Biomaterial Database

Head and neck manifestations of histiocytosis-X in children. 1989

L J DiNardo, and R F Wetmore

Department of Otolaryngology and Human Communication, Children's Hospital of Philadelphia, PA 19104.

The clinical presentation and course of 100 children aged 6 months to 22 years who had histiocytosis-X and were treated at the Children's Hospital of Philadelphia from 1974 to 1987 were reviewed. Sixty-three percent of patients presented with complaints referable to the head and neck. Lesions of the skull occurred most frequently, followed by cephalic rash, osseous mandibular destruction, enlarged neck nodes, and gingival disruption. Overall, during the course of the disease, 82% of the children with histiocytosis-X developed head and neck manifestations. There were ten deaths in this series, all associated with diffuse or multifocal disease. Interestingly, four of these patients presented with cephalic rash as a harbinger of diffuse and eventually fatal illness. The method of diagnosis and management of histiocytosis-X is discussed.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D008297	Male		Males
D009333	Neck	The part of a human or animal body connecting the HEAD to the rest of the body.	Necks
D001847	Bone Diseases	Diseases of BONES.	Bone Disease,Disease, Bone,Diseases, Bone
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D005145	Face	The anterior portion of the head that includes the skin, muscles, and structures of the forehead, eyes, nose, mouth, cheeks, and jaw.	Faces
D005148	Facial Dermatoses	Skin diseases involving the FACE.	Favre-Racouchot Syndrome,Nodular Elastoidosis,Facial Dermatosis,Nodular Elastosis,Dermatoses, Facial,Dermatosis, Facial,Elastoidoses, Nodular,Elastoidosis, Nodular,Elastoses, Nodular,Elastosis, Nodular,Favre Racouchot Syndrome,Nodular Elastoidoses,Nodular Elastoses,Syndrome, Favre-Racouchot
D005260	Female		Females
D006646	Histiocytosis, Langerhans-Cell	A group of disorders resulting from the abnormal proliferation of and tissue infiltration by LANGERHANS CELLS which can be detected by their characteristic Birbeck granules (X bodies), or by monoclonal antibody staining for their surface CD1 ANTIGENS. Langerhans-cell granulomatosis can involve a single organ, or can be a systemic disorder.	Histiocytosis X,Langerhans-Cell Granulomatosis,Langerhans-Cell Histiocytosis,Hand-Schueller-Christian Disease,Hand-Schueller-Christian Syndrome,Hand-Schüller-Christian Disease,Hand-Schüller-Christian Syndrome,Hashimoto-Pritzger Disease,Histiocytosis, Generalized,Histiocytosis-X,Langerhans Cell Granulomatosis,Langerhans Cell Granulomatosis, Pulmonary,Langerhans Cell Histiocytosis,Letterer-Siwe Disease,Non-Lipid Reticuloendotheliosis,Pulmonary Histiocytosis X,Pulmonary Langerhans Cell Granulomatosis,Schueller-Christian Disease,Systemic Aleukemic Reticuloendotheliosis,Type 2 Histiocytosis,Aleukemic Reticuloendothelioses, Systemic,Aleukemic Reticuloendotheliosis, Systemic,Cell Granulomatoses, Langerhans,Cell Granulomatosis, Langerhans,Cell Histiocytoses, Langerhans,Cell Histiocytosis, Langerhans,Disease, Hand-Schueller-Christian,Disease, Hand-Schüller-Christian,Disease, Hashimoto-Pritzger,Disease, Letterer-Siwe,Disease, Schueller-Christian,Diseases, Hashimoto-Pritzger,Generalized Histiocytoses,Generalized Histiocytosis,Granulomatoses, Langerhans Cell,Granulomatosis, Langerhans Cell,Granulomatosis, Langerhans-Cell,Hand Schueller Christian Disease,Hand Schueller Christian Syndrome,Hand Schüller Christian Disease,Hand Schüller Christian Syndrome,Hashimoto Pritzger Disease,Hashimoto-Pritzger Diseases,Histiocytoses, Generalized,Histiocytoses, Langerhans Cell,Histiocytoses, Type 2,Histiocytosis X, Pulmonary,Histiocytosis, Langerhans Cell,Histiocytosis, Type 2,Langerhans Cell Granulomatoses,Langerhans Cell Histiocytoses,Letterer Siwe Disease,Non Lipid Reticuloendotheliosis,Non-Lipid Reticuloendothelioses,Reticuloendothelioses, Non-Lipid,Reticuloendothelioses, Systemic Aleukemic,Reticuloendotheliosis, Non-Lipid,Reticuloendotheliosis, Systemic Aleukemic,Schueller Christian Disease,Syndrome, Hand-Schueller-Christian,Syndrome, Hand-Schüller-Christian,Systemic Aleukemic Reticuloendothelioses,Type 2 Histiocytoses