Papular xanthoma E75.5

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 22.10.2022

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Synonym(s)

papular xanthoma

History
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Winkelmann, 1981

Definition
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Very rare, quite predominantly acquired, normolipemic, cutaneous non-Langerhans cell histiocytosis without any systemic involvement

Etiopathogenesis
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S.u. Non-Langerhans cell histiocytosis. Association with angiokeratoma fordyce has been described.

Manifestation
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Men are affected about 4 times more often than women.

Localization
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Trunk, extremities, more rarely face (nose region).

Clinical features
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Mostly solitary 0.1-1.2 cm large, rarely occurring in plural, red to red-brown, firm, asymptomatic papules with a smooth, shiny surface. As far as the sparse data available so far allow this statement, three forms of progression are distinguished:

Histology
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Dense, dermal, non-epidermotropic infiltrate under a regular epidermis with numerous xanthomatized macrophages (foam cells) mixed with numerous multinucleated giant Touton-type cells.

Immunohistology: Histiocytes pos. for KiM1p, neg. for factor VIII, S100 and CD1a.

Differential diagnosis
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Literature
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  1. Breier F et al (2002) Papular xanthoma: a clinicopathological study of 10 cases. J cutan Pathol 29: 200-206
  2. Caputo R et al (2003) Papular xanthoma associated with angiokeratoma of Fordyce; considerations on the nosography of this rare non-Langerhans cell histiocytoxanthomatosis. Dermatology 206: 165-168
  3. Winkelmann RK (1981) Cutaneous syndromes of non-X-histiocytosis. A review of the macrophage-histiocytic disease of the skin. Arch Dermatol 117: 667-672

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Last updated on: 22.10.2022