Correspondence

Aleukemic Leukemia Cutis in an Adolescent Boy

N Engl J Med 1999; 340:893-894March 18, 1999

Article

To the Editor:

Slightly elevated bluish-gray nodules developed on the lower legs of a 16-year-old boy in April 1997. The nodules were asymptomatic, and the boy was otherwise in good health. Examinations by various physicians yielded no diagnosis. The boy consulted our clinic because of rapid progression over the next four months. A physical examination disclosed numerous 1-to-4-cm bluish-gray nodules on the entire body surface but predominantly on the legs (Figure 1Figure 1Multiple Bluish-Gray Nodules on the Legs of a 16-Year-Old Boy.). The nodules were not pruritic, painful, or tender. The examination was otherwise normal. The erythrocyte sedimentation rate was normal, as were the complete blood count and the results of thoracic and abdominal computed tomography.

A histologic examination of biopsy specimens of several nodules revealed dense, patchy cellular infiltrates in the dermis and subcutaneous fat, especially in perivascular and periadnexal regions. The infiltrate was composed of medium-sized round-to-oval cells with hyperchromic, kidney-shaped nuclei. Immunochemical studies showed expression of myeloperoxidase and CD68 in 30 to 40 percent of the atypical cells and a proliferation rate of 30 percent. On the basis of these findings, we diagnosed cutaneous acute myelogenous leukemia of the myelomonocytic subtype (French–American–British classification M4). Bone marrow aspiration and biopsy as well as flow cytometry of bone marrow and peripheral blood revealed no leukemic cells. In contrast, cytologic and histochemical examinations of swabs and cytospin preparations of biopsy material disclosed blast cells of myeloid lineage (Figure 1, inset) indicative of aleukemic leukemia cutis. A cytogenetic analysis revealed no chromosomal abnormalities.

Leukemia may be accompanied by two types of skin changes: the specific infiltrates, characteristic of leukemia cutis, or the nonspecific infiltrates, called leukemids.1 Leukemia cutis usually occurs in patients with myeloid leukemia,2-4 especially the myelomonocytic and monocytic types of acute myeloid leukemia, in which it is strongly associated with leukemic involvement of other extramedullary sites.2,3 Leukemic changes in bone marrow or peripheral blood usually precede skin involvement.4 In rare instances, leukemia cutis is the first sign of leukemia, preceding the diagnosis of systemic leukemia by several months or even years.4 This condition, known as aleukemic leukemia cutis, has a poor prognosis in adults.4,5 Cases in children or adolescents are extremely rare, and no reliable data on prognosis are available.

The case presented here should remind physicians that unusual nodular skin eruptions may be a sign of leukemia, even when there are no changes in the peripheral blood and bone marrow. Immunohistochemistry is then necessary to confirm the diagnosis of aleukemic leukemia cutis.

Ralf Husak, M.D.
Ulrike Blume-Peytaki, M.D.
Constantin E. Orfanos, M.D.
Free University of Berlin, 12200 Berlin, Germany

5 References

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Citing Articles (5)

Citing Articles

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   Lyn M. Duncan, Johanna L. Baran, Judith A. Ferry. 2011. Cutaneous Lymphomas. , 281-326.
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   P Chris Anderson, Mitchell A. Stotland, James G. H. Dinulos, Ann E. Perry. (2010) Acute Lymphocytic Leukemia Presenting as an Isolated Scalp Nodule in an Infant. Annals of Plastic Surgery 64:2, 251-253
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   M. Fernández-Guarino, M.J. Blanchard Rodríguez, M. García-Cosío, A. Harto Castaño, P. Jaén Olasolo. (2007) Tumoración indurada y asintomática de tres semanas de evolución en paciente con leucemia mieloide aguda. Revista Clínica Española 207:6, 307-309
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   Fernando Arias, Rosa Vives, María Luisa Gómez-Dorronsoro. (2006) Cutaneous nodes in a patient with advanced papillary carcinoma of the thyroid. Clinical and Translational Oncology 8:9, 692-693
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   Cesare Massone, Andreas Chott, Dieter Metze, Katrin Kerl, Luigi Citarella, Esmeralda Vale, Helmut Kerl, Lorenzo Cerroni. (2004) Subcutaneous, Blastic Natural Killer (NK), NK/T-cell, and Other Cytotoxic Lymphomas of the Skin: A Morphologic, Immunophenotypic, and Molecular Study of 50 Patients. The American Journal of Surgical Pathology 28:6, 719-735
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