CUTANEOUS GRANULOMATOUS LESIONS IN PATIEN TS WITH PRIMARY IMMUNODEFICIENCY SYNDROMES

Introduction. Cutaneous granulomatous lesions rarely occur in primary immunodeficiency syndromes. Cases reports. We observed chronic granulo matous lesions on the skin of 5 children with inborn immunodeficiency syndromes. The deficiency was of the mixed type in all 5 cases and inc luded major hypogammaglobulinaemia in 4. Erythemato-squamous infiltrat ed plaques were found in 4 children and erythematous nodules in the fi fth. Extra-cutaneous lesions (cavum and rectocolon) occured in 2 child ren. Search for an infectious cause was negative. Antituberculosis dru gs were tried in 3 children as a test regimen and were ineffective. Sy stemic corticosteroids gave major clinical improvement in 2 children. Discussion. Several pathogenic processes have been hypothesized to exp lain the development of granulomatous lesions in immunodeficiency synd romes. The action of an unknown infectious agent has been suspected. A n intrinsic anomaly in immmune function regulation, particularly in a disequilibrium in the complex cytokine network controlling the formati on of granulomas could also be involved. Systemic corticosteroid thera py appears to be effective but must be given with caution in these pat ients with immune deficiency.