized by significant morbidity and mortality. Considering the risks associated with a delayed diagnosis or misdiagnosis and the potential for overlap in clinical features and treatment, evaluation for suspected pemphigus disease often requires thorough clinical assessment and laboratory testing. Diagnosis is focused on individual biopsies for histopathology and direct immunofluorescence. Additional laboratory methods used for diagnosis include indirect immunofluorescence and enzyme-linked immunosorbent assay. Recent advancements, including anti-CD20 therapy, have improved the efficacy and reduced the morbidity of pemphigus treatment. This contribution presents updates on the pathophysiology, clinical features, diagnostic work-up, and medical management of pemphigus. Improved strategies for diagnosis and clinical assessment are reviewed, and newer treatment options are discussed.

scholarly journals The value of trichoscopy in the differential diagnosis of scalp lesions in pemphigus vulgaris and pemphigus foliaceus

2014 ◽  
Vol 89 (6) ◽  
pp. 1007-1012 ◽  
Author(s):  
Marta Sar-Pomian ◽  
Marta Kurzeja ◽  
Lidia Rudnicka ◽  
Malgorzata Olszewska
Keyword(s):  
Differential Diagnosis ◽  
Pemphigus Vulgaris ◽  
Pemphigus Foliaceus ◽  
Scalp Lesions

scholarly journals Clinical, histopathological and immunofluorescent study of vesicobullous lesions of skin

2019 ◽  
Vol 7 (4) ◽  
pp. 1288
Author(s):  
Seeram Satish Kumar ◽  
Bhagyalakshmi Atla ◽  
Guru Prasad Patnala ◽  
Kukkala Saraswati Sarat Srinivas ◽  
Saraswathi Samantra ◽  
...  
Keyword(s):  
Bullous Pemphigoid ◽  
Histopathological Examination ◽  
Pemphigus Vulgaris ◽  
Direct Immunofluorescence ◽  
Pemphigus Foliaceus ◽  
Female Ratio ◽  
Medical College ◽  
Intensive Investigation ◽  
Male To Female

Background: Vesiculobullous diseases have been the focus of intensive investigation in recent years. However, these disorders are still associated with substantial morbidity, considerable mortality and impaired quality of life. Accurate diagnosis of vesiculobullous lesions of skin entails evaluation of clinical, histopathologic and immunofluorescence findings.Methods: Hospital based prospective study for a period of 24 months from August 2014 to July 2016 in the Department of Pathology at Andhra Medical College, Visakhapatnam, India. Total of 50 patients aged 3-70 years with vesiculobullous lesions of both sexes attending the Department of Dermatology were selected and analysed clinically, histopathological examination and direct immunofluorescence (DIF).Results: In the present study, majority of patients presented between 51-60 yrs of age (32%) with male to female ratio of 1.08:1 and mean age of 46.02 years. Pemphigus vulgaris constituted the most common vesiculobullous disorder (32%) followed by bullous pemphigoid and pemphigus foliaceous, 18% each. Bullae were located intra epidermally in 68% and sub epidermally in 32% of the patients. DIF was positive in 80% of the cases. Overall clinicopathological correlation was established in 74%. Overall histopathological and direct immunofluorescence correlation was established in 78%. Out of 50 cases, 35 cases (70%) correlated clinically and histo-pathologically with direct immunofluorescence.Conclusions: In the present study, on histopathological examination alone pemphigus foliaceus and pemphigus vulgaris could be differentiated. Direct immunofluorescence was useful in differentiating epidermolysis bullosa acquisita from bullous pemphigoid which have similar histopathological picture. This study proves that direct immunofluorescence is confirmatory as well as diagnostic for vesiculobullous disorders.

Immunohistochemistry for Immunoglobulin G4 on Paraffin Sections for the Diagnosis of Pemphigus

2012 ◽  
Vol 136 (11) ◽  
pp. 1402-1407 ◽  
Author(s):  
Xuefeng Zhang ◽  
Elizabeth Hyjek ◽  
Keyoumars Soltani ◽  
Vesna Petronic-Rosic ◽  
Christopher R. Shea
Keyword(s):  
Normal Skin ◽  
Intercellular Junctions ◽  
Pemphigus Vulgaris ◽  
Direct Immunofluorescence ◽  
Pemphigus Foliaceus ◽  
Paraffin Sections ◽  
Specific Test ◽  
Immunoglobulin G4 ◽  
Frozen Tissue ◽  
Fresh Frozen

Context.—Pemphigus is a group of autoimmune vesiculobullous diseases characterized by immunoglobulin G (IgG) antibodies directed against desmosomal adhesion proteins, with IgG4 being the predominant subclass in active diseases. Direct immunofluorescence for IgG performed on fresh-frozen tissue plays a crucial role in diagnosing pemphigus. However, the diagnosis might be hindered when frozen tissue is not available. Objective.—To evaluate the usefulness of immunohistochemistry for IgG4 performed on paraffin sections as a diagnostic test for pemphigus. Design.—Eighteen immunofluorescence-proven pemphigus cases (12 pemphigus vulgaris, 6 pemphigus foliaceus) were studied. Four normal skin specimens and 32 nonpemphigus vesiculobullous disease specimens served as controls. Paraffin sections of all cases were examined immunohistochemically for IgG4 expression. Positivity was defined as distinct, condensed, continuous immunoreactivity localized to the intercellular junctions of keratinocytes. Results.—The immunostains were independently evaluated in a masked manner by 3 pathologists, with a 100% interobserver agreement. Nine of 12 pemphigus vulgaris cases (sensitivity 75.0%), and 4 of 6 pemphigus foliaceus cases (sensitivity 66.7%), were positive for IgG4 immunostain. The overall sensitivity was 72.2%. One control specimen (bullous pemphigoid) showed IgG4 positivity (specificity 97.2%). In specimens demonstrating acantholysis, 8 of 10 pemphigus vulgaris cases (sensitivity 80.0%) and 4 of 4 pemphigus foliaceus cases (sensitivity 100.0%) were positive for IgG4. The overall sensitivity for specimens with acantholytic lesions was 85.7%. Conclusion.—Immunohistochemistry for IgG4 provides a reasonably sensitive and highly specific test for diagnosing pemphigus, especially when frozen tissue is not available, and active acantholytic lesions are examined.

Drug-induced pemphigus foliaceus with features of pemphigus vulgaris

2001 ◽  
Vol 144 (2) ◽  
pp. 421-422 ◽  
Author(s):  
K. Ogata ◽  
H. Nakajima ◽  
M. Ikeda ◽  
Y. Yamamoto ◽  
M. Amagai ◽  
...  
Keyword(s):  
Pemphigus Vulgaris ◽  
Pemphigus Foliaceus ◽  
Drug Induced

Clinical outcomes of syphilis in HIV-negative and HIV-positive MSM: occurrence of repeat syphilis episodes and non-treponemal serology responses

2021 ◽  
pp. sextrans-2020-054887
Author(s):  
Silvia Achia Nieuwenburg ◽  
Ricardo Jamie Sprenger ◽  
Maarten Franciscus Schim van der Loeff ◽  
Henry John Christiaan de Vries
Keyword(s):  
Hiv Infection ◽  
Controlled Trial ◽  
Clinical Manifestations ◽  
Response To Treatment ◽  
Disease Stage ◽  
Hiv Positive ◽  
Sexual Risk Behaviour ◽  
Serological Response ◽  
History Of ◽  
Hiv Negative

ObjectivesHIV-positive men who have sex with men (MSM) may be at a higher risk of repeat syphilis, have different clinical manifestations and have a different serological response to treatment compared with HIV-negative MSM. The objective of this study was to assess whether HIV-negative and HIV-positive MSM with infectious syphilis (primary, secondary or early latent) differed in history of previous syphilis episodes, disease stage and non-treponemal titre of initial and repeat episodes, and the titre response 6 and 12 months after treatment. Furthermore, determinants associated with an inadequate titre response after treatment were explored.MethodsThis retrospective analysis used data of five longitudinal studies (four cohorts; one randomised controlled trial) conducted at the STI clinic in Amsterdam, the Netherlands. Participants were tested for syphilis and completed questionnaires on sexual risk behaviour every 3–6 months. We included data of participants with ≥1 syphilis diagnosis in 2014–2019. Pearson’s χ² test was used to compare HIV-negative and HIV-positive MSM in occurrence of previous syphilis episodes, disease stage of initial and repeat syphilis episode and non-treponemal titre treatment responses.ResultsWe included 355 participants with total 459 syphilis episodes. HIV-positive MSM were more likely to have a history of previous syphilis episodes compared with HIV-negative MSM (68/90 (75.6%) vs 96/265 (36.2%); p<0.001). Moreover, HIV-positive MSM with repeat syphilis were less often diagnosed with primary syphilis (7/73 (9.6%) vs 36/126 (28.6%)) and more often diagnosed with secondary syphilis (16/73 (21.9%) vs 17/126 (13.5%)) and early latent syphilis (50/73 (68.5%) vs 73/126 (57.9%)) (p=0.005). While not significantly different at 12 months, HIV-negative MSM were more likely to have an adequate titre response after 6 months compared with HIV-positive MSM (138/143 (96.5%) vs 66/74 (89.2%); p=0.032).ConclusionsIn repeat syphilis, HIV infection is associated with advanced syphilis stages and with higher non-treponemal titres. HIV infection affects the serological outcome after treatment, as an adequate titre response was observed earlier in HIV-negative MSM.

Coexistence of IgA antibodies to desmogleins 1 and 3 in pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus

2007 ◽  
Vol 156 (4) ◽  
pp. 635-641 ◽  
Author(s):  
L.F. Mentink ◽  
M.C.J.M. de Jong ◽  
G.J. Kloosterhuis ◽  
J. Zuiderveen ◽  
M.F. Jonkman ◽  
...  
Keyword(s):  
Pemphigus Vulgaris ◽  
Pemphigus Foliaceus ◽  
Paraneoplastic Pemphigus ◽  
Iga Antibodies
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