Medical Care
Leukemia cutis is a local manifestation of an underlying systemic disease; therefore, it should be managed with systemic chemotherapy. Literature on aleukemic leukemia cutis is limited, but because diagnosis of leukemia cutis portends poor prognosis in acute leukemia, the treatment should be directed at eradicating the leukemic clone by using aggressive systemic chemotherapy and stem cell transplant possibly in first remission.
The treatment of leukemia should be determined by the subtype of leukemia and by the patient's ability to tolerate a treatment regimen. This is primarily dependent on the overall medical condition of the patient, including any comorbid conditions that may exist. Under certain circumstances, such as resistant or recurrent skin disease, local treatment in the form of electron beam therapy can be used. However, in most of these cases, reinduction systemic chemotherapy must be added unless medically contraindicated by the patient's comorbidity.
In general, most patients on chemotherapy should receive prophylaxis for common infectious agents, including herpes simplex virus (HSV), Candida species, and P carinii. They should also receive symptomatic treatment for mucocutaneous complications of chemotherapy. These treatments include ketoconazole troches (for thrush) and viscous lidocaine alone or a compound containing lidocaine solution, diphenhydramine hydrochloride, and aluminum hydroxide suspension, ie, "magic mouthwash," (for stomatitis) or chlorhexidine and may relieve symptoms. Symptomatic treatment, including topical steroids, mentholated lotions, or topical lidocaine preparations (Lida-Mantle), for medication- or radiation-induced skin eruptions is often helpful. Preparations containing hyaluronic acid or a medication containing emollients called Biafine can speed the healing of skin with radiation damage.
Patients are usually best managed by a team that includes dermatologists and hematologist and oncologist input.
Refer to Acute Myelogenous Leukemia and Acute Lymphoblastic Leukemia.
Radiation therapy
Radiation therapy can be used to palliate symptoms of pain and pruritus in patients with leukemia cutis who are not candidates for systemic chemotherapy. Addition of radiation therapy to skin lesions after complete response to systemic chemotherapy provides no additional benefit. In a rare instance, radiation may be used to treat isolated skin relapse when bone marrow shows complete remission and no other site of extramedullary relapse is evident. If these patients are subsequently treated with chemotherapy, reports detail severe radiation recall phenomenon occurring with chemotherapy drugs like cytarabine and clofarabine. [43] Treatment options for leukemia cutis are expanding rapidly. Helical irradiation of the total skin (HITS) therapy has been modified as simultaneous integrated boost (SIB)–helical arc radiotherapy of total skin (HEARTS) and used to treat acute myeloid leukemia in someone with disseminated leukemia cutis. [44]
Surgical Care
Most patients require central venous catheter placement for chemotherapy delivery, if they do not have one.
Consultations
Consult a hematologist and an oncologist if the patient presents with aleukemic leukemia cutis. Consult with a bone marrow transplantation physician if the patient is a candidate for such intensive therapy. Consider a consultation with a radiation oncologist for skin-directed therapy, if appropriate.
Diet
Neutropenic patients may be placed on diet of well-cooked foods with avoidance of deli meats, fresh fruits, and fresh vegetables to avoid associated risk of infection.
Activity
Patients may be cautioned to avoid strenuous activity.
Complications
Complications include the following:
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Infection: As previously mentioned, patients are at risk for a variety of opportunistic infections.
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Bleeding: Bleeding as a result of thrombocytopenia is common.
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Reactions to chemotherapy
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Graft versus host disease: Graft versus host disease following bone marrow transplantation is common.
Prevention
Patients should avoid crowded public places because of the risk of infection. Patients should avoid contact with others who are ill.
Patients should avoid receiving live vaccines. However, this may not apply under certain circumstances. Patients should avoid contact with persons who have been recently vaccinated with live vaccines.
Long-Term Monitoring
Further biopsies may be performed as required to rule out recurrences, graft versus host disease after bone marrow transplantation (BMT), or unusual infections resulting from immunosuppression. Appropriate prophylactic antifungal, antiviral, and Pneumocystis carinii pneumonia (PCP) agents and supportive growth factor treatment are needed.
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Involvement of the face in a patient with acute myelogenous leukemia. Courtesy of Grant Anhalt, MD.
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Red-brown papules can be seen in leukemia cutis. They are confluent in this patient. Courtesy of Nevena Damjanov, MD, and Elizabeth Prechtel.
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Papules and nodules on the face of an African American patient with acute myelogenous leukemia (AML). Courtesy of Mona Mofid, MD.
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A patient with typical plum-colored lesions seen in leukemia cutis. This patient had acute myelogenous leukemia. Courtesy of Grant Anhalt, MD.
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This photograph shows linear areas, which are more violaceous in color, likely due to trauma to the area, such as excoriation, which results in hemorrhage into the skin. Frequent hemorrhage into the skin can make any inflammatory skin lesion appear more violaceous in patients with leukemia. Courtesy of Nevena Damjanov, MD, and Elizabeth Prechtel.
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Low-power view of leukemia cutis acute myeloblastic leukemia (AML-M1). Note the perivascular and periadnexal infiltrate with relative epidermal sparing. Courtesy of Kim Hiatt, MD.
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This is a higher power view of leukemia cutis acute myeloblastic leukemia (AML-M1). This photo illustrates a perivascular infiltrate of leukemic cells. The nuclei are round to oval with little cytoplasm. Courtesy of Kim Hiatt, MD.
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Leukemia cutis of acute monocytic leukemia. Perivascular and periadnexal infiltration is also present, but the cell morphology is distinct. Many of the nuclei are folded or indented. The cytoplasm of the leukemic cells is gray-blue and more abundant than in the M1 subtype. Courtesy of Kim Hiatt, MD.
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Low-power view of acute promyelocytic leukemia cutis with a perivascular and periadnexal but also interstitial infiltrate, with epidermal sparing but significant upper dermal edema, which could be confused with Sweet syndrome at a low-power view. Courtesy of Kim Hiatt, MD.
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Acute promyelocytic leukemia cutis at high power. The round-to-indented nuclei with prominent cytoplasmic granules are evident. Courtesy of Kim Hiatt, MD.
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Photo illustrates leukocyte esterase staining of the cytoplasm of the leukemic cells in acute promyelocytic leukemia. Courtesy of Kim Hiatt, MD.
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Leukemia cutis at low power demonstrating a Grenz zone and intercalation of leukemic cells between collagen bundles. Courtesy of Keliegh Culpepper, MD.
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Infiltration of leukemic cells between collagen bundles.
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Infiltration of dermoepidermal junction by clonal T cells in Sézary syndrome.
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Diffuse macules and papules on the scalp of a patient with chronic myelogenous leukemia.
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Gingival infiltration in a patient with acute myelogenous leukemia.
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Diffuse truncal eruption of infiltrated papules and plaques in chronic lymphocytic leukemia.
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Close-up photo of diffuse truncal eruption of infiltrated papules and plaques in chronic lymphocytic leukemia.
Tables
Type of Leukemia |
Incidence in the United States |
Percentage of Patients with Leukemia Cutis (%) |
AML |
2.5 cases per 100,000 population |
13 |
Acute lymphocytic leukemia |
1.3 cases per 100,000 population |
3 |
Chronic myelogenous leukemia (CML) |
1-2 cases per 100,000 population |
2-8 |
Chronic lymphocytic leukemia (CLL) |
2.3 cases per 100,000 population |
8 |
Hairy cell leukemia |
0.6-2.9 cases per 1,000,000 population |
8 |
Adult T-cell leukemia |
Extremely low |
40-70 |
Cell Lineage |
CD Antigen Marker |
T cell |
CD45 (LCA) strongly positive CD45RO usually strongly positive CD3 positive but only scattered |
B cell |
CD20 strongly positive but scattered in normal B cells, weakly positive or negative in abnormal small B cells, positive in abnormal large B cells CD43 usually negative |
Granulocytes |
Lysozyme strongly positive in well and poorly differentiated granulocytes Chloroacetate esterase positive in well-differentiated granulocytes CD68 usually negative in all granulocytes CD43 positive, MPO sometimes positive |
Monocytes |
Lysozyme strongly positive in well and poorly differentiated monocytes Chloroacetate esterase usually negative CD68 positive in well-differentiated monocytes CD163 less sensitive but more specific than CD68 CD117 |
