Changes in clinical features, histologic findings, and antigen profiles with development of pemphigus foliaceus from pemphigus vulgaris

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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Clinical and Immunologic Characterization in 26 Indian Pemphigus Patients

Journal of Cutaneous Medicine and Surgery ◽

10.2310/7750.2013.12097 ◽

2013 ◽

Vol 17 (5) ◽

pp. 321-331 ◽

Cited By ~ 2

Author(s):

Daisuke Tsuruta ◽

Amrinder J. Kanwar ◽

Keshavamurthy Vinay ◽

Shunpei Fukuda ◽

Hiroshi Koga ◽

...

Keyword(s):

Clinical Features ◽

Immunosorbent Assay ◽

Indirect Immunofluorescence ◽

Pemphigus Vulgaris ◽

Enzyme Linked Immunosorbent Assay ◽

Pemphigus Foliaceus ◽

Lower Sensitivity ◽

Before And After ◽

Desmoglein 3

Background: Pemphigus shows geographically variable characteristics. Objective: To study the clinical and immunologic characteristics of Indian pemphigus patients before and after treatment. Methods: Twenty-six Indian pemphigus patients were analyzed with regard to age, gender, clinical features, treatments and response, the results of histopathology, direct and indirect immunofluorescence (IF), enzyme-linked immunosorbent assay (ELISA), and immunoblot analyses. Results: There were 22 pemphigus vulgaris (PV) and 4 pemphigus foliaceus (PF) patients. Direct and indirect IF was positive in 95.8% and 56% of patients, respectively. Indices of ELISA were lower in our study. Immunoblot assays detected the 130 kDa desmoglein-3 in 10 PV patients and the 160 kDa desmoglein-1 in 1 PV patient; 190 kDa periplakin was unexpectedly detected in 8 patients. Conclusion: Indian pemphigus patients showed several unique characteristics, including younger population, predominance of PV, low ELISA indices, lower sensitivity of indirect IF and immunoblotting, and the presence of the 190 kDa periplakin in nearly one-third of patients.

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