LUPUS LIKE LESIONS IN A PATIENT WITH X-LINKED CHRONIC GRANULOMATOUS-DISEASE AND RECOMBINANT X-CHROMOSOME

Lupus-like lesions in X-linked chronic granulomatous disease (X-CGD) a re rare. To our knowledge, only 2 cases have previously been published . We report a 2.5-year-old boy with X-CGD whose clinical findings were consistent with cutaneous lupus erythematosus. Conventional histopath ology showed epidermal atrophy, parakeratosis, follicular plugging and areas of hydropic degeneration. The most striking feature was a neutr ophilic interstitial infiltrate with leukocytoclasia in the upper derm is. The X chromosome of our patient - studied with 2 endonucleases (Ps tI and TaqI) and 5 probes (P99.6, pERT 87.8, pERT87.15, XJ1.1 and 754) - was ncombinant, but we believe that this is an incidental finding, not related to the disease. Neutrophilic infiltrate and leukocytoclasi a could be characteristic histopathologic findings of lupus-like lesio ns in these patients.