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Case Reports
. 2012 Jul 31;2(3):203a03.
doi: 10.5826/dpc.0203a03. Print 2012 Jul.

Systemic mastocytosis associated with chronic myelomonocytic leukemia and xanthogranuloma

Philipp Tschandl  1 , Leonhard Müllauer, Harald Kittler
Affiliations
Case Reports

Systemic mastocytosis associated with chronic myelomonocytic leukemia and xanthogranuloma

Philipp Tschandl et al. Dermatol Pract Concept. .

Abstract

A patient with a history of non-diagnostic bone marrow biopsies presented with a red to brown maculopapular rash on the back. Biopsies confirmed multiple xanthogranulomas as well as a mastocytosis. A consequently performed bone marrow biopsy verified a systemic mastocytosis and a chronic myelomonocytic leukemia (CMML) type I. HERE, WE DESCRIBE FOR THE FIRST TIME IN THE LITERATURE A PATIENT WITH THREE DISEASES OCCURRING SYNCHRONOUSLY: CMML, xanthogranulomas and systemic mastocytosis. Two of them at a time are known to be associated and may be indicative of a common progenitor cell.

Keywords: CMML; Systemic mastocytosis; leukemia; xanthogranuloma.

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Figures

Figure 1
Figure 1
Clinical presentation of the patient. A) A widespread erythematous macular and papular rash on the back of the patient. B) Close-up clinical image of a positive Darier sign.
Figure 1
Figure 1
Clinical presentation of the patient. A) A widespread erythematous macular and papular rash on the back of the patient. B) Close-up clinical image of a positive Darier sign.
Figure 2
Figure 2
Histopathologic images of the mastocytosis. A) Hematoxylin and eosin (H&E). B) CD117 / c-KIT—Immunohistochemistry. C) Tryptase. D) Giemsa.
Figure 2
Figure 2
Histopathologic images of the mastocytosis. A) Hematoxylin and eosin (H&E). B) CD117 / c-KIT—Immunohistochemistry. C) Tryptase. D) Giemsa.
Figure 2
Figure 2
Histopathologic images of the mastocytosis. A) Hematoxylin and eosin (H&E). B) CD117 / c-KIT—Immunohistochemistry. C) Tryptase. D) Giemsa.
Figure 2
Figure 2
Histopathologic images of the mastocytosis. A) Hematoxylin and eosin (H&E). B) CD117 / c-KIT—Immunohistochemistry. C) Tryptase. D) Giemsa.
Figure 3
Figure 3
Clinical and dermatoscopic view of the xanthogranuloma. A) Clinical overview. B) Clinical close-up of a papular lesion. C) Dermatoscopic image of the same lesion. The dermatoscopic pattern is yellow strucutureless, which is typical for xanthogranuloma.
Figure 3
Figure 3
Clinical and dermatoscopic view of the xanthogranuloma. A) Clinical overview. B) Clinical close-up of a papular lesion. C) Dermatoscopic image of the same lesion. The dermatoscopic pattern is yellow strucutureless, which is typical for xanthogranuloma.
Figure 3
Figure 3
Clinical and dermatoscopic view of the xanthogranuloma. A) Clinical overview. B) Clinical close-up of a papular lesion. C) Dermatoscopic image of the same lesion. The dermatoscopic pattern is yellow strucutureless, which is typical for xanthogranuloma.
Figure 4
Figure 4
Histopathologic images of the lesion shown in Figure 3. A) H&E: A collection of lipid-laden macrophages and multinucleated giant cells, mostly of Touton type, in the dermis. B) Immunohistochemistry for CD1a: The macrophages were positive for CD68 (not shown) but negative for CD1a.
Figure 4
Figure 4
Histopathologic images of the lesion shown in Figure 3. A) H&E: A collection of lipid-laden macrophages and multinucleated giant cells, mostly of Touton type, in the dermis. B) Immunohistochemistry for CD1a: The macrophages were positive for CD68 (not shown) but negative for CD1a.
See this image and copyright information in PMC

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