Non-Langerhans cell histiocytoses comprise a heterogeneous group characterized by the proliferation of cells with macrophage features. Xanthogranuloma (XG) is the most typical clinical manifestation of this group and is normolipidemic. XG typically occurs in children, usually presenting as a single lesion, though it can manifest at any age. It is extremely rare for XG to occur in adults, where it typically presents as multiple lesions.
Both juvenile and adult forms of XG have been linked to several blood dyscrasias, although the adult variant has a more isolated history. Adults are infrequently affected, and when they are, it nearly always presents as a solitary tumor. The diversity of forms of XG makes it a unique condition.
We report the case of a middle-aged gentleman presenting with gradually enlarging multiple brown-to-yellowish cutaneous lumps on his arms and legs, without systemic manifestations. Histopathologic examination revealed yellowish lesions composed of sheets of foamy histiocytes and intervening collagen bundles, which were immunopositive for cluster of differentiation 68 (CD68), confirming the diagnosis of XG.
Although adult XG is not considered a paraneoplastic phenomenon, it is frequently associated with hematological malignancies. The likelihood of extracutaneous involvement remains uncertain, and the pathophysiology of the disease is poorly understood. This case of multiple adult xanthogranuloma (MAXG) adds to the scant literature and highlights the need for further research into this rare condition.
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