Xanthoma disseminatum

LAST UPDATED: Aug 02, 2021

Introduction

This is a rare non-familial disease, characterised by proliferation of non–Langerhans, histiocytic cells in which lipid deposition is a secondary event.

This chapter is set out as follows:

History
Clinical findings
Images
Management

History

Mainly affects children and young adults
Males are more commonly affected than females

Clinical findings

Distribution

Face, trunk and proximal extremities, especially at flexural sites
Symmetrical distribution

Morphology

Yellow-brown papules, and nodules
Lesions become confluent, especially in the flexures, to form xanthomatous plaques, which may then become verrucous

Additional features

In 30% of cases the mucous membranes are affected, including from the lips to upper respiratory tract
Meningeal involvement is common, leading to diabetes insipidus in up to 40% of cases, seizures and growth retardation
Other organ involvement is much less common

Clinical Images

Please refer to notes on image rights at bottom of the page with regards to individual image ownership.

Management

Xanthoma disseminatum is self-limiting but may persist for many years or even decades
Depending on symptoms, further investigations and a multi-disciplinary team approach may be needed
Skin lesions may be disfiguring - treatment with a carbon dioxide laser can have good results

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Image Rights - The PCDS would like to thank Dermatoweb, DermQuest (Galderma), and others who have contributed images. All named individuals and organisations maintain copyright for the relevant images.