Cutaneous Presentation of Juvenile Chronic Myelogenous Leukemia: A Diagnostic and Therapeutic Dilemma

Abstract

Annular, erythematous, circinate plaques were the first manifestation of juvenile chronic myelogenous leukemia (JCML) in an otherwise healthy 2.5-year-old boy who had had these lesions since 6 months of age. The lesions showed an atypical hematopoietic infiltrate on biopsy. Biopsy of a bone marrow specimen and peripheral blood smear were normal six months before leukemic transformation. At 3 years of age the boy developed splenomegaly, thrombocytopenia, and petechiae, and a bone marrow aspirate and cell marker studies were regarded as consistent with, if not diagnostic of, JCML. Four previous cases of cutaneous leukemic infiltrate associated with JCML have been published. Our patient had recurring urticarial-like plaques for two years before the initial bone marrow finding of JCML. Given the poor prognosis and progressively evolving course of JCML, it may be appropriate to consider therapy before bone marrow changes, based on the presence of the cutaneous eruption with the appropriate findings on skin biopsy and an elevated fetal hemoglobin.

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

“…Objectively, JMML is related to organ infiltration, such as the liver and spleen, and other frequent infiltration sites are the skin and lungs. 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].…”

Juvenile Chronic Myelogenous Leukemia

“…The first case demonstrates the importance of distinguishing JCML from Epstein-Barr virus infection, and the second case, which has been reported elsewhere, 10 is a Juvenile Chronic Myelogenous Leukemia good example of the diagnostic problems that arise when JCML presents in an extramedullary site such as in the skin.…”

Skin Infiltration of Juvenile Myelomonocytic Leukemia

“…Leukemic skin infiltration tends to be indurated erythema and often appears on acral regions, including the hands, feet, and ears (10). It is very interesting that eruptions due to skin infiltration preceded any other manifestation in the three cases reported by Heskel (7), Buescher (8), and Sires (9).…”

“…To our best knowledge, there have been only seven reported cases of JMML with skin infiltration of leukemic cells (6–11). In these cases, skin eruptions have been described as erythematous maculopapular lesions on the legs (6), erythematous indurated circular areas with central clearing on the abdominal wall and eczematous papular eruption on the trunk and arms (6), figurate, red, urticarial lesions on the trunk and arms (7), many annular erythematous lesions, measureing 2 to 3 cm predominantly on the legs and buttocks (8), annular, erythematous, circinate plaques on the trunk and extremities (9), and indurated erythema on the auricles and the extremities (10). One case was revealed to be skin infiltration by postmortem examination (11).…”