[Cutaneous granulomatous lesions in congenital immune deficiencies. 5 cases]

Cutaneous granulomatous lesions rarely occur in primary immunodeficiency syndromes.

We observed chronic granulomatous lesions on the skin of 5 children with inborn immunodeficiency syndromes. The deficiency was of the mixed type in all 5 cases and included major hypogammaglobulinaemia in 4. Erythemato-squamous infiltrated plaques were found in 4 children and erythematous nodules in the fifth. Extra-cutaneous lesions (cavum and rectocolon) occurred in 2 children. Search for an infectious cause was negative. Anti-tuberculosis drugs were tried in 3 children as a test regimen and were ineffective. Systemic corticosteroids gave major clinical improvement in 2 children.

Several pathogenic processes have been hypothesized to explain the development of granulomatous lesions in immunodeficiency syndromes. The action of an unknown infectious agent has been suspected. An intrinsic anomaly in immune function regulation, particularly in a disequilibrium in the complex cytokine network controlling the formation of granulomas could also be involved. Systemic corticosteroid therapy appears to be effective but must be given with caution in these patients with immune deficiency.