A Case of Diffuse Normolipemic Plane Xanthoma Associated with Multiple Myeloma / 대한피부과학회지

Diffuse plane xanthoma (DPX) presents with symmetric yellow-orange plaques primarily on the neck, upper trunk, flexural folds, and the periorbital region. Based on serum lipid and lipoprotein levels, these xanthomas are classified as normolipemic or hyperlipoproteinemic DPX. Diffuse normolipemic plane xanthoma (DNPX) is a rare condition that is not well studied yet. It is associated with reticulo-endothelial diseases, particularly multiple myeloma and monoclonal gammopathy of unknown significance (MGUS). A 62-year-old woman developed yellowish hyperpigmented papules and diffuse patches in the medial canthal area of her neck. Based on a skin biopsy and laboratory analyses, she was diagnosed with DNPX associated with multiple myeloma. This diagnosis demonstrates that dermatological lesions should be carefully assessed as they may be the first manifestation of an underlying hematological disease. We report herein a rare case of diffuse plane xanthoma associated with multiple myeloma and review the relevant literature.

Diffuse Normolipemic Plane Xanthoma with Multiple Myeloma / 대한피부과학회지

Diffuse plane xanthoma (DPX) is a rare variant of plane xanthoma and DPX appears as yellow flat patches or slightly elevated plaques on the eyelids, neck, upper trunk and flexural areas. Lynch and Winkelmann divided it into two groups according to the presence of hyperlipidemia. Diffuse normolipemic plane xanthoma is often associated with reticuloendothelial proliferative disease such as multiple myeloma and monoclonal gammopathy with unknown significance (MGUS). We report here on a case of diffuse normolipemic plane xanthoma associated with multiple myeloma in a 59 year-old man.

A Case of Diffuse Plane Xanthoma / 대한피부과학회지

Diffuse plane xanthoma (DPX) was first described by Altman and Winkelmann in 1962. Patients with DPX usually exhibit yellow, flat or slightly elevated plaques that gradually develop on the eyelids, neck, upper trunk, and flexures. Histopathologically, histiocytic foamy cells diffusely spread in the upper dermis with an admixture of lymphocytes and histiocytes. This disease may occur with either hyperlipoproteinemic or normolipoproteinemic states. DPX with hyperlipoproteinemia is associated with familial hyperlipidemia or liver cirrhosis. DPX with normolipoproteinemia is often associated with hematological problems such as multiple myeloma, lymphoma, chronic lymphatic leukemia, chronic myeloid leukemia, and chronic myelomonocytic leukemia. We report a case of diffuse plane xanthoma with hyperlipoproteinemia.

A Case of Diffuse Plane Xanthoma Associated with Diffuse Large B Cell Lymphoma / 대한피부과학회지

Diffuse plane xanthoma is a group of plane xanthomas that present in yellow to yellowish-brown flat patches on the periorbital area, neck, upper trunk and flexural folds. It is rare and often associated with reticulo-endothelial malignancies such as multiple myeloma, monoclonal gammopathy of unknown significance and other lympho-proliferative disorders. We report a case of diffuse plane xanthoma associated with monoclonal gammopathy of unknown significance and diffuse large B cell lymphoma in a 75 year-old woman.

A Case of Diffuse Plane Xanthoma Associated with Monoclonal Gammopathy of Unknown Significance and Normolipoproteinemia / 대한피부과학회지

Diffuse plane xanthoma is a group of plane xanthomas that appear as yellow-brown flat patches or slightly elevated plaques with a wide-spread distribution. Cutaneous xanthomas may occur either hyperlipoproteinemic or normolipoproteinemic states. Diffuse plane xanthoma with normolipoproteinemia is often associated with multiple myeloma, other reticulo-endothelial malignancies and monoclonal gammopathy of unknown significance(MGUS). We report a case of diffuse plane xanthoma associated with monoclonal gammopathy of unknown significance(MGUS) and normolipoproteinemia.

Two Cases of Diffuse Plane Xanthoma

Diffuse plane xanthoma characteristically presents with asymptomatic, yellow-orange plaques that usually involve periorbital areas, neck, upper trunk and flexural folds. Al-though most cases of diffuse plane xanthoma are associated with multiple myeloma, several cases have been described with different lymphoproliferative disorders and other miscellaneous diseases. We report herein two cases of diffuse plane xanthoma which are not associated with underlying disease.