Persistent Erythema Infectiosum-Like Rash as a Prodrome of Acute Lymphocytic Leukemia
Descrição do Produto
Pediatric Dermatology Vol. 11 No. 2 156-159
Persistent Erythema Infectiosum-Like Rash as a Prodrome of Acute Lymphocytic Leukemia Seung Min Lee, M.D., Dong Gun Kim, M.D., and Dongsik Bang, M.D. [Department of Dermatology, Yonsei University College of Medicine, Seoul, Korea\
Abstract: A 7-year-old boy had erythema infectiosum with typical manifestations. Over more than 40 days, these iesions showed no sign of regression, and chronic anemia became progressiveiy more severe. Electron microscopic examination performed 20 days after onset showed abnormal Sezary-like lymphocytes. Bone marrow biopsy, which was performed to evaluate the anemia, was consistent with acute lymphocytic leukemia. Persistent parvovirus B19 infection may be connected with immunosuppression. Therefore, early electron microscopic study and bone marrow biopsy may be helpfui for early diagnosis of hematologic malignancies. .
Erythema infectiosum, first described in 1886 by Tschamer, is a benign infectious exanthem. This usually asymptomatic disease begins abruptly with erythema of the cheeks, referred to as a slapped check appearance, and extends to the trunk and extremities. The periora! area, eyelids, and chin are usually spared. A characteristic feature of erythema infectiosum is the evanescent nature of the rash, which fades within a week but subsequently briefly recurs several times (1). Human parvovirus B19 is now known to be the etiologic agent of erythema infectiosum (2-5) as well as other disorders such as postinfectious arthropathy in adults (6-7), transient aplastic crisis associated with various hemoiytic anemias, and immunosuppressive states (8-14). We present a patient with persistent erythema infectiosum and chronic anemia lasting more than 40 Address correspondence to Seung Min Lee, M.D.. Yongdong Severence Hospital. Yonsei University College of Medicine. Department of Dermatology, Young Dong P.O. Box 1217, Seoul, Korea.
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days, who subsequently was diagnosed as having acute lymphocytic leukemia. CASE REPORT A 7-year-old boy had a 7-day history of an erythematous. maculopapular eruption and a 20-day history of mild fever, headache, and arthralgia of both knees. Physical examination revealed a warm, indurated, erythematous. maculopapular rash on the face, prominent on the malar eminences, with circumoral pallor and lacelike erythema on the extensor surfaces and trunk (Figs. 1 and 2). Hepatosplenomegaly and small, coin-sized, cervical lymph nodes were noted on palpation. His temperature was 37.3°C. The white blood cell count was 12500/ mm"^. with 43% polymorphonuclear cells, 52% lymphocytes. 3% monocytes, 1% basophils, and 1% eosinophils. and platelet count of 238,000/mm"'. The
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Figure 1. trythematous maculopapular rash on both cheeks (slapped cheek appearance) and forehead with circumoral pallor.
hemoglobin level was 10.2 g/dl, the hematocrit was 29.9%. Erythrocyte sedimentation rate was 55 mm/ hour and antistreptolysin titer was 400 IU. Results of antinuclear antibody. C-reactive proteins, and LE cell studies were negative. The levels of immunoglobulins and C^ and C4 were normal. Peripheral blood smear showed no abnormal blasts. A skin biopsy specimen from an erythematous patch on the arm showed a perivascular infiltrate of mononuclear cells with spongiosis of the epidermis (Fig. 3). The patient was treated symptomatically with acetaminophen and antihistamines, but the diffuse morbiliform eruption on his face, trunk, and extremities lasted for 40 days. At that time the boy appeared anemic and had a great deal of joint pain. His hemoglobin was 7.3 g/dl, and hematocrit 22.7%. A bone marrow examination showed abnormal lymphoblast proliferation and erythroid hypoplasia (Fig. 4). Reevaluation of electron microscopic findings of a previous biopsy specimen 20 days after onset revealed classic Sezary-like cells showing markedly convoluted nuclei with poor cytoplasm (Fig. 5). The patient began chemotherapy with prednisolone, vineristine. and L-asparaginase, and central nervous system prophylaxis with methotrexate, cytosine-arabinoside, and hydrocortisone. He has
Figure 2. Maculopapular erythema with lacelike appearance on both legs.
received maintenance therapy for two years after remission without relapse. DISCUSSION
Erythema infectiosum has long been presumed to be due to a virus, and occurs in epidemics in the spring and summer (1). After the publication of a report by Anderson et al (2), Plummer et al tested serum taken from 12 patients with erythema infectiosum during a miniepidemic in 1980 and identified parvovirus BI9 in 11. Human parvovirus B19 is a single-stranded DNA virus that has been associated with erythema infectiosum (2-5), poly arthralgia and arthritis, aplastic crisis in patients with hemoglobulinopathies. immunosuppression (8-14), hydrops and fetal death (15). The most common manifestation of parvovirus B19 infection is erythema infectiosum (16). The illness usually begins with the sudden appearance of livid erythema of the cheeks. The rash lasts 2 to 39 days (mean 11 days) (20). Our patient had the characteristic slapped cheek
158 Pediatric Dermatology Vol. 11 No. 2 June 1994
Figure 3. Biopsy specimen from the forearm shows spongiotic epidermis and perivascular infiltration of mononuclear cells. (Hematoxylin & eosin magnification lOOx,)
Figure 4. Bone marrow examination shows abnormal lymphoblast proliferation and erythroid hypoplasia. (Giemsa; magnification 400x.)
appearance and lacelike erythema on both extremities and trunk, and also a mild fever with general weakness. Laboratory findings revealed chronic anemia without evidence of leukemia at the time of onset. Morbiliform eruption and anemia lasted about 40 days, at which time his anemia became severe. In parvovirus B19 infection, anemia, aplastic crisis, and chronic bone marrow failure may result. The virus lytically infects erythroid precursors in the bone marrow and inhibits erythropoiesis (11,17, 18); there is also virus-induced perturbation of human megakaryocytopoiesis (19). Chronic bone marrow failure resulting from parvovirus B!9 infection was reported in a child with Nezelof syndrome (10), and children with acute lymphocytic leukemia and chronic parvovirus B19 infection are described in the literature (11.12). In an immunocompromised host, infection with parvovirus B19 can persist and cause chronic bone marrow failure, usuaiiy manifested as anemia (10,12). Those patients had no characteristic skin manifestations, and the infection was detected by serologic assay with specific antibodies and DNA detection in serum and organ specimens. Our patient provides several points for consideration. His preceding persistent parvovirus Bt9 infection with characteristic skin manifestations led to acute lymphocytic leukemia (ALLl FAB classification). Infection with parvovirus has been implicated in affecting rapidly dividing bone marrow cells and causing chronic bone marrow failure in immunocompromised hosts. Whether the parvovirus
Figure 5. Electron microscopy demonstrates cells with marked convoluted nuclei and poor cytoplasm (Sezary-like cell). (Magnification (A) 5850x, (B) 17,250x.)
Lee et al: Erythema Infectiosum-Like Rash
infection is an etiologic agent of ALL is not clear at this point. An electron microscopic specimen taken 20 days after onset revealed Sezary-like lymphocytes with marked folded nuclei. Definitive diagnosis of ALL could not be established by clinical and laboratory tests at this time. Incubation of normal human lymphocytes with pokeweed mitogen or phytohemagglutinin results in the presence of 5% to 11% of cells with light microscopic and ultrastructural appearance indistinguishable from that of Sezary cells. Therefore Sezary cells are simulated or transformed lymphocytes (21). Their presence is not indicative of malignancy, but we assumed the ultrastructural environment already might be changed. We suggest persistent parvovirus B19 infection may indicate an immunosuppressed status. Performing early electron microscopic studies and bone marrow examination may therefore be helpful in early diagnosis of hematologic malignancies in children. REFERENCES 1. Arnold HL. Odom RB, James WD. Disease of the skin—clinical dermatology. Viral disease. Philadelphia: WB Saunders, 1990:476-477. 2. Anderson MJ. Jones SE. Eisher-Hoch SP. et al. Human parvovirus. the cause of erythema infectiosum (fifth disease). Lancet I983;I:I378. 3. Anderson MJ, Lewis E, Kidd IM. Cohen BJ. An outbreak of erythema infectiosum associated with human parvovirus infection. J Hyg 1984:93:85-93. 4. Plummer EA, Hammond GW, Eorward K. et al. An erythema infectiosum-like illness caused by human parvovirus infection. N Engl J Med 1985:313:74-79. 5. Okabe N, Koboyashi S. Tatsuzawa O. et al. Detection of antibodies to human parvovirus in erythema infectiosum (fifth disease). Arch Dis Child 1984:59: 1016-1019. 6. White DG. Wool!" AD. Mortimer PP. Cohen BJ. Blake DR. Bacon PA. Human parvovirus arthropathy. Lancet I985;l:419-421. 7. Reid DM. Reid TMS, Brown T, Rennie JAN. Eastmond CJ. Human parvovirus-associated arthritis: a ciinicai and laboratory description. Lancet 1985:1: 422-424.
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8. Pattison JR, Jones SE. Hodson J, et al. Parvovirus infections and hypoplasic crisis in sickle-cell anemia. Lancet 1981;l:664-^65. 9. Serjeant GR. Topley JM. Mason K, et al. Outbreak of aplastic crisis in sickle cell anemia associated with parvovirus-like agent. Lancet 1981:2:595-597. 10. Kurtzman GJ. Ozawa K, Cohen B. Hanson G. Oseas R. Young NS. Chronic bone marrow failure due to persistent BI9 parvovirus infection. N Engl J Med 1989:5:287-294. 11. Kurtzman GJ. Frickhofen N. Kimball JRN. Jenkins DW, Nienhuis AW, Young NS. Pure red cell aptasia of 10 years" duration due to persistent parvovirus B19 infection and its cure with immunoglobulin therapy. N Engl J Med 1989:8:519-523. 12. Kurtzman GJ. Cohen H. Meyers P. Amunullah A, Young NS. Persistent B19 parvovirus infection as a cause of severe chronic anemia in children with acute lymphocytic leukemia. Lancet 1988:19:1159-1162. 13. Koch WC, Massey G. Russel CE. Adier SP. Manifestations and treatment of human parvovirus B19 infection in immunocompromised patients. J Pediatr 1990; 116:355-359. 14. VanHorn DK, Mortimer PP, Young N. Hanson GR. Human parvovirus-associated red cell aplasia in the absence of underlying hemolytic anemia. Am J Pediatr Hematol Oncol I986;8(3):235-239. 15. Anand A. Gray ES. Brown T, et al. Human parvovirus infection in pregnancy and hydrops fetalis. N EnglJ Med 1987:316:183-186. 16. Bialeki C, Eeder HM Jr. Grant-Kels JM. The six classic childhood exanthems: a review and update. J Am Acad Dermato! 1989:21:891-903. 17. Mortimer PP, Humphries RK. Moore JG. Purcell RH, Young NS. A human parvovirus-like virus inhibits hematopoietic colony formation in vitro. Nature 1983:302:426-429. 18. Ozawa K, Kurtzman G. Young N. Replication of the BI9 parvovirus in human bone marrow cell cultures. Science 1986:233:883-886. 19. SrivastavaBA. Bruno E. Briddell R. et al. Parvovirus BI9-induced perturbation of human megakaryocytopoiesis in vitro. Am Soc Hematol 1990:76:19972004. 20. Behrman RE, Vaughan VC. Nelson's text book of pediatrics: infectious disease. Philadelphia: WB Saunders, 1987. 21. Lever WE, Schaumburg-Lever G. Histopathology of the skin: lymphoma and leukemia. Philadelphia: JB Lippincott, 1990.
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