Abstract
This article describes a case of pemphigus vulgaris in a relatively young patient with exacerbated characteristics, widespread lesions, and rapid evolution. A 40-year-old woman sought treatment, complaining about severe oral pain and dysphagia. Intraoral inspection revealed ulcerated lesions on the lips, buccal mucosa, and floor of the mouth. The number of lesions quickly increased and resembled erythema multiforme. She also presented blistering lesions on the back, abdomen, neck, and eyes. Incisional oral biopsy revealed pemphigus vulgaris. This report emphasizes the importance of early diagnosis of an oral biopsy in systemic diseases and the management of the lesions.
1
Introduction
Pemphigus is a group of rare, potentially life-threatening diseases, characterized by autoimmune mucocutaneous reaction that is characterized by intraepithelial blister formation resulting from breakdown of the cellular adhesion between epithelial cells . It can affect 0.1 to 0.5 per 100 000 persons per year .
Pemphigus can be classified into 6 types: pemphigus vulgaris (PV), pemphigus vegetans, pemphigus erythematosus, pemphigus foliaceus, paraneoplastic pemphigus, and immunoglobulin (Ig) A pemphigus . Pemphigus vulgaris is the main variant and the only one that usually affects the mouth and precedes skin lesions in most cases . Pemphigus vulgaris can be a serious and potentially fatal disease. Therefore, early diagnosis is important to perform ideal management of the affected patients.
This article reports a severe case of PV with previous oral lesions manifestation.
2
Case report
A 40-year-old woman presented to our institution complaining about severe oral pain and dysphagia. Intraoral inspection revealed ulcerated lesions on the lips, buccal mucosa, and floor of the mouth. In some areas, the oral mucosa was detaching and bleeding when touching ( Fig. 1 ). The Nikolsky sign was negative.
There was no relevant medical history, and the disease presented rapid evolution. Therefore, an incisional biopsy was performed in the margin of 2 different lesions. After 7 days, the number of lesions increased; and they were more extensive, involving lips and tongue, with constant bleeding and lip scabs formation resembling erythema multiforme. She also presented blistering lesions on the back, abdomen, neck, and eyes ( Fig. 2 ). At this point, the patient was already hospitalized under dermatologists care; and the presumptive diagnoses were PV, benign mucous membrane pemphigoid, or erythema multiforme. The biopsy confirmed PV.
Microscopically, we observed mucosal epithelium with suprabasal acantholytic blisters and mild perivascular mononuclear infiltrate, with presence of acantholytic cells (Tzanck cells) in the suprabasal cleft and lateral disjunction between the basal cells ( Fig. 3 A, B ). The possibility of paraneoplastic pemphigus was investigated, but direct immunofluorescence (DIF) demonstrated IgG presence in intercellular regions of the basal layer ( Fig. 3 C, D). The hypothesis of paraneoplastic origin was therefore discarded.
Treatment consisted of 100 mg/d oral dose of prednisone associated with a topical antiseptic and corticosteroid manipulated mouthwash solution (clobetasol propionate 0.05%, tetracycline 250 mg, nystatin 100 000 UI, and lidocaine 2%). After 2 months of treatment, the lesions showed considerable regression ( Fig. 4 ). Subsequently, the dose was constantly decreased up to 20 mg/d; and the patient was released, but continued follow-up monthly. After 9 months, she is stable and free of new lesions.
2
Case report
A 40-year-old woman presented to our institution complaining about severe oral pain and dysphagia. Intraoral inspection revealed ulcerated lesions on the lips, buccal mucosa, and floor of the mouth. In some areas, the oral mucosa was detaching and bleeding when touching ( Fig. 1 ). The Nikolsky sign was negative.
