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Pyodermatitis-pyostomatitis vegetans is a rare mucocutaneous dermatosis often associated with gastrointestinal disorders, especially with inflammatory bowel disease. It is clinically characterized by erythematous lesions with multiple pustules and erosions affecting the mucosal surfaces. Cutaneous lesions are characterized by exudative and vegetating plaques affecting frequently the axillae and groins. The clinical diagnosis is supported by histologic findings, whereas immunofluorescence studies are useful to rule out other entities such as pemphigus3. Herein we report the case of a young man who was misdiagnosed as having IgA pemphigus for 8 years due to positive immunofluorescence findings. The clue for the final diagnosis was the diagnosis of a concomitant ulcerative colitis, which prompted us to reconsider his cutaneous disease.

In 53.4% of the patients, the use of chrysotherapy resulted in the complete clearing of the disease, discontinuation of all systemic therapies and induced a long-term clinical remission. Prednisone doses were able to be reduced in the remaining 46.6%. Any side-effects were reversible with drug discontinuation. Gold therapy showed efficacy as a secondary line treatment in refractory pemphigus vulgaris.

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