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Case Report

Pemphigus vulgaris of the oral cavi-

ty in a patient with coeliac disease:
coincidence or relationship?
F. Carroccio1, V. Rodolico2, A. Padovano Di Leva3, G. Campisi1, D. Compilato1
1Department of Surgical, Oncological and Oral Sciences, Sector of Oral Medicine “V. Margiotta”, University of Paler-

mo, Italy; 2Department of Sciences for Health Promotion, University of Palermo, Italy; 3Department of Surgical Sci-
ences, University of Foggia, Italy

Background. CD is a lifelong T cell-mediated enteropathy triggered by the ingestion of gluten in genetically suscep-
tible subjects. The disease has a heterogeneous range of clinical presentations. Among atypical aspects of CD some
affections interest the oral cavity. Pemphigus vulgaris (PV), can be associated with CD-auto-antibodies and a clinical
improvement of the PV after the gluten-free diet has been reported.
Case report. A 43-year-old Caucasian female referred to our Unit for a 1-year history of recurrent oral lesions treated with
anti-mycosis drugs without any clinical improvement. The patient was in good general health, although she had meteorism
and dyspepsia. The intra-oral examination revealed bullous lesions on the lower labial mucosa, on the dorsum of the
tongue, on the left buccal mucosa and on the soft palate. These clinical features led us to consider as potential diagnosis
an immune-mediated muco-cutaneous bullous diseases. Red blood cells count, iron, folate and vitamin B12 were normal.
The auto-antibodies specific for the immune-mediated bullous diseases showed a positivity for anti-skin and anti-
desmoglein 3. The incisional biopsy of the lingual bulla confirmed the diagnosis of pemphigus. The patient started the treat-
ment with deflazacort tablets and clobetasol-based ointment for topical application on oral lesions, with poor clinical results.
Despite the patient refer us to suffer only of few gastrointestinal symptoms we decided to investigate for CD. Anti-transglut-
aminase antibodies were negatives; however, the CD-predisposing haplotype DQ8 was present. A duodenal biopsy con-
firmed the diagnosis of CD. The patient started the gluten-free diet with clinical improvement of the oral lesions.
Conclusions. Although the association between CD and phemphigus has not been widely studied, we think that
serological and genetic study for CD should be considered at least in complicated patients with oral and/or systemic
immune-mediated diseases.
References
• Pastore L, Carroccio A, Compilato D, et al. Oral manifestation of celiac disease. J Clin Gastroenterol 2008; 42:224-32.
• Mejri K, Abida O, Kallel-Sellami M et al. Spectrum of autoantibodies other than anti-desmoglein in pemphigus patients. J Eur
Acad Dermatol Venereol 2011; 25:774-81.
• Drago F, Cacciapuoti M, Basso M, et al. Pemphigus improving with gluten-free diet. Acta Derm Venereol 2005; 85:84-5.

Annali di Stomatologia 2013; Suppl. 2: 1-48 9