Association of Pemphigus Vulgaris and Herpes Simplex Virus Infection

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Abstract
A 39‐year‐old man developed sore throat with swallowing difficullies in late September 1983. A suspicion of foreign body in the esophagus was ruled out by esophagosc opy. At that time, the patient was in good general health, with normal body temperature. The only pertinent physical finding was a wide erosion over the right side of the palate, fie was admitted 3 weeks later to the otorhinolaryngology department at Rambam Medical Center because of a sudden worsening of his oral complaints and rise in body temperature to 38C with general malaise. On examination, there were wide erosions on the palate; and on laryngoscopy, the epiglottis showed edema, redness, and fibrin deposits. Total peripheral blood count showed hemoglobin 16 mg/dL and leukocytes 10.500/mm3, with a differential count of 75% polymorpho‐nuclears, 1% stabs, 11% lymphocytes, l% monocytes, 5% eosinophils, and 7% atypical cells. The results of the following routine blood examinations were normal: glucose, urea, electrolytes, calcium, phosphorus, alkaline phosphatase, creatinine, uric acid, albumin, globulin, and complement C3 and C4. Electrophoresisand immunoelectrophoresis of serum proteins and urinalysis results were within normal values. Antinuclear factor was negative. The diagnosis of acute epiglottitis was made, and the patient was treated by intravenous crystalline penicillin with only slight improvement. A week later, a blistering eruption appeared on the skin, and the patient was transferred to the dermatologic department for further investigation. On examination, the eruption consisted of flaccid vesicles, bullae, and erosions on the back, dorsa of hands, and inguinal folds; erosions of the oral mucosa were still present. Nikoisky sign was positive. A biopsy specimen from the skin showed a suprabasal blister, and the basal cells remain attached to the dermis, giving a “row of tombstones” appearance (Fig. 1). The diagnosisof pemphigus vulgaris was made, and the patient was put on an initial dose of daily 100 mg fluocortolone. After 2 weeks, the dosage was elevated to 150 mg per day, and 10 days later, improvement was achieved. The dosage of oral corticosteroids was cautiously tapered, and the patient was discharged at the end of November 1983.The patient was well until mid‐March 1984, on a maintenance dose of 50 mg fluocortolone every 48 hours. New oral lesions appeared suddenly, had a spontaneous remission a few days later, and reappeared again a week later, and the patient was readmitted to the dermatologit department. The fluocortolone was increased to a daily dose of 50 mg. The patient's general condition was good, with moon face and erosions on the oral mutosa and epiglottis. The relevant laboratory test results were leukocytes 10500/mm3, with a differential count of 76% polymorphonuciears, 3% eosinophils, 17% lymphocytes, and 4% atypical cells. Cytologic smears Irom oral erosions revealed acantholytic and multinucieated cells, some with inclusion bodies, HSV type 1 was isolated from oral smears. Serum antihodies against HSV were detected by complement fixation tesl in a liter of 1:40. During this hospitalization, a loose vesicle also appeared on the abdomen, which on histologic: examination showed a suprahasal blister, few acantholytic cells, and multinucleated cells in the detached epidermis (Fig. 2). Direct immunofluores‐cence studies showed a deposition of intercellular IgG. Circulating antiepithelial IgG antibodies were present in a titer of 1:40. Because of the herpetic infection and to enable discontinuation of corticosteroid treatment, gold therapy was started with a weekly dose of 50 mg aurothioglucose intra‐muscularly. A slow improvement was observed in the oral and epiglottal lesions. In mid‐June 1984, new vesicles appeared on the oral mucosa and HSV was again isolated; a spontaneous remission followed a week later. After a total dose of 500 mg of gold compound was reached, the dosage of corticosteroids was gradually discontinued.