Unilesional pemphigus vulgaris of the scalp after cochlear implantation.

In December 2004, a 69-year-old man came to the ENT unit of the University of Parma complaining of a painful cutaneous lesion in the left retroauricular region. The patient reported that the lesion appeared 4 weeks after a cochlear implantation procedure performed elsewhere 2 months before because of a bilateral profound deafness. The lesion was initially managed by his practitioner with oral and topical antibiotics in the suspicion of impetigo. Nevertheless, no benefits came after a 15-day antibiotic course. The patient's medical history included diagnosis of oral pemphigus vulgaris (PV) 6 years before that was treated with oral azathioprine and topical clobetasol dipropionate ointment. After complete clinical remission of the disease, a low-dose immunosuppressive therapy was prescribed for the patient, which was stopped 1 month before cochlear implant surgery. Clinical ear, nose, and throat examination documented 2 erosive areas located in the left retroauricular region along the surgical scar of the cochlear implant, extending several centimeters around it. No other similar lesions were documented on the rest of the skin; no erosive mucosal lesions of the nasal cavities, oral cavity, pharynx, and larynx were found on flexible fiber-optic endoscopy. No palpable neck masses were present, and complete blood cell count was normal. Given the history of PV, a perilesional biopsy was performed in the suspicion of a reactivation of the disease. Histopathologic evaluation showed isolated basal keratinocytes in a “tombstone” fashion, suprabasal cleavage of the epidermis, and acantholytic cells. These clues were also evident in the follicular structure, which presented acantholysis of the outer root sheath epithelium. Indirect immunofluorescence demonstrated focal fluorescence between keratinocytes at a dilution of 1/80, thus confirming the diagnosis of PV. Results of hematologic investigations and total-body computed tomography scan, performed to reveal a possible paraneoplastic origin of PV, were all negative. The patient was treated with oral methylprednisone, 1 mg/kg of body weight, and oral azathioprine, 150 mg/d, with rapid improvement of the lesion. Complete clinical resolution was reached in 5 weeks, and therapy was consequently tapered. After 12 months from treatment institution, the result of indirect immunofluorescence was negative; and the patient was well and free from PV under low-dose oral methylprednisone and azathioprine.