Xanthomatosis generalized plane E78.2 + Xanthomatose

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 20.10.2022

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Synonym(s)

Diffuse plane normolipemic xanthomas; Generalized Xanthelasma; Normolipemic plane xanthoma; xanthelasma corporis; Xanthelasma generalized; Xanthoma planum diffusum; Xanthomas diffuse plane normolipemic

Definition
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Generalized xanthomatosis, but by definition without disturbance of lipid metabolism. According to recent findings, this systemic disease is classified as a histiocytosis(xanthogranuloma family).

For further information, see Diffuse normolipemic plane xanthomas below.

Localization
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Neck, neck, hand lines, axillae, groins.

Clinical features
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0.2-1.5 cm large, yellow to yellow-brownish, occasionally also purple, flat, moderately firm, shiny papules with smooth surface (no scale deposits), which tend to confluence.

Associations with plasmocytic immunocytomas are found in 40% of patients. Rarely are chronic or acute myeloid leukemias or B-cell lymphomas associated.

Frequently xanthelasma of the eyelids. No infestation of the mucous membranes.

Histology
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Foam cells in the upper and middle corium; electron microscopy: detection of Langerhans granules in single, non-lipidized histiocytes.

General therapy
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Check blood count, blood sedimentation and, if necessary, bone marrow biopsy, detection of monoclonal immunoglobulins, detection of osteolytic foci in the bone, treatment of the existing underlying disease in cooperation with internists.

Operative therapie
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Operative intervention is usually not possible due to the extent of the xanthomas. See below xanthelasma.

Progression/forecast
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Progressive clinical picture; only rarely spontaneous remissions.

Literature
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  1. Broeshart JH et al (2003) Normolipemic plane xanthoma associated with adenocarcinoma and severe itch. J Am Acad Dermatol 49: 119-122
  2. Winkelmann RK (1983) Normolipemic xanthoma planum and its associated syndromes. dermatologist 34: 159-163

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 20.10.2022