OMENNS SYNDROME

A 7-month-old boy presented with a 6-month history of a skin eruption of the scalp and the diaper area (Pig. 1) resembling seborrheic dermat itis. Since the age of 3 months, he had developed high grade fever, di ffuse pruritic skin lesions, stomatitis (Pig. 2), chronic diarrhea, di ffuse lymphadenomegaly, and failure to thrive. The parents were noncon sanguineous and no similar disease had ever been reported in the famil y. Physical examination showed alopecia, generalized lymphadenopathy, and hepatosplenomegaly. The patient's skin was dry with generalized er ythematous, scaling pruritic eruptions and small violaceous nodules on the extremities. Laboratory investigations revealed: slight normochro mic anemia, leukocytosis (18,000/mm(3)) with lymphocytosis (42%), and mild eosinophilia (12%); decreased levels of all classes of serum immu noglobulins were observed with normal IgE levels. Examination of phago cytosis, opsonization, and granulocyte chemotaxis revealed no abnormal ities. The number of circulating T cells was within normal limits, whe reas that of B cells was low. Recall antigens and T- and B-lymphocyte proliferative response to mitogens and Candida albicans proteins resul ted in poor stimulation. Chromosomal analysis of mononuclear cells fro m the peripheral blood and skin fibroblasts showed a normal male Kario type, and HLA typing did not reveal any maternal chimerism. A skin bio psy specimen showed satellite cell necrosis of Keratinocytes and a den se lymphohistiocytic and eosinophilic perivascular infiltrate in the d ermis. A specimen from a lymph node showed a picture consistent with d ermatopathic lymphadenopathy, with an increase of cells with histiocyt ic appearance and a dense infiltrate of eosinophils. On electron micro scopic examination, no cells with Birbeck's granulations were found in the skin infiltrate and lymph nodes. A bone marrow biopsy was normal. One month later the patient developed generalized exfoliative erythro derma with recurrent cytomegalovirus infections and staphylococcal sep ticemia. Despite antimicrobial and prednisone therapy, the patient die d of toxic shock at the age of 9 months. An autopsy showed diffuse lym phohistiocytic infiltration involving the skin and lymph nodes and ext reme lymphocytic depletion in the thymus. Meningitis and pneumonia wer e also present.