Skin Infiltration of Juvenile Myelomonocytic Leukemia

Abstract

We described a two-year-old boy who developed a skin infiltration from JMML. Several indurated erythematous lesions were seen on his back on his first visit to our department. Edematous erythemas had repeatedly appeared on his auricles and feet for the previous six months. He had had a high fever for a month. Hepatosplenomegaly and superficial lymphadenopathy were recognized. Laboratory investigation showed leukocytosis and anemia. The diagnosis of JMML was confirmed by the findings of myeloid hyperplasia in his bone marrow and the spontaneous colony formation and GM-CSF hypersensitivity in a culture of bone marrow cells. Histopathologically, large atypical mononuclear cells were infiltrated throughout the dermis in a perivascular and interstitial distribution in a skin biopsy specimen. These cells were CD3 (-), CD20 (-), CD45 (+), CD68 (+) and myeloperoxidase (+). Bone marrow transplantation and then cord blood stem cell transplantation were performed but soon rejected. The indurated erythematous lesions appeared again soon after the relapse of JMML. There are other reported cases of JMML with skin infiltration that preceded any other manifestations of the disease. JMML cells in some patients, including our case, seem to have a great affinity for the skin, and skin biopsy aids in early detection of this disease.

Juvenile myelomonocytic leukemia characterized by cutaneous lesion containing Langerhans cell histiocytosis-like cells

“…Skin lesions are reported to appear in 36-48% of patients [2,3], and most of them have been reported as reactive lesions, referred to as ''leukemids'', neurofibromatosis, and xanthogranuloma [10,11]. On the other hand, several cases with leukemic skin infiltration have been reported [12,13]. In these case reports, leukemic cells were positive for CD14, CD36, CD68, lysozyme, myeloperoxidase, and XIIIa by immunohistochemical studies.…”

Histologic Findings in Skin Biopsy in a JMML Rash: A Case Report and Review of Literature

“…Rashes, commonly of the maculopapular type, frequently accompany JMML. Although such rashes have been well documented in the literature, there are only few case reports in which the rashes have actually been biopsied and studied adequately by IHC (Table 1) [2–6]. In this case report, we describe the immunophenotype results of one such biopsy and review previous cases of skin biopsies with JMML involvement.…”

Chilblain‐like Leukemia Cutis

“…A chilblain‐like eruption due to leukemia cutis has been reported in a 70‐year‐old man as a diagnostic clue to the blast crisis of chronic myelocytic leukemia (6). It should be noted that leukemia cutis can also be fleeting; a recurrent edematous erythema of the ears and feet of a 2‐year‐old boy due to skin infiltration of JMML has also been reported (7).…”