Direct immunofluorescence of the outer root sheath in anagen and telogen hair in pemphigus vulgaris and pemphigus foliaceus

Background: Since the outer root sheath of hair follicle is structurally analogous to epidermal keratinocytes, direct immunofluorescence of hair may be an alternate method for monitoring disease activity of pemphigus vulgaris. Our objective was to find the effectiveness of direct immunofluorescence of outer root sheath as an alternate method in monitoring patients with pemphigus vulgaris. Materials and methods: Sixteen diagnosed cases of pemphigus vulgaris and in clinical remission for at least three months were enrolled. Skin biopsy sample and five plucked scalp hair samples of each patient were stained with Fluorescein isothiocyanate conjugate .Results: Hair direct immunofluorescence was positive in 13 (81.25%) patients and skin direct immunofluorescence was also positive in 13 patients (81.25%).Conclusion: Direct immunofluorescence of hair is a simple, specific and non-invasive method, and provides the opportunity to avoid repeated skin biopsies as an appropriate alternative technique for the disease monitoring in patients with pemphigus vulgaris.

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The Significance of Scalp Involvement in Pemphigus: A Literature Review

BioMed Research International ◽

10.1155/2018/6154397 ◽

2018 ◽

Vol 2018 ◽

pp. 1-8 ◽

Cited By ~ 4

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.

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Assessment of the role of direct immunofluorescence of the outer root sheath in the diagnosis of pemphigus patients

Journal of the Egyptian Womenʼs Dermatologic Society ◽

10.1097/01.ewx.0000483141.15448.e4 ◽

2016 ◽

Vol 13 (2) ◽

pp. 83-91

Author(s):

Fairouz Badran ◽

Eisha A. Moniem ◽

Lalia Abdo ◽

Rasha Genedy ◽

Shaimaa Ismail

Keyword(s):

Direct Immunofluorescence ◽

Outer Root Sheath ◽

Root Sheath

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An Updated Review of Pemphigus Diseases

Medicina ◽

10.3390/medicina57101080 ◽

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.

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

Dermatology Reports ◽

10.4081/dr.2021.9286 ◽

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.

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Desmoglein 3 anchors telogen hair in the follicle

Journal of Cell Science ◽

10.1242/jcs.111.17.2529 ◽

1998 ◽

Vol 111 (17) ◽

pp. 2529-2537 ◽

Cited By ~ 5

Author(s):

P.J. Koch ◽

M.G. Mahoney ◽

G. Cotsarelis ◽

K. Rothenberger ◽

R.M. Lavker ◽

...

Keyword(s):

Cell Adhesion ◽

Hair Loss ◽

Pemphigus Vulgaris ◽

Normal Structure ◽

Structure And Function ◽

Hair Follicles ◽

Outer Root Sheath ◽

Root Sheath ◽

And Function ◽

Desmoglein 3

Little is known about the function of desmosomes in the normal structure and function of hair. Therefore, it was surprising that mice without desmoglein 3 (the autoantigen in pemphigus vulgaris) not only developed mucous membrane and skin lesions like pemphigus patients, but also developed hair loss. Analysis of this phenotype indicated that hair was normal through the first growth phase (‘follicular neogenesis’). Around day 20, however, when the hair follicles entered the resting phase of the hair growth cycle (telogen), mice with a targeted disruption of the desmoglein 3 gene (DSG3-/-) lost hair in a wave-like pattern from the head to the tail. Hair then regrew and was lost again in the same pattern with the next synchronous hair cycle. In adults, hair was lost in patches. Gentle hair pulls with adhesive tape showed that anagen (growing) hairs were firmly anchored in DSG3-/- mice, but telogen hairs came out in clumps compared to that of DSG3+/− and +/+ littermates in which telogen hairs were firmly anchored. Histology of bald skin areas in DSG3-/- mice showed cystic telogen hair follicles without hair shafts. Histology of hair follicles in early telogen, just before clinical hair loss occurred, showed loss of cell adhesion (acantholysis) between the cells surrounding the telogen club and the basal layer of the outer root sheath epithelium. Electron microscopy revealed ‘half-desmosomes’ at the plasma membranes of acantholytic cells. Similar acantholytic histology and ultrastructural findings have been previously reported in skin and mucous membrane lesions of DSG3-/- mice and pemphigus vulgaris patients. Immunoperoxidase staining with an antibody raised against mouse desmoglein 3 showed intense staining on the cell surface of keratinocytes surrounding the telogen hair club in normal mice. Similar staining was seen in human telogen hair with an anti-human desmoglein 3 antibody. Finally, a scalp biopsy from a pemphigus vulgaris patient showed empty telogen hair follicles. These data demonstrate that desmoglein 3 is not only critical for cell adhesion in the deep stratified squamous epithelium, but also for anchoring the telogen hair to the outer root sheath of the follicle and underscore the importance of desmosomes in maintaining the normal structure and function of hair.

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Clinical, histopathological and immunofluorescent study of vesicobullous lesions of skin

International Journal of Research in Medical Sciences ◽

10.18203/2320-6012.ijrms20191341 ◽

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.

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

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2011-0425-oa ◽

2012 ◽

Vol 136 (11) ◽

pp. 1402-1407 ◽

Cited By ~ 11

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.

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