Images (3)
Xanthomatosis generalized planeE78.2 + Xanthomatose
Synonym(s)
Diffuse plane normolipemic xanthomas; Generalized Xanthelasma; Normolipemic plane xanthoma; xanthelasma corporis; Xanthelasma generalized; Xanthoma planum diffusum; Xanthomas diffuse plane normolipemic
DefinitionThis section has been translated automatically.
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.
LocalizationThis section has been translated automatically.
Neck, neck, hand lines, axillae, groins.
Clinical featuresThis section has been translated automatically.
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.
HistologyThis section has been translated automatically.
Foam cells in the upper and middle corium; electron microscopy: detection of Langerhans granules in single, non-lipidized histiocytes.
General therapyThis section has been translated automatically.
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 therapieThis section has been translated automatically.
Operative intervention is usually not possible due to the extent of the xanthomas. See below xanthelasma.
Progression/forecastThis section has been translated automatically.
Progressive clinical picture; only rarely spontaneous remissions.
LiteratureThis section has been translated automatically.
- Broeshart JH et al (2003) Normolipemic plane xanthoma associated with adenocarcinoma and severe itch. J Am Acad Dermatol 49: 119-122
- Winkelmann RK (1983) Normolipemic xanthoma planum and its associated syndromes. dermatologist 34: 159-163