myelomonocytic [5, 8] or chronic myeloid [6] leukemia. Vail et al. [5] reported a case of DNPX associated with chronic myelomonocytic leukemia and proposed that the cells in the skin lesions represent direct cutaneous infiltration by the same leukemic cells found in other organs. Kim et al. [6] reported a case of DNPX associated with chronic myeloid leukemia. Interestingly, the clinical features of the present case were very similar to those observed in the case described by Kim et al., namely: DNPX was associated with leukemia, the appearance of xanthelasma preceded, by a long period of time, the development of skin lesions at other sites, and the development of skin lesions at other sites was associated with leukemia progression. The present case, together with the case reported by Kim et al. [6], support the hypothesis proposed by Vail et al. [5] described above. In the present case, we speculate that the foamy cells in DNPX lesions are derived from abnormal monocytes that originated from myeloid progenitor cells. Therapies such as Erbium YAG laser and probucol have been reported to be effective for a limited number of DNPX lesions [9, 10]. However, the successful treatment of widespread DNPX lesions associated with hematologic disease has yet to be reported. We plan to treat the CMML-1 using hematopoietic stem cell transplantation in the present case.
Disclosure. Financial support: none. Conflict of interest: none. 1

Division of Dermatology, Division of Medical Oncology/Hematology, 3 Clinical Laboratory, Kobe University Hospital, 7-5-2 Kusunoki-cho, Chuo-ku, Kobe 650-0017, Japan 2

Masahiro OKA1 Atsuo OKAMURA2 Seiji KAWANO3 Takeshi FUKUMOTO1 Masanobu SAKAGUCHI1 Chikako NISHIGORI1

1. Altman J, Winkelmann RK. Diffuse normolipemic plane xanthoma. Arch Dermatol 1962; 85: 633-40. 2. Goldsmith LA. Xanthomatoses and lipoprotein disorders. In: Fitzpatrick TB, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz SI (eds). Dermatology in General Medicine, 6 th edn. McGraw-Hill, New York, 2003: 1466-74. 3. Burgdorf WHC, Zelger B. The histiocytoses. In: Elder DE, Elenitsas R, Johnson BL, Murphy GF (eds). Lever’s Histopathology of the Skin, 9th edn. Lippincott-Raven, Philadelphia, 2005: 681-703. 4. Brunning RD, Orazi A, Germing U, et al. Myelodysplastic syndrome/neoplasms. In: Swerdlow SH, Campo E, Harris NL, et al. (eds). World health organization classification of tumors. Pathology and genetics. Tumors of haematopoietic and lymphoid tissues. Lyon: IARC Press 2008; Vol 2: 88-107. 5. Vail JT Jr., Adler KR, Rothenberg J. Cutaneous xanthomas associated with chronic myelomonocytic leukemia. Arch Dermatol 1985; 121: 1318-20. 6. Kim KJ, Lee DP, Suh HS, et al. Diffuse plane xanthoma in a patient with chronic myeloid leukemia. J Dermatol 2004; 31: 503-5. 7. Loo DS, Kang S. Diffuse normolipidemic plane xanthomas with monoclonal gammopathy presenting as urticarial plaques. J Am Acad Dermatol 1996; 35: 829-32. 8. Marcoval J, Moreno A, Bordas X, Gallardo F, Peyrí J. Diffuse plane xanthoma: clinicopathologic study of 8 cases. J Am Acad Dermatol 1998; 39: 439-42. EJD, vol. 24, n◦ 1, January-February 2014

9. Lorenz S, Hohenleutner S, Hohenleutner U, Landthaler M. Treatment of diffuse plane xanthoma of the face with the Erbium:YAG laser. Arch Dermatol 2001; 137: 1413-5. 10. Miyagawa F, Fukumoto T, Kobayashi N, Asada H. Successful treatment of diffuse normolipemic plane xanthoma with probucol. Case Rep Dermatol 2013; 5: 148-51. doi:10.1684/ejd.2013.2256

Clinicopathological features and treatment of uremic calciphylaxis: a case series Calciphylaxis is a rare, life threatening necrosis of skin occurring predominately in end-stage renal failure (ESRF) [1]. A non-uremic form is also well described, defined as the absence of ESRF, renal transplantation, acute kidney injury requiring renal replacement therapy (RRT) and severe chronic kidney disease (CKD) [2]. Associations with the risk of calciphylaxis include high serum phosphate, calcium/phosphate product and aluminium, secondary hyperparathyroidism, warfarin and steroid use, obesity, liver disease, female sex, and hypoalbuminemia [3-5]. Mortality rates are 60-80% and death results mainly from sepsis [5]. Multiple therapies are described, however, the evidence base is poor and there are no reported randomised controlled trials (RCTs) in this disease. In this retrospective uremic calciphylaxis case series, we report clinicopathologic characteristics of patients treated in our centre over a 10-year period with the aim of identifying high risk features and potentially successful treatment strategies. Using a histopathology search, 15 cases of calciphylaxis presenting to Barts Health NHS Trust were identified over 10 years (2001 to 2011), with follow-up to November 2013. Clinical data were extracted from medical notes. All skin biopsies were re-examined by a dermatopathologist. All 15 patients (11 female, 4 male) had skin biopsies from clinically suspicious lesions, which were consistent with calciphylaxis. The mean age at diagnosis was 50 years (range 22-67 years). 13 patients had ESRF requiring RRT - 11 haemodialysis; 2 peritoneal dialysis. The mean time from dialysis commencement to diagnosis of calciphylaxis was 44 months (1-132 months). 5 patients had renal transplants (4 cadaveric; 1 allogeneic). 3 had failed grafts requiring RRT and 2 had CKD stage 3. All 15 had uremic calciphylaxis by definition [2]. Clinical presentations included necrotic ulceration, indurated plaques, hypopigmented papules and erythematous patches. All had leg involvement, 2 involved the buttocks, 1 the penis, 1 the abdominal wall and 1 the arms. Nine had distal disease, 5 proximal disease and 1 had both. 2 of 9 (22%) with distal disease died within 12 months, compared to 4 of 5 (80%) with proximal disease. Mean serum laboratory investigations with reference ranges were: Corrected calcium – 2.18 (2.12-2.65 mmol/L); phosphate – 1.73 (0.8-1.5 mmol/L); parathyroid hormone – 91 (1.1-6.8 pmol/L); calcium × phosphate product – 3.78 (