CUTANEOUS MANIFESTATIONS OF CHRONIC-GRANULOMATOUS-DISEASE - A REPORT OF 4 CASES AND REVIEW OF THE LITERATURE

Background: Chronic granulomatous disease represents a group of geneti c disorders in which impaired intracellular microbial killing by phago cytes leads to recurrent bacterial and fungal infections and granuloma formation. Cutaneous disease occurs in 60% to 70% of cases. The chara cteristic histologic finding of pigmented lipid macrophages in viscera l granulomas has not been described previously in the skin. Objective: Our purpose was to review our experience of skin disorders in chronic granulomatous disease. Methods: We studied the clinical and histologi c findings in four patients with chronic granulomatous disease and unu sual skin lesions, We reviewed the skin disorders seen in five additio nal patients with chronic granulomatous disease referred to the pediat ric dermatology clinic. The literature was reviewed for previously rep orted cutaneous manifestations of chronic granulomatous disease.Result s: A teenage boy with chronic granulomatous colitis had nonulcerating cutaneous granulomas from which no organisms were isolated. Histologic examination of both skin and bowel revealed the characteristic golden -yellow granular pigment in macrophages. A second boy had cutaneous as pergillosis involving the left foot; histologic examination revealed m acrophages containing yellow-brown pigment at the periphery of the gra nulomatous inflammation. Two children had vesicular skin lesions. Thes e lesions were recurrent in one boy for several years. In the second c hild they were associated with fatal intracranial and pulmonary infect ion. Histologic examination in both cases revealed a subcorneal polymo rphonuclear infiltrate and perivascular macrophages containing yellow- brown pigment. Cultures were either negative or revealed organisms tha t are normally nonpathogenic skin commensals, such as coagulase-negati ve staphylococci. Conclusion: The cutaneous manifestations of chronic granulomatous disease encompass a variety of infections and inflammato ry lesions. Diagnostic and therapeutic problems may arise because of d ifficulty in isolating a causative organism. The characteristic pigmen ted macrophages of visceral granulomas can also be found in skin lesio ns.