Chronic granulomatous disease accompany with ichthyosis

Chronic granulomatous disease, is a genetic disease characterized by a disorder of oxidative metabolizm in cells capable of phagocytosis such as neutrohils, machrophages and eosinophils. Its incidence is 1/1.000.000 and more commen in males. Most of the patients present within the first year especially first 3 months of their life with soft tissue infection, lymphadenitis and abscesses in liver and spleen.
4 year old male child attended to our hospital with complaint of swelling on the neck and fever. His past medical history included common recurrent exudative lesions at various parts of his body and he had been investigated for neck mass a few times. His physical examination yielded right cervical lymphadenomegali 5x5 cm diameter, fixed to underlying tissue and dryness and fissures on the lower extremites. He was diagnosed as chronic granulomatous disease and ichthyosis vulgaris after laboratury and clinical workup and his lymphadenitis was controlled by anthbiotic therapy.
Chronic granulomatous disease should be considered in the differential diagnosis of recurrent lymphadenitis and though very rare it may accompany diseases with neutrophil disfunction such as ichthyosis, lazy-leukocyte syndrome, Cheidak-Higashi syndrome and Job syndrome.

Kronik granülomatoz hastalýk ve iktiyozis birlikteliði