Dermatology Online Journal

Pemphigus vulgaris manifesting as a sole persistent lesion on the lower lip: A case report
Mohammad Shahidi Dadras MD¹, Mehdi Qeisari MD², Soudabeh Givrad MD³
Dermatology Online Journal 15 (6): 7

1. Associate Professor of Dermatology, Skin Research Centre, Shahid Beheshti University (M.C.), Shohada-e-Tajrish Hospital, Tehran, Iran. src@sbmu.ac.ir
2. Resident of Dermatology, Skin Research Centre, Shahid Beheshti University (M.C.), Shohada-e-Tajrish Hospital, Tehran, Iran. prashtian@yahoo.com
3. General Practitioner, Skin Research Centre, Shahid Beheshti University (M.C.), Shohada-e-Tajrish Hospital, Tehran, Iran. soodabeh.givrad@gmail.com

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Abstract

Oral lesions in patients with pemphigus vulgaris commonly manifest as slowly healing erosions with irregular and ill-defined borders of the buccal mucosa and palate. These lesions may be the initial and sole presentation of pemphigus vulgaris. Herein, we describe a 37-year-old woman suffering from pemphigus vulgaris who had only a solitary crusted erosion on the lower lip persisting for eight months without any progression.

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Introduction

Oral lesions, the initial presentation of pemphigus vulgaris in 50 to 70 percent of cases, may develop months before the skin lesions arise. In fact, oral lesions may be the sole manifestation of pemphigus vulgaris in many patients [1]. These lesions appear as slowly healing erosions with irregular and ill-defined borders of the buccal mucosa and the palate; they often extend as additional epithelium sloughs [1].

Here, we present a case of pemphigus vulgaris that manifested as a solitary crusted erosion on the lower lip and persisted for eight months without showing any progression.

Report

Figure 1
Figure 1. Elliptical crusted erosion located on the mid-lower lip

A 37-year-old woman complained of a crusted ulceration on the lower lip accompanied by irritation and burning sensation for nearly eight months (Fig. 1). Various topical treatments had been prescribed for her in her over this period; however, none had promoted healing of the lesion.

Physical examination revealed a solitary, well-defined, crusted lesion on the mid-lower lip. Further evaluation did not disclose any other lesions of buccal mucosa or perioral skin.

Our initial diagnosis was factitious cheilitis; therefore, we prescribed topical steroid and zinc oxide for her. Moreover, the patient was instructed to carefully avoid licking or biting the affected area. Nevertheless, two weeks later the patient returned to the clinic showing no signs of improvement. Evaluation of a smear of the lesion for leishman bodies yielded negative results. A biopsy was then performed to rule out pemphigus vulgaris and mucosal lichen planus.

Figure 2	Figure 3
Figures 2, 3, and 4. Lip mucosa showing suprabasal acantholysis (Tomb-stone pattern)

Figure 4

Histopathological evaluation of the biopsy specimen demonstrated a focal area of suprabasal bullae formation and acantholysis compatible with pemphigus vulgaris. A few neutrophils and eosinophils were also detected. There were neither necrotic keratinocytes nor vacuolar interface dermatitis (Figs. 2-4). Furthermore, direct immunofluorescence testing revealed a 2+ intercellular, mainly suprabasal, IgG and C3 deposition, in addition to 1+ IgA and negative IgM; all these factors favored the diagnosis of pemphigus vulgaris. There was no basement-membrane zone immunoglobulin deposition.

Figure 5
Figure 5. The first monthly follow-up visit

Chemistry panel, liver function tests, and complete blood count were all within normal range. Chest radiography, mammography, abdomino-pelvic sonography, and electrocardiogram showed no abnormalities; PPD testing revealed a negative result.

The patient was prescribed 40 mg/day of oral prednisolone and 100 mg/day azathioprine. Significant improvement was noted during the first monthly follow-up visit (Fig. 5). Once clearing was achieved, her prednisolone was tapered by 5 mg per month. At the present time, about 1 year after treatment initiation, she is clear and being maintained on 5 mg prednisolone per day and 100mg of azathioprine per day.

Discussion

Pemphigus vulgaris, a blistering autoimmune disease of skin and mucous membranes, is identified histologically by the formation of intraepidermal bullae situated above the basal layer, associated with acantholysis. Direct and indirect immunofluorescence reveal the presence of circulating IgG, directed mostly against desmosomal desmoglein 3, in intercellular areas between keratinocytes [2]. The mean age of the disease onset is 40 to 60 years [3]. The prevalence of the disease is reported to be nearly equal between sexes in most parts of the world [3]. However, some studies report a higher prevalence among women [4, 5].

Patients with pemphigus vulgaris commonly suffer from mucosal involvement that may precede cutaneous manifestations by months or may be the sole manifestation of the disease. The presenting site of the disease varies according to the geographical area. Nearly 50 to 70 percent of patients present with oral lesions [1]. In Iran, 62 percent of pemphigus vulgaris patients referred to skin clinics complain of oral lesions [6]. Intact bullae are rarely observed in the oral cavity; in fact, most patients present with irregular erosions with ill-defined borders that tend to heal very slowly and often extend [1]. These erosions are commonly detected in buccal mucosa and the palate [1, 2, 7]; some cases may progress to involve the pharynx and larynx, causing hoarseness and dysphagia [1, 8]. Other mucous membranes occasionally involved comprise the nasal mucosa, esophagus, conjunctiva, anus, penis, vagina, cervix, and labia [1].

In our patient the presentation of pemphigus vulgaris was noteworthy from three aspects. First, oral lesions commonly occur in buccal mucosa, palate and gingivia [1, 7, 8, 9]. However, the presenting lesion in this patient was located on the middle of her lower lip. A solitary lip lesion is a highly unusual presenting sign. Although lesions may extend from mucosal areas toward the vermillion lip, creating thick fissured hemorrhagic crusts, the lip is rarely the initial presenting site. Due to its uncommon manifestation, that persisted for eight months, a series of incorrect diagnoses were established for our patient including factitious cheilitis, recurrent herpes labialis, leishmaniasis, and mucosal lichen planus. Nonetheless, in a study performed on 55 patients with oral lesions of pemphigus vulgaris, a six month delay in diagnosis is not very uncommon [7].

In addition, unlike the usual irregular and ill-defined lesions of pemphigus vulgaris, this patient had a regularly-shaped elliptical erosion with well defined borders. Futhermore, this patient's pemphigus had persisted focally on her lower lip for eight months without any peripheral extension and with a negative Nikolsky sign. Despite the fact that pemphigus vulgaris may remain confined to the oral mucosa for months without involving the skin, mucosal erosions normally extend peripherally throughout the course of the disease.

The hallmark of pemphigus vulgaris is the acantholysis accompanied by cellular detachment situated just above the suprabasal stratified squamous epithelium [8]. The diagnosis is confirmed by the presence of IgG depositions on the surface of the epithelial keratinocytes, intracellularly by direct immunofluorescence evaluation. Complement component C3, IgM, and IgA are not detected much less often than IgG and staining is less intense. In fact, direct immunofluorescence testing (DIF) can be acknowledged as the most sensitive and accurate way of diagnosing mucosal pemphigus vulgaris [1].

In our patient, histopathology and direct immunofluorescence confirmed the diagnosis of pemphigus vulgaris and ruled out the differential diagnoses of paraneoplastic pemphigus [10], mucosal lichen planus, erythema multiforme, actinic cheilitis, SCC, herpes, and apthous ulcers [11]. Furthermore, a negative smear for leishman bodies excluded the diagnosis of leishmaniasis.

Although, topical treatments, such as potent steroids and tacrolimus [12], can be used in mild oral or cutaneous pemphigus, because of possible aggressive behavior we used the preferred and most established treatment of this disease initially to minimize the possibility of treatment resistance and progression of the disease.

Acknowledgment: This project was funded by the Skin Research Centre, Shahid Beheshti University, M.C., Shohada-e-Tajrish Hospital. I, Mohammad Shahidi Dadras, had full access to all the data in this report and take responsibility for the integrity of the data. Authors declare no financial interest in this manuscript and no affiliation (relationships) to disclose.

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