Aplasia cutis congenita is a rare disease with less than 300 cases reported in the literature. A patient with extensive involvement of the skin was observed for 25 years. During adolescence, a reactive depression related to the residual cicatricial alopecia on the left side of the scalp developed. The psychiatric distress disappeared after corrective surgery was performed. It is recommended that patients with this disorder be regularly observed to select cases appropriate for early plastic surgery.
Discoid lupus erythematosus (DLE)-like lesions and recurrent aphthous-like stomatitis have often been described in carriers of X-linked chronic granulomatous disease (CGD). The capacity of the polymorphonuclear leucocytes to reduce nitroblue tetrazolium (NBT) after stimulation with phorbol myristate acetate (NBT test), a function of normal oxidative metabolism, was determined in 34 patients with DLE of whom 17 also suffered from recurrent stomatitis. The NBT test turned out to be normal in all 34 patients, indicating that none of them were carriers of X-linked CGD. In spite of the negative results of this study it is recommended that all female patients suffering from DLE in combination with recurrent aphthous-like stomatitis are screened by means of the NBT test, because this examination is simple and inexpensive, and because of the importance of identifying carriers of CGD with a view to genetic guidance.
The treatment of onychomycosis has previously often been protracted and unsuccessful. Terbinafine has been shown to be effective in short-term regimens. In this double-blind, placebo-controlled study, 148 patients with toenail dermatophytosis were randomized to treatment with either 250 mg terbinafine daily or placebo for 3 months. An additional treatment was given for 3 months to patients whose infection had not responded. The patients were followed clinically and mycologically through 12 months. After 3 months 82% of the terbinafine-treated group, versus 5% of the placebo group, showed significant improvement, i.e. negative culture and growth of unaffected nail more than 2 mm (p = < 0.0001). After 12 months clinical and mycological cure was seen in 40% of the patients treated with terbinafine for 3 or 6 months, while 67-81% were clinically cured, but with positive microscopy. Side-effects occurred in 13.5% of the terbinafine group, versus 5.4% of the placebo group, and were mild. 250 mg terbinafine daily for 3 months was significantly more effective than placebo. The efficacy did not appear to improve with additional treatment for 3 months.
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