Xanthoma Disseminatum Presenting with Hoarseness

Abstract

Introduction: Xanthoma disseminatum (XD) is a rare, benign, non-Langerhans cell histiocytic disorder with unknown etio-pathology. It manifests with multiple, grouped, red-brown to yellow papules and nodules involving the skin, mucous membranes, and internal organs with a predilection for flexures and the face.

Case report: We report a patient who presented with disseminated xanthomatous papules and nodules involving the face, neck, trunk, axilla, groin, and oral cavity, along with hoarseness of voice. Video laryngoscopy revealed multiple yellowish nodules over the base of the tongue, vallecula, laryngeal surface of the epiglottis, ary-epiglottic folds, interarytenoid region, and subglottic region. Histopathology was suggestive of xanthoma disseminatum and the patient was treated with tablet acitretin 25mg daily for three months without any response. Following this, the patient was prescribed tablet thalidomide 100 mg daily without any significant improvement at the end of two months.

Conclusion: Xanthoma disseminatum is a very rare form of non-Langerhans cell histiocytosis that classically presents with cutaneous xanthomas, mucosal xanthomas, and diabetes insipidus. Hoarseness of voice due to lesions involving the larynx is a rare symptom. Because the disease has punctated, numerous relapses and causes morbidity to the patient, its multisystem manifestations have to be known. Therefore, xanthoma disseminatum has to be kept in mind as a differential diagnosis for hoarseness of voice.

Keywords: Hoarseness; Vocal cord; Xanthoma.

Fig 1

Multiple discrete yellow to reddish-brown papules and nodules present all over the anterior trunk with lesions over the bilateral shoulder coalescing to form plaques and giving a band like appearance.

Fig 2

Multiple yellow to reddish-brown papules and nodules grouped together over the left axilla.

Fig 3

Multiple yellow to reddish-yellow papules and nodules present over the laryngeal surface of the epiglottis, bilateral ary-epiglottic folds, interarytenoid region, and subglottic region.

Fig 4

A, Histopathological picture of skin biopsy specimen demonstrating diffuse dermal infiltration by histiocytes (H & E, 100 X). B, Higher magnifications demonstrating both histiocytes few of which are xanthomatized (H & E, 400X)