ABSTRACT
Two children are described with the Sweet syndrome (acute febrile neutrophilic dermatosis), a rare skin disorder usually seen in middle-aged women. Typical features include spiking fever, neutrophilic leukocytosis, raised painful erythematous plaques and nodules reflecting a cutaneous dermal infiltrate composed of polymorphonuclear leukocytes and rapid resolution in response to systemically administered corticosteroid. The eruption is believed to represent a hypersensitivity reaction to antecedent infection or concurrent malignancy.
Subject(s)
Dermatitis/diagnosis , Child , Child, Preschool , Dermatitis/complications , Dermatitis/drug therapy , Epidermal Cells , Epidermis/pathology , Facial Dermatoses/complications , Facial Dermatoses/diagnosis , Facial Dermatoses/drug therapy , Female , Fever/complications , Fever/drug therapy , Humans , Infant , Leukocytosis/complications , Leukocytosis/drug therapy , Male , Middle Aged , Neutrophils , Parapsoriasis/complications , Parapsoriasis/diagnosis , Parapsoriasis/drug therapy , Prednisone/therapeutic use , SyndromeABSTRACT
Three unrelated children (one girl and two boys) have had since birth a syndrome characterized by a permanent skin rash which becomes more intense during flare-ups associated with fever, lymphadenopathy, splenomegaly, and arthritis symmetrically involving the large joints. In one boy, typical psoriasis was observed at age 3 years. In two patients, roentgenograms of the joints showed early patellar ossification and an abnormal epiphyseal appearance. The three children also had neurologic involvement, with mental retardation, enlarged head circumference, eye lesions, late closure of the anterior fontanel, and a chronic meningitis with infiltration by polymorphonuclear cells. No immunologic abnormalities were found, but polymorphonuclear cells infiltrated the skin, lymph nodes, synovial fluid, and CSF.