JUVENILE PEMPHIGUS FOLIACEUS TREATED WITH SUBLESIONAL CORTICOSTEROIDS

In May 1990, at the age of 16, a Caucasian non-Jewish man presented wi th superficial erosions on the chest, back, and neck. Examination at t hat time revealed superficial multiple flaccid bullae 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 intraepider mal vesicle with an infiltration of polymorphonuclear leukocytes, He w as treated with fluorinated topical corticosteroids. On a follow-up vi sit in August 1990, there were no active lesions on the chest, back an d 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 th e chest wall, in November 1990, revealed an intraepidermal separation in the upper malpighian layers associated with acantholytic cells. The re was a moderately dense lymphocytic infiltrate in the underlying der mis, A direct immunofluorescence study of the perilesional skin demons trated the presence of immunoglobulin G (IgG) and C3 in the intercellu lar space of the upper stratum malphigii epidermis. Indirect immunoflu orescence using monkey esophagus as substrate demonstrated a pemphigus antibody titer of 1:160. A serologic study by immunoblot analysis usi ng 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 corticos teroid creams, In addition, the areas were treated with sublesional in jections of triamcinalone acetonide at a concentration of 10 mg/cm(3) and a total of 2 cm(3) 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 cm(3) of 10 mg/cm(3) of triamcinalone a cetonide was injected. The volume injected depended on the number of l esions. The lesions showed healing and re-epithelialization within 10 days of injection. Most lesions responded to one injection, but some r equired 2-3 sessions of injections, When lesions were injected for the second or third time or subsequently, the concentration was reduced t o 7.5 mg/cm(3) or 5 mg/cm(3). The frequency of sublesional corticoster oid (SLCS) injection continued to decrease with the passage of time. B y June 1991, significant clearing of the lesions was observed, and, by September 1991, topical and sublesional injection therapy was discont inued, Thereafter, the patient developed scattered sporadic lesions on the torso, scalp, and extremities. These lesions were secondary to tr auma suffered during sports and athletic activities. The lesions would heal spontaneously and occasionally required sublesional injection. I n February 1992, a mild recurrence of lesions was observed on the scal p and trunk. The lesions were similarly treated with SLCS injections a nd topical therapy and promptly responded. The patient had been regula rly followed at 3-3 1/2-month intervals. Occasionally, an isolated les ion has been observed. Most lesions have responded to topical and/or S LCS therapy. Since January 1993 the patient has not had any lesions. H e has grown 9 in in height over the last 6 years. He has finished coll ege education and is in overall good health. He has never received any oral corticosteroid therapy. No obvious local or systemic side-effect s have been observed with SLCS injection, The areas of involvement ini tially showed post-inflammatory hyperpigmentation, which has subsequen tly disappeared.