Xanthoma Disseminatum Presenting with Hoarseness

Document Type: Case Report

Authors

1 Department of Dermatology, Venereology and Leprology, JIPMER, Puducherry, India.

2 Department of ENT, JIPMER, Puducherry, India.

3 Department of Pathology, JIPMER, Puducherry, India.

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

Main Subjects


1. Ghorpade A. Xanthoma disseminatum with koebnerized pearly penile lesions in an Indian man. Int J Dermatol. 2009 ;48:996-8.

2. Mahajan VK, Sharma AL, Chauhan PS, Mehta KS, Sharma V, Sharma S. Xanthoma disseminatum: a red herring xanthomatosis. Indian J Dermatol Venereol Leprol. 2013 ;79:253-4.

3. Varotti C, Bettoli V, Berti E, Cavicchini S, Caputo R. Xanthoma disseminatum: a case with extensive mucous membrane involvement. J Am Acad Dermatol. 1991 ;25:433-6.

4. Oka M, Oniki S, Komatsu M, Ikeda T, Matsuo M, Miyamoto Y, et al. Xanthoma disseminatum with intracranial involvement: case report and literature review. Int J Dermatol. 2010 ;49:193-9.

5. Ansarin H, Berenji Ardestani H, Tabaie SM, Shayanfar N. Xanthoma disseminatum with tumor-like lesion on face. Case Rep Dermatol Med. 2014;2014:1-4.

6. Ozçelik U, Doğru D, Akçören Z, Coşkun T, Kaya S, Göçmen A. Xanthoma disseminatum: a child with respiratory system involvement and bronchiectasis. Pediatr Pulmonol. 2005 ;39:84-7.

7. Zelger B, Cerio R, Orchard G, Fritsch P, Wilson-Jones E. Histologic and immunohistochemical study comparing xanthoma disseminatum and histiocytosis X. Arch Dermatol 1992;128:1207-12. 

8. Attia A M, Bakry O A, Mohamed E E. Xanthoma disseminatum: A progressive case with multisystem involvement. J Postgrad Med 2014; 60: 69-71.

9. Khezri F, Gibson LE, Tefferi A. Xanthoma disseminatum: effective therapy with 2-chlorodeoxyadenosine in a case series. Arch Dermatol. 2011 ;147:459-64.