Cutaneous lymphohistiocytic infiltrates in patients with hypogammaglobulinemia

Six patients (5 to 15 years of age) with hypogammaglobulinemia had scattered papular lesions in association with widespread dermatitis. These disfiguring lesions were a portal of entry for secondary infections and led to social withdrawal. Biopsy specimens from the papules demonstrated dense lymphohistiocytic infiltrate limited to the dermis. Infectious causes (bacterial, fungal, and viral) were excluded. The skin lesions failed to respond to conventional treatment, including topical corticosteroids. Replacement therapy with monthly infusions of high doses of immune serum globulin resulted in gradual improvement and ultimate clearing of the lesions. This unusual skin abnormality, which is unresponsive to traditional treatment, may provide the earliest clue to the presence of hypogammaglobulinemia.