deamino-arginine-vasopressin and Histiocytosis--Non-Langerhans-Cell

Xanthoma disseminatum (XD) is a rare and potentially progressive non-Langerhans-cell histiocytosis. To date, a few cases of XD with spontaneous complete resolution have been described. The present report describes a 16-year-old girl who presented with yellow to red-brown papules and nodules on her eyelids, cheeks, axillae, back and buttocks. Indirect laryngoscopy showed multiple xanthomatous plaques on the larynx, posterior pharynx, epiglottis, and vocal cords. Additional findings were polyuria, polydipsia, and amenorrhea. Skin biopsy and electron microscopy results confirmed the diagnosis of XD. The patient was treated with fenofibrate, simvastatin, desmopressin, and sex-hormone replacement therapy. Her skin lesions began to slowly fade 6 years after disease onset, eventually resolving spontaneously and completely, but leaving an atrophic scar, frank anetoderma, and persisting diabetes insipidus. This case report together with a review of the English-language literature on the long-term follow-up of XD patients provides additional information on the natural history of this disease.

Topics: Adolescent; Amenorrhea; Anetoderma; Antidiuretic Agents; Biopsy; Cicatrix; Deamino Arginine Vasopressin; Dermatologic Agents; Diabetes Insipidus; Female; Fenofibrate; Gadolinium DTPA; Histiocytosis, Non-Langerhans-Cell; Hormone Replacement Therapy; Humans; Radionuclide Imaging; Remission, Spontaneous; Simvastatin

Topics: Adrenal Cortex Hormones; Adult; Biopsy, Needle; Brain Diseases; Brain Mapping; Deamino Arginine Vasopressin; Diabetes Insipidus; Disease Progression; Drug Therapy, Combination; Follow-Up Studies; Histiocytosis, Non-Langerhans-Cell; Humans; Immunohistochemistry; Magnetic Resonance Imaging; Male; Monitoring, Physiologic; Risk Assessment; Severity of Illness Index; Skin

Topics: Antineoplastic Combined Chemotherapy Protocols; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Disease Progression; Histiocytoma; Histiocytosis, Non-Langerhans-Cell; Humans; Infant; Male; Pituitary Gland; Skin; Skin Neoplasms; Treatment Outcome

Topics: Adolescent; Amnion; Carbamazepine; Conjunctival Diseases; Corneal Diseases; Cyclophosphamide; Deamino Arginine Vasopressin; Drug Therapy, Combination; Epilepsy; Eyelid Diseases; Histiocytosis, Non-Langerhans-Cell; Human Growth Hormone; Humans; Hydrocortisone; Hypopituitarism; Male; Microscopy, Acoustic; Suture Techniques

Topics: Deamino Arginine Vasopressin; Histiocytosis, Langerhans-Cell; Histiocytosis, Non-Langerhans-Cell; Humans; Infant; Lung; Male