Gingival pemphigus vulgaris preceding cutaneous lesion: A rare case report

Department of Periodontics, Armed Forces Medical College, Pune, India.
Journal of Indian Society of Periodontology 09/2012; 16(4):588-591. DOI: 10.4103/0972-124X.106922
Source: PubMed


Pemphigus is a group of autoimmune diseases characterized by formation of intraepithelial bullae in skin and the mucous membrane. Pemphigus vulgaris affects the oral mucosa in nearly all cases. Pemphigus vulgaris is characterized by auto antibodies directed against desmosome-associated protein antigens (desmoglein-3) found in epithelial and epidermal intercellular substance. We report here a case of pemphigus vulgaris of gingiva in an adult female patient at an early stage followed by dermatologic involvement. Perilesional incision was taken and histopathological and direct immunofluorescence was done for identification of specific antibodies. The oral lesions were treated with 0.1% Triamcinolone acetonide ointment and Prednisolone 20 mg twice daily with multivitamins was administered systemically for skin lesion.

11 Reads
  • [Show abstract] [Hide abstract]
    ABSTRACT: Pemphigus vulgaris (PV) frequently begins with oral lesions and progresses to skin lesions. A patient is described who developed skin lesions during follow-up and whose only initial symptom was desquamative gingivitis (DG). A 31-year-old woman presented with a 2-month history of painful gingiva. The diagnosis of PV was made according to clinical, histopathological, and immunofluorescent criteria. Topical corticosteroid (0.1% triamcinolone acetonide) was provided for the treatment of DG. Evaluation of the circulating autoantibody titers to desmoglein (Dsg)1 and Dsg3 was conducted by enzyme-linked immunosorbent assay (ELISA). The gingival PV lesions went into remission with the use of topical corticosteroid, although the patient experienced occasional recurrent oral lesions that required retreatment. She had regular follow-ups and remained relatively stable for several months. However, relapse and worsening of the oral lesions and the onset of skin lesions occurred after 26 months. Using ELISA, a change in the autoantibody profile corresponding to the transition from mucosal PV to mucocutaneous PV was confirmed. In all ELISA studies conducted throughout the course of the patient's disease, the Dsg3 ELISA was consistently high ranging from 150 to 200. However, the Dsg1 ELISA remained low, ranging from 10 to 30. After 26 months, Dsg3 (index value of 150) and Dsg1 (index value of 114) ELISA levels were elevated, consistent with the transition to mucocutaneous PV. In cases in which the lesions are limited to the oral cavity, PV sometimes may be managed successfully using only topical corticosteroids. However, it may not be possible to reduce the circulating Dsg autoantibody titers without systemic immunosuppression. The sustained high Dsg3 antibody level may cause "epitope spreading" and induce skin lesions. It may be prudent to determine post-treatment levels of Dsg using ELISA and, in consultation with the physician, recommend the addition of systemic therapy if Dsg3 levels remain elevated.
    Journal of Periodontology 03/2008; 79(2):369-75. DOI:10.1902/jop.2008.070258 · 2.71 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Vesiculobullous diseases are frequently encountered by the practicing dermatologist. However, these disorders are often difficult to diagnose and manage. The oral cavity may be overlooked as a source of diagnostic information. Oral manifestations of vesiculobullous diseases may occur independently or precede cutaneous involvement by a year or more. Maintenance of oral hygiene, discomfort that results in poor hydration and nourishment, and secondary infection must be addressed to assure disease control and patient comfort. The three vesiculobullous disorders that occur most commonly in the oral cavity are pemphigus vulgaris, cicatricial pemphigoid, and erythema multiforme. This article will discuss their oral presentation, diagnosis, and management.
    Seminars in dermatology 07/1994; 13(2):78-86.
  • [Show abstract] [Hide abstract]
    ABSTRACT: Pemphigus vulgaris (PV) is a rare, chronic, intraepithelial bullous disease with a potentially fatal outcome. Oral lesions are a hallmark of PV and occur in almost all cases, and represent the preliminary symptom in more than half of the patients. Gingival lesions are very common and, when solitary, often first recognized by periodontist. In the literature, gingival localization of PV are usually described as desquamative gingivitis (DG) and/or as vesiculobullous lesions of the free and attached gingiva; in our experience, early lesions only rarely appears as extensive erythema and erosions. PV at the onset may frequently appear on gingiva as isolated blisters and/or erosions mainly located to the free gingiva, very little in extension and hardly to recognize as bullous lesions.
    Journal Of Clinical Periodontology 06/2001; 28(5):489-93. DOI:10.1034/j.1600-051x.2001.028005489.x · 4.01 Impact Factor
Show more