Diffuse normolipemic plane xanthoma

Altman and Winkelmann, 1962

"Normolipemic planar xanthomas with paraproteinemia" were first described by Altman and Winkelmann in 1962. It is an acquired, rare, plaque-shaped, diffuse (non-papular) clinical manifestation of the histiocytoses (belonging to the xanthogranulomas ), which may occur associatively with hematologic diseases (e.g., paraproteinemia).

Clinically, this systemic disease is characterized by symmetrical yellowish-orange patches or plaques (the changes are not small-focal papular) that occur preferentially on the neck, upper trunk, flexural folds, and periorbital region. The skin manifestations may cause significant itching. Their occurrence may be associated with hematologic disorders, particularly multiple myeloma and monoclonal gammopathy. Furthermore, the coincident occurrence of leukemias or lymphomas (e.g., mycosis fungoides) is observed.

multicentric reticulohistiocytosis, generalized eruptive histiocytomas, xanthoma disseminatum, juvenile xanthogranuloma.

Xanthoma disseminatum: onset as early as infancy; papular efflorescences that may aggregate into large humped palques.

treatment of a possible underlying hematological disease.

In individual cases, positive results could be achieved by applying a simvastatin ointment (Zha S et al. 2022).

1. Cohen YK et al (2015) Diffuse normolipemic plane xanthoma associated with monoclonal gammopathy. Dermatol Pract Concept 5:65-67.
2. Cota C et al (2020) Diffuse (Generalized) Plane Xanthoma Misdiagnosed as Carotenoderma: Usefulness of Reflectance Confocal Microscopy. Am J Dermatopathol 42:e167-169.
3. Gupta M et al (2019) Diffuse normolipemic plane xanthoma with underlying photosensitive dermatoses. BMJ Case Rep 12: e230720.
4. Momen S et al (2017) Diffuse normolipemic plane xanthoma on striae. J Eur Acad Dermatol Venereol 31:e450-452.
5. Silva DM et al (2010) Diffuse plane idiopathic normolipemic xanthoma with hiperesplenism. An Bras Dermatol 85:73-76.
6. Souza MCC et al (2020) Diffuse normolipemic plane xanthoma and hepatitis C: chance? An Bras Dermatol 95: 261-262. figs.
7. Verdolini R et al (2015) Yellow changes of the skin: a quiz diffuse plane normolipemic xanthomatos is associated with multiple myeloma. Acta DermVenereol 95:762-763.
8. Wiznia LE et al. (2017) Hepatitis C. virus and its cutaneous manifestations: treatment in the direct-acting antiviral era. J Eur Acad Dermatol Venereol 8:1260-1270.
9. Wollina J et al (2018) Diffuse normolipemic plane xanthoma (DNPX) of the neck without xanthelasma palpebrum. Open Access Maced J Med Sci 6:123-125.
10. Yoshimura T et al (1994) Generalized normolipemic plane xanthomatosis associated with relapsing polychondritis. Acat Derm Venereol 74: 221-223.
11. Zha S et al (2022) Topical simvastatin Improves Lesions of Diffuse Normolipemic Plane Xanthoma by Inhibiting Foam Cell Pyroptosis. Front Immunol 13:865704.