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[Disseminated cicatricial pemphigoid]

O Braun-Falco, H H Wolff, E Ponce

    Der Hautarzt; Zeitschrift Fur Dermatologie, Venerologie, Und Verwandte Gebiete
    |May 1, 1981
    PubMed
    Summary

    This case study presents a rare form of cicatricial pemphigoid in a 68-year-old man, characterized by skin lesions and blisters without mucous membrane involvement. Azathioprine showed effectiveness in treatment.

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    Area of Science:

    • Dermatology
    • Immunodermatology

    Background:

    • Disseminated cicatricial pemphigoid is an extremely rare autoimmune blistering disease.
    • This condition primarily affects the skin, often leading to scarring.

    Observation:

    • A 68-year-old male presented with disseminated prurigo-like nodules, keloidiform plaques, tense blisters, and atrophic scars on the trunk and extremities.
    • Mucous membranes were unaffected in this patient.
    • Histopathology revealed subepidermal blisters with intact epidermis, and a blister floor showing fibroplasia, angioplasia, and minimal eosinophilic infiltrate.

    Findings:

    • Direct immunofluorescence demonstrated linear deposition of IgM and C3 in the basement membrane zone.
    • Electron microscopy indicated subepidermal microvesicles and fibrin deposits in early lesions, preceding edema and blister formation.
    • No circulating antibodies were detected.

    Implications:

    • This case highlights the clinical and histological features of a rare variant of cicatricial pemphigoid.
    • The findings contribute to understanding the pathogenesis of subepidermal blistering diseases.
    • Azathioprine emerged as a potentially effective treatment option for this rare condition.

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