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© 2000 American Society for Clinical Oncology
Unusual Manifestations of Acute LeukemiaArthur Skarin MD Consultant Editor
Klinikum der Universität Regensburg, Regensburg, Germany CASE 2. LEUKEMIA AND RASH: PARANEOPLASTIC OR DRUG-INDUCED? A 57-year-old man presented with a 5-week history of fatigue, anemia, and loss of weight. Physical examination revealed pale erythematous, nonpruritic plaques confined to the neck (Fig 1). The blood count showed anemia (hemoglobin level, 7.8 g/dL) and leukocytosis (19.9/nL) with 16% blasts. A bone marrow aspirate was diagnostic of acute myelogenous leukemia (AML-M0). Five days after completing induction chemotherapy with thioguanine, cytarabine, and daunorubicin, he developed fever and extension of the erythema to the face, neck, and chest with increasing purpura. The differential diagnosis for this rash included drug hypersensitivity, urticaria, vasculitis, and neutrophilic dermatoses, such as Sweet’s syndrome or atypical pyoderma gangrenosum.1 Treatment with antibiotics and topical corticosteroids had no effect on the fever or the erythema. A skin biopsy established the diagnosis of neutrophilic eccrine hidradenitis (NEH), showing lymphocytic and neutrophilic infiltrate in and around the eccrine glands (Fig 2).
The patient was also treated with antipyretics. Five days later, his fever disappeared and the erythema faded within 2 weeks. Three weeks after chemotherapy, the leukemia was in complete remission. The second cycle of chemotherapy included high doses of cytarabine and mitoxantrone. Five days after chemotherapy, the fever recurred, and at day 7 the skin lesions worsened despite prophylactic use of topical corticosteroids. The erythematous lesions then spread to the right outer ear and were associated with painful swelling of the submandibular region and periorbital edema. These features have been described as a severe inflammatory exacerbation of NEH.1 As with the first induction chemotherapy, the fever disappeared with no obvious relation to any of the treatments and the skin lesions faded again. As consolidation treatment, an additional cycle of thioguanine/cytarabine/daunorubicin chemotherapy was given along with systemic corticosteroids for the last 4 days. Although treated with the same cytotoxic drugs as in the first cycle, he did not develop this rash again. NEH is a rare dermatosis that erupts after cytotoxic drug treatment of AML (64%), other leukemias, or solid tumors.2 One patient, like ours, was reported to have the erythema before treatment.4 Four patients have been described with NEH and human immunodeficiency virus (HIV) infection.3 The predominant manifestations are fever and erythematous plaques or macules (purpuric to hyperpigmented) mostly on the head, neck, and trunk.1 Severe courses, with involvement of the ear, tender and painful lesions, and periorbital edema, have also been described. A skin biopsy is necessary to confirm the diagnosis. Histopathologic findings typically show a dense neutrophilic infiltrate within and around the eccrine glands, with necrosis of eccrine epithelial cells1 (Fig 2), unlike Sweet’s syndrome, in which whole corium is infiltrated by neutrophils and edema is found. Because NEH is a self-limiting eruption, most cases do not require treatment. At least 60% of patients with NEH have recurrent symptoms with subsequent chemotherapeutic treatment. Attempts to prevent NEH with dapsone, ibuprofen, and systemic corticosteroids have unproven efficacy.1 A direct toxic effect of chemotherapeutic agents to eccrine cells has been proposed as the most probable cause of NEH. This hypothesis is supported by the observation that intradermal injection of cytotoxic drugs resulted in the histologic findings of NEH.5 Efforts to link NEH with specific chemotherapeutic agents, such as cytarabine, have been unsuccessful.2 In the present case, NEH developed with the onset of AML before any chemotherapy had been administered. Our case and a similar report on NEH heralding the onset of AML4 suggest that NEH is a paraneoplastic phenomenon rather than a chemotherapy toxicity–induced dermatosis. This hypothesis is further supported by reports of NEH exacerbation under induction chemotherapy, but not during later cycles, similar to the clinical course in our case.6 These clinical courses of NEH and the appearance of NEH in HIV-infected patients3 suggest that NEH is a dermatosis correlating with changes in the immune status, and only its exacerbation seems to be induced by chemotherapy. Copyright 2000 American Society of Clinical Oncology REFERENCES 1. Susser WS, Whitaker-Worth DL, Grant-Kels JM: Mucocutaneous reaction to chemotherapy. Am Acad Dermatol 39: 367-398, 1999 2. Wenzel FG, Horn TD: Nonneoplastic disorders of the eccrine glands. Am Acad Dermatol 38: 1-17, 1998[Medline] 3. Krischer J, Rutschmann O, Vollenweider-Roten S, et al: Neutrophil eccrine hidradenitis in a patient with AIDS. J Dermatol 25: 199-200, 1998 [Medline]
4.
Pierson JC, Helm TN, Taylor JS, et al: Neutrophilic eccrine hidradenitis heralding the onset of acute myelogenous leukemia. Arch Dermatol 129: 791-792, 1993
5.
Templeton SF, Solomon AR, Swerlick RA: Intradermal bleomycin injections into normal human skin. Arch Dermatol 130: 577-583, 1994 6. Fitzpatrick JE, Bennion SD, Reed OM, et al: Neutrophilic eccrine hidradenitis associated with induction chemotherapy. J Cutan Pathol 14: 272-278, 1987[Medline]
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Copyright © 2000 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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