Farzad Nourollah-Zadeh
Eric T Stoopler
Thomas P Sollecito
Victoria P Werth
| Farzad Nourollah-Zadeh | Eric T Stoopler | Thomas P Sollecito | Victoria P Werth |
Farzad Nourollah-Zadeh
Eric T Stoopler, Thomas P Sollecito, Victoria P Werth
Oral Medicine, University of Pennsylvania School of Dental Medicine
Introduction
Pemphigus vulgaris (PV) is a chronic vesiculobullous disease with high morbidity and mortality. Involvement of the oral mucosa is common and typically precedes onset of cutaneous lesions. PV of the feet is uncommon possibly owing to lower expression of PV antigens at this site.
Methods
Case Summary A 64-year-old female complained of oral lesions of eight months duration. Prior to onset of oral lesions, two large, intact bullae developed on the left foot which was diagnosed as cellulitis and managed with antibiotics. Subsequently, the patient developed gingival bleeding and ulceration followed by cutaneous facial lesions, which was transiently managed with multiple, short-term courses of prednisone. Medical history included osteoporosis, vitiligo, and basal cell carcinoma on left face. Medications included multivitamins and no known drug allergies. Family history, social history and review of systems were non-contributory. Extraoral examination did not reveal lymphadenopathy, salivary gland enlargement, thyromegaly or cutaneous lesions. Intraoral examination revealed coated tongue. Clinical photos provided by patient demonstrated irregular ulcerations on the buccal mucosa and tongue. Differential diagnosis included bullous pemphigoid and mucous membrane pemphigoid. Gram stain of the tongue did not reveal yeast infection. Indirect immunofluorescence with monkey esophagus detected cell surface staining up to a titer of 1:512. Enzyme linked immunosorbent assay (ELISA) testing was positive for anti-desmoglein 3 only, with a value of 208 units (<37 units). Clinical and laboratory findings were consistent with PV. The patient subsequently re-developed oral lesions and failed topical therapies, which included dexamethasone 0.5mg/5ml rinses, fluocinonide gel 0.05% and nystatin rinses. She was referred to Dermatology for further evaluation and management of PV. Upon re-development of cutaneous lesions, a skin biopsy from the face revealed suprabasilar acantholysis. The patient experienced short-term benefit with prednisone and is currently managed with rituximab and fluocinonide gel 0.05% for oral lesions with substantial benefit.
Results
None
Conclusion
This is an unusual case of PV due to the rare presentation of intact pedal bullae as the initial manifestation of the disease and onset preceding development of oral lesions.