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Neonatal lupus erythemalosus (NLE) is an autoimmune disease whose major findings are skin lesions and congenital heart block. Affected infants have maternal, transplacentally acquired, autoantibodies to Ro/SSA, La/SSB, or U,-RNP antigens. Anti-Ro/SSA is the predominant autoantibody, present in about 95% of cases. Mothers of babies with NLE may be asymptomatic initially or may have Sjogrent syndrome, lupus erythematosus, overlap syndrome or, uncommonly, leukocytoclastic vasculitis. When evaluating a young woman with a cutaneous leucocytoclastic vasculitis, dermatologists should be aware of the possible presence of antibodies related to NLE. If any patient suffering a disorder related to NLE becomes pregnant, testing for autoantibodies and close obstetric prenatal care with fetal echocardiogram is necessary. In cases of fetal bradycardia, treatment with dexamethasone or betamethasone should be considered, as these drugs are accessible to the fetal circulation.

In 1995, a 30-year-old woman presented with a 6-day history of fever and pruritic pustules on her left hand. Physical examination revealed confluent pustules surrounded by erythema on the dorsal aspect of the left hand, palm, and wrist (Figure 1). There were no lesions in other locations. Her medical history was otherwise unremarkable. A complete laboratory blood cell count showed a slightly low white blood cell count (3600/μL [reference range, 4500-10 800/μL]; to convert to 10/L, multiply by 0.001). Findings from biochemical tests were within reference range. Cultures of the pustules were negative for organisms. Findings from skin biopsy specimens showed an intraepidermal pustule with mild acantholysis and moderate mononuclear infiltrate in dermis. A diagnosis of localized pustulosis was made. The patient was initially treated with topical and oral antibiotics with poor response. After a month, oral retinoids and topical steroids were added to her treatment, but the patient experienced new flares. Seven months after the onset of the process, she complained about arthralgias and chest and back pain. Imaging studies (radiography, computed axial tomography) revealed sacroileitis. A diagnosis of incomplete SAPHO syndrome (sacroileitis, acne, pustulosis, hyperostosis, osteitis) was made. Therapy was begun with methotrexate, 7.5 mg/wk, and the dosage was subsequently increased to 10 mg/wk. Two months later there was no improvement, so oral cyclosporine was added to her drug regimen but was poorly tolerated because of headache. During the following 4 years the patient subsequently failed treatment with sulfone, prednisone, colchicine, sulfasalazyn, and numerous nonsteroidal antiinflammatory drugs (NSAIDs).

REPORT OF A CASE A 28-year-old heterosexual man was found to be seropositive for the human immunodeficiency virus (HIV) in 1990. The patient had recurrent perianal ulcerations and vesicular periungual lesions that were treated with intermittent courses of oral acyclovir. His clinical history was relevant for oral candidiasis. Medications taken included didanosine, sulfamethoxazole, trimethoprim, fluconazole, and ethambutol hydrochloride. When the patient was seen initially in November 1994, the painful perianal ulceration was persistent for the previous 2 months and unresponsive to oral acyclovir treatment. The 18-cm perianal ulcer had a vesicular border (Figure 1). The CD4 cell count was 0.08× 109/L (80/μL). Initial treatment consisted of intravenous acyclovir (10 mg/kg for 8 hours for 21 days) without result. The skin biopsy taken from the active border of the lesion showed the presence of epidermal multinucleated giant cells and intranuclear inclusions consistent with herpes simplex virus (HSV) infection, without