Leukemia Cutis

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INTRODUCTION Leukemia is a result of neoplastic proliferation of bone marrow-derived leukocytes, the majority of which are of B-cell origin. The disease may be subdivided into acute or chronic forms. The acute form presents with anemia, thrombocytopenia, hemorrhage, adenopathy, hepatosplenomegaly, and a rapidly fatal course. The chronic indolent form is often incidentally diagnosed following prolonged episodes of fever, weight loss, and infection. The acute leukemias are more likely to show eye involvement than chronic leukemias. Leukemia cutis may occur concurrently with bone marrow involvement, as an isolated site of relapse, or as the initial manifestation of leukemia. Leukemia cutis occurs in 25% to 30% of infants with congenital leukemia. In older persons the incidence of leukemia cutis at diagnosis is approximately 10% in acute myeloid leukemia and 1% in acute lymphoblastic leukemia. Between 75% and 90% of patients with leukemia will show eye or adnexal involvement at some stage of the disease.

CLINICAL PRESENTATION Leukemia cutis of the eyelid skin manifests most commonly as multiple 1 to 2.5 cm discrete nodules ranging from a solitary lesion to involvement of 70% of the body surface. These lesions rarely ulcerate, and may be associated with urticaria and pruritis. Lesions vary in color from blue to red, to purple to green, to brown, depending on the amount of myeloperoxidase present within the immature blast cells. Diffuse infiltration of the dermis by leukemic cells may be seen. Associated ocular involvement may include lesions of the retina, optic nerve, globe, or conjunctiva. The retina shows the most frequent clinical involvement in leukemia with hemorrhage, cotton-wool spots, venous dilatation, micro-aneurysms, and leukemic infiltrates.

Eyelid Infiltrative Disorders
(Courtesy of Charles S. Soparkar, M.D.) (Courtesy of Charles S. Soparkar, M.D.)

Associated orbital disease is not uncommon and presents with pain, lid edema, and exophthalmos. Systemic manifestations include purpura due to thrombocytopenia, urticaria, pruritis, erythema multiforme, leonine facies, alopecia, exfoliative dermatitis, and infection with opportunistic organisms may be seen. Death may result from infection or hemorrhage.

HISTOPATHOLOGY The appearance of the leukemic infiltrate is a reflection of the underlying form of leukemia.

An example of acute monocytic leukemia is illustrated, demonstrating neoplastic cells with folded, monocytoid nuclei.

Leonine Facies Differential Diagnosis

DIFFERENTIAL DIAGNOSIS The differential diagnosis includes syphilis, dermatitis, leprosy, erythema multiforme, exfoliative dermatitis, and infection.

TREATMENT The treatment of leukemia depends upon the stage of the disease, the cell type, site of involvement, and clinical symptoms. Systemic disease is best treated with chemotherapy, and bone marrow transplants following total irradiation of the myelopoietic tissues. Central nervous system leukemia may be treated with intrathecal methotrexate, and cutaneous nodules may be palliated with local X-ray irradiation. Death may result from infection or hemorrhage.

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