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Tuberculosis of the Skin

13-cis-retinoic acid has been found to inhibit the release of mediators of Inflammation by neutrophils. The proposed mechanisms include profound inhibition of superoxide anion production and lysosomal enzyme release.̂ With this in mind, we used 13-cis-retinoic acid in a case of pemphigus vulgaris (PV) in which severe steroid-induced complications had developed to diminish the inflammation and for its steroidsparing effect. A 29-year-old woman was referred to the University of Michigan Dermatology Service with a 9-year history of PV. Her disease began during her first pregnancy at the age of 20 years, manifested by oral vesicles and erosions with subsequent clearing. There was a similar episode during her second pregnancy. At the age of 25 years during her third pregnancy, the appearance of oral lesions was followed for the first time by persistent vesicles involving her entire body. She was placed on prednisone (40 mg/day for 6 months, tapering to 10 mg/day over a course of 3 months). Over the course of 3 years, she developed severe cushingnoid features and vertebral compression fractures. Sulfasalazine without prednisone was not clinically or immunologically helpful. Physical examination revealed severe erosions and vesicles on her oral mucosa, trunk, and extremities. Other features included cushingnoid facies and habitus, severe steroid-induced abdominal striae, and a disproportionate trunk-to-extremity ratio consistent with vertebral compression fractures. Skin biopsy showed an intraepidermal vesicle with acantholytic cells. Direct Immunofluorescence showed IgG and complement deposited within the intercellular space (ICS) above the basal layer. Indirect immunofluorescence revealed circulating IgC at a titer of 1 :320 directed against the ICS of rat tongue epithelium. Spinal x-rays showed four vertebral compression fractures consistent with prolonged steroid usage. While in the hospital, she was started on 60 mg of prednisone, and topical steroids were applied to the erosions. The patient improved markedly over a course of 4 weeks, and at discharge, the erosions had completely healed with the exception of a few scattered erosions in the groin and perianal regions. On the day of discharge, the prednisone was decreased 40 mg/day and 13-cis-retinoic acid at a dose of 1 mg/kg (40 mg/day) was Instituted. As an outpatient she tolerated the 1 3-cis-retlnoic acid well. After 6 weeks, the dosage of retinoid was increased to 80 mg/day in an attempt to heal some persistent erosions and to prevent occasional new vesicle formation. Over the course of 2 months, there seemed to be no change in disease activity. Side effects included dry skin and severely chapped lips. With gradual lowering of her dose of prednisone to 20 mg/day, she developed numerous vesicles despite maintaining the 13-cisretinoic acid at 80 mg/day. The retinoid was stopped and the prednisone increased to 40 mg/day, with gradual subsiding of disease activity. It seems that 13-cis-retinoic acid was unable to induce or maintain remission In this patient despite doses that have been effective in retinoid-responsive diseases, such as nodulocystic acne and lamellar ichthyosis. Although it is possible that other retinoids, such as etretinate or arotinoid, may prove efficacious, it appears that 13-cis-retinoic acid may play a minimal role in the treatment of pemphigus vulgaris.