Juvenile pemphigus foliaceus treated with sublesional corticosteroids.

In May 1990, at the age of 16, a Caucasian non-Jewish man presented with superficial erosions on the chest, back, and neck. Examination at that time revealed superficial multiple flaccid bullkae and erosions on the upper chest, upper back, and neck. There were no oral, genital, or ocular lesions. A skin biopsy of a new lesion revealed an intraepidermal vesicle with an infiltration of polymorphonuclear leukocytes. He was treated with fluorinated topical corticosteroids. On a follow-up visit In August 1990, there were no active lesions on the chest, back and neck; however, in November 1990, a recurrence of flaccid bullae was noted on the upper back, upper chest, upper arms, and abdomen. Lesions were also observed in the axillae and groin. Biopsy of a lesion on the chest wall, in November 1990, revealed an intraepidermal separation in the upper malpighian layers associated with acantholytic cells. There was a moderately dense lymphocytic infiltrate in the underiying dermis. A direct immunofluorescence study of the perilesional skin demonstrated the presence of immunoglobulin G (IgG) and C3 in the intercellular space of the upper stratum malphigii epidermis. Indirect immunofluorescence using monkey esophagus as substrate demonstrated a pemphigus antibody titer of 1:160. A serologic study by immunobiot analysis using normal human epidermis as substrate demonstrated that the patient's serum bound to a 160 kD protein. The patient was treated with saline soaks to the involved area followed by application of topical corticosteroid creams. In addition, the areas were treated with sublesional injections of triamcinalone acetonide at a concentration of 10 mg/cm3 and a total of 2 cm3 was injected at various sites. Two weeks later the patient was injected at sites not previously injected. Thereafter, the patient was seen at 1 or 2–month intervals; often, but not always, at each visit a maximum of 2 cm3 of 10 mg/cm3 of triamcinalone acetonide was injected. The volume injected depended on the number of lesions. The lesions showed healing and re-epithelialization within 10 days of injection. Most lesions responded to one injection, but some required 2–3 sessions of injections. When lesions were injected for the second or third time or subsequently, the concentration was reduced to 7.5 mg/cm3 or 5 mg/cm3. The frequency of sublesional corticosteroid (SLCS) injection continued to decrease with the passage of time. By June 1991, significant clearing of the lesions was observed, and, by September 1991, topical and sublesional injection therapy was discontinued. Thereafter, the patient developed scattered sporadic lesions on the torso, scalp, and extremities. These lesions were secondary to trauma suffered during sports and athletic activities. The lesions would heal spontaneously and occasionally required sublesional injection. In February 1992, a mild recurrence of lesions was observed on the scalp and trunk. The lesions were similarly treated with SLCS injections and topical therapy and promptly responded. The patient had been regularly followed at 3–3½-month intervals. Occasionally, an isolated lesion has been observed. Most lesions have responded to topical and/or SLCS therapy. Since January 1993 the patient has not had any lesions. He has grown 9 in in height over the last 6 years. He has finished college education and is in overall good health. He has never received any oral corticosteroid therapy. No obvious local or systemic side-effects have been observed with SLCS injection. The areas of involvement initially showed post-inflammatory hyperpigmentation, which has subsequently disappeared.