The Journal of Dermatology Vol.31 No.6

The Journal of DermatologyVol. 31: 503–505, 2004Letters to the EditorDiffuse Plane Xanthoma in a Patient withChronic Myeloid LeukemiaKey words: diffuse plane xanthoma; chronicmyeloid leukemiaTo the Editor:Diffuse plane xanthoma (DPX) was firstdescribed by Altman and Winkelman in1962 (1). They insisted that the features ofthis disease were xanthelasma palpebrarum,diffuse plane xanthoma of the head, neck,trunk, and extremities, and normal plasmalipids level (1–3). DPX is sub-divided intotwo groups, idiopathic and underlying disease-associated(1–3). To date, only one caseof DPX associated with chronic myeloidleukemia (CML) has been reported. Weherein describe a patient with DPX whichspread from xanthelasma palpebrarumafter detection of CML.An 84-year-old Korean woman presentedwith a three month history of progressive,diffuse, yellowish patches and plaques onthe face, neck, and upper chest. Four yearsearlier, she was diagnosed with CML andhad received chemotherapy with hydroxyurea.In this patient, it appeared that theCML had been activated immediately after asplenectomy, which was performed to treatabdominal pain with splenomegaly, becausethe compensated state had been broken.The yellowish patches on the periorbitalarea first appeared approximately 40 yearsearlier and had not changed until the detectionof the CML. After the activation of theCML, the yellowish patches on the periorbitalarea had become widespread to othersites on the face, including the forehead,cheek, and temple. Three months beforethe first visit, similar lesions developed onthe neck and upper chest. Some of these lesionswere slightly elevated. There was noitching or tenderness. Physical examinationrevealed multiple, relatively ill-defined, diffuse,yellowish patches and some plaques onthe periorbital area, cheek, forehead, neck,and upper chest (Fig. 1). Histopathologicfindings showed diffuse xanthomatous cells,some around blood vessels, in the upper tomid-dermis and subcutaneous fat tissues(Fig. 2). The complete blood count includedthe following values: hemoglobin, 10.7g/dl; white blood cell count, 11,500/mm 2 ,with 58% polymorphonuclear leukocytes,29% lymphocytes, 10% monocytes, and 2%eosinophils. The platelet count was14,700/mm 2 . Serum cholesterol and triglyceridelevels were normal, although the plasmaconcentration of high-density lipoprotein(HDL) was low (28 mg/dl, normalFig. 1. Multiple, relatively ill-defined, diffuse,yellowish patches and some plaques on the periorbitalarea, cheek, forehead, neck, andupper chest

504Kim et alFig. 2. Many xanthomatous cells in upper tomid-dermis (H & E, ×400).value; 45–65). A diagnosis of diffuse planexanthoma associated with CML was made.To date, most reported cases of DPX havebeen associated with underlying systemicdiseases. These include multiple myeloma,monoclonal gammopathy, leukemia, lymphoma,Sezary syndrome, Castleman’s disease,Waldenstrom’s macroglobulinemia,cryoglobulinemia, Langerhans cell histiocytosis,and POEMS syndrome (1, 2, 4–9).However, some authors have suggested thatthis phenomenon may be explained on thebasis of authors tending to report only thosepatients with a significant association. Patientswithout underlying disorders may beunder-reported (3, 9). Recently, Marcovel etal.(9) reported that only three of eight patientshad a reticuloendothelial disease andthat the true incidence of an underlying diseaseseemed to be lower than expected.However, patients with DPX must be clinicallyfollowed because this disease can precedethe occurrence of an associated condition.Various types of leukemia have beenknown to be associated with DPX; these includechronic myelomonocytic leukemia,chronic lymphocytic leukemia, chronicmonoblastic leukemia, chronic granulocyticleukemia, and CML (1, 2, 4–7, 9). Amongthese, only one case of CML associated DPXwas described in 1964 by Freud et al.(4).Our patient may be the second case of DPXwith CML.The pathogenesis of DPX remains unknown.However, some possible hypotheseshave been suggested (3, 7). In cases associatedwith monoclonal gammopathy, it is assumedthat paraprotein-lipoprotein complexesmay be recognized by scavenger receptorson macrophages, resulting in thedevelopment of cutaneous xanthomas (10).A second hypothesis is that DPX is a histiocytosis-derivedxanthomatosis in the spectrumof non-X histiocytosis (9). Otherwise,Vail et al.(7) suggested that the cells in theskin lesions represent direct cutaneous infiltrationby the same leukemic cells found inother organs. Our patient had had periorbitalxanthelasma for about 40 years. Theselesions had been silent until the CML wasdetected, and then they spread to other siteson the face, neck, and upper chest immediatelyafter the CML was activated. We thinkthat this clinical course may support theabove third hypothesis by Vail et al. Furthermore,this case also suggests that abruptspread of pre-existing xanthelasma shouldbe followed by full-examination for the detectionof any underlying systemic disease.Our case had a low level of high-densitylipoprotein (HDL) although her triglycerideand cholesterol levels were within normallimits. Although the exact roles of HDLin the xanthoma have not yet been established,HDL is thought to exert a protectiveeffect against tissue cholesterol deposition,and a low plasma level of HDL results in adefective removal of intracellular cholesterol(11). Therefore, it is not surprisingthat low levels of HDL have been reportedin some patients with xanthelasma andtuberous xanthoma (11). To date, however,there has been no research regarding therole of HDL in DPX. It would be worth examiningthe effects of HDL in the developmentof DPX.Kyoung Jin KimDeuk Pyo LeeHo Seok Suh*

Diffuse Plane Xanthoma with Chronic Myeloid Leukemia505Mi Woo LeeJee Ho ChoiKee Chan MoonJai Kyoung KohDepartment of DermatologyAsan Medical CenterUniversity of Ulsan College of MedicineSeoul, Korea*Department of DermatologyUlsan University Hospital, Ulsan, KoreaCorrespondence to: Kyoung Jin Kim, M.D.,Ph.D.Department of DermatologyAsan Medical CenterUniversity of Ulsan College of Medicine388-1, Poongnap-dong, Songpa-guSeoul 138-736, KoreaReferences1) Altman J, Winkelmann RK: Diffuse normolipemicplane xanthoma: Generalized xanthelasma, ArchDermatol, 85: 633–640, 1962.2) Lynch PJ, Winkelmann RK: Generalized planexanthoma and systemic disease, Arch Dermatol,93: 639–646, 1966.3) Akhyani M, Daneshpazhooh M, Seirafi H,Naraghi ZS: Diffuse plane xanthoma in an otherwisehealthy woman, Clin Exp Dermatol, 26:405–407, 2001.4) Freud P, Plachta A, Speer FD, Luhby AL:Leukemic xanthomatosis, Amer J Dis Child, 88:43–61, 1964.5) Stockman A, Delanghe J, Geerts M, Naeyaert JM:Diffuse plane normolipaemic xanthomatosis in apatient with chronic lymphatic leukaemia andmonoclonal gammopathy, Dermatology, 204:351–354, 2002.6) Derrick EK, Price ML: Plane xanthomatosis withchronic lymphatic leukemia, Clin Exp Dermatol,18: 259–260, 1993.7) Vail JT, Adler KR, Rothenberg J: Cutaneous xanthomasassociated with chronic myelomonocyticleukemia, Arch Dermatol, 121: 1318–1320, 1985.8) Chang SE, Choi JH, Sung KJ, Moon KC, Koh JK,Ro JY: POEMS syndrome with xanthomatouscells, Am J Dermatopathol, 21: 567–570, 1999.9) Marcoval JM, Moreno AM, Bordas X, Gallardo F,Peyri J: Diffuse plane xanthoma: Clinicopathologicstudy of 8 cases, J Am Acad Dermatol, 39:439–442, 1998.10) Loo DS, Kang S: Diffuse normolipidemic planexanthomas with monoclonal gammopathy presentingas urticarial plaques, J Am Acad Dermatol,35: 829–832, 1996.11) Parker F: Normocholesterolemic xanthomatosis,Arch Dermatol, 122: 1253–1257, 1996.