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Acute myelomonocytic leukemia revealed by a chickenpox-like rash
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Journal of the European Academy of Dermatology and Venereology 6 (1996) 76-79
Case report
Acute myelomonocytic leukemia revealed by a chickenpox-like rash Bruno Hoen a~* , Anne C Neidhardt a, Catherine Aghassian a, VCronique Dorvaux b, Francis Witz b, Philippe Canton a a Department of Infectious and Tropical Diseases, University of Nancy Medical Center, 54511 Vandoeuure, France b Department of Internal Medicine, Division of Clinical Hematology, University of Nancy Medical Center, Nancy, France
Abstract An unusual case of leukemia cutis is presented. A 42-year-old man presented with a vesicular skin rash mimicking chickenpox. Skin biopsy firmly ruled out the diagnosis of varicella and revealed an infiltration of the superficial derma by myeloblasts. Bone marrow aspirates confirmed the diagnosis of acute myelomonocytic leukemia (type M4). Keywords: Leukemia cutis; Myelomonocytic leukemia; Varicella
1. Introduction Leukemia cutis may sometimes complicate the course of or even reveal acute myelogenous leukemia. The following case-report illustrates how a vesicular skin rash mimicking chickenpox may be a very unusual presentation of leukemia cutis. 2.
Case report
A 42 year-old male patient with no medical past history was referred to hospital for a vesicular skin rash associated with fever and dyspnea. * Corresponding author. Tel.: (33) 83 15 41 12; fax: (33) 83 15 35 34.
He had been febrile (39°C) for 3 weeks. Cutaneous eruption had started at the same time as fever. It was initially located to the upper limbs and then spread to the trunk, lower limbs and scalp. The patient developed dyspnea progressively over the last week before admission. At that time he had lost 3 kg of body weight and still had high-grade fever. Skin rash consisted in erythematous vesiculo-papular plaques with crusted lesions (Fig. 1A). All lesions seemed to be of identical age. They were located on the torso, scalp and the four limbs. Some of them formed small clusters (Fig. 1B). The patient complained of mild pruritus and had orthopnea. Chest examination showed no auscultatory change. Chest Xray disclosed bilateral interstitial opacities (Fig. 2). Arterial blood partial pressure of oxygen was 62 torr.
0926-9959/96/$15.00 0 1996 Elsevier Science B.V. All rights reserved SSDZO926-9959(95)00143-3
B. Hoen et al. /J. Eur. Acad. Dermatol. Venereal. 6 (1996) 76-79
Fig. 1. (A) Vesicular rash of the trunk. (B) Eruption in the forearm; some lesions form small clusters.
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B. Hoen et al. /J. Eur. Acad. Dermatol. Venereal. 6 (1996) 76-79
pathic effect of Herpesviridae. Bronchoalveolar lavage fluid contained 8% myeloblasts and 40% basophil granulocytes. Viral cultures of bronchoalveolar lavage fluid remained negative. Eventually the diagnosis of acute myelomonocytic leukemia beginning with manifestations of cutaneous and pulmonary infiltration was retained. After chemotherapy was initiated, skin lesions healed and chest X-ray returned to normal within three weeks.
3. Discussion
Fig. 2. Chest X-ray on patient’s admission.
Three diagnoseswere discussed: . severe chickenpox in an adult who could not recollect having developed varicella during childhood. The long-lasting evolution together with the fact that all vesicles had the same age did not support this hypothesis. However chickenpox in an immunocompromised host could not be ruled out firmly and the patient was given intravenous acyclovir 10 mg/kg body weight 3 times daily . dermatitis herpetiformis was unlikely becauseof the high-grade fever and the absence of severe pruritus . leukemia cutis revealing an hematologic malignancy. White blood cell count showed 7220 leukocytes/mm3 (5% pqlymorphonuclear and 77% atypical cells). Hemoglobin concentration was 9.1 g/d1 and platelet count was 100000/mm3. Bone marrow examination confirmed the diagnosis of acute myelomonocytic leukemia, with bone marrow eosinophilia, type M4 according to the French-American-British (FAB) classification. Skin biopsy showed a monomorphic infiltration of the superficial derma by myeloblasts and no cyto-
When understanding leukemia cutis as a specific lesion secondary to leukemic infiltration of the skin, it appears that this entity is rather rare. Baer et al. [ll identified biopsy-proven leukemia cutis in 18 out of 877 patients (2%) with acute myelogenous leukemia. Ten of these patients had FAB type M4 leukemia. None of the 18 patients presented with vesicular lesions. In a series of 42 casesof leukemia cutis, the distribution of associated leukemia included 3 casesof acute lymphocytic leukemia, 16 of chronic lymphocytic leukemia, 12 of acute granulocytic leukemia, 3 of chronic granulocytic leukemia, 5 of acute monocytic leukemia and 3 of acute myelomonocytic leukemia [2]. In none of these caseswere vesicles described as part of skin lesions. Vesicles have been described in few cases of chronic lymphocytic leukemia [2,31 and a unique bullous lesion has been reported during the course of an acute monocytic leukemia (FAB type M.5) [4]. However vesicles are considered to be quite exceptional in acute myelogenous leukemia. In an old series, Epstein et al. 151reported 2 cases of “myeloid leukemia” with vesicles or bullae. However in these two cases,leukemia was not acute and skin lesions were considered as leukemids rather than leukemia cutis. In acute myelogenous leukemia, beyond its clinical aspects, leukemia cutis seems to be frequently associated with the development of extramedullary disease at other sites El], as was the case in our observation. This occurrence might also worsen the prognosis of leukemia.
B. Hoen et al. /J. Eur. Acad. Dermatol. Venereol. 6 (1996) 76-79
References [l] Baer MR, Barcos M, Fare11H, Preisler HD. Acute myelogenous leukemia with leukemia cutis. Eighteen cases between 1969 and 1986. Cancer 1989;63:2192-2200. [2] Su WPD, Buechner SA, Li CY. Clinicopathologic correlations in leukemia cutis. J Am Acad Dermatol 1984;11:121-128. [3] Bureau Y, Barr&e H, Litoux P, Bureau B. Leucose
19
lymphoide avec des lesions cutanees visiculobulleuses. Bull Sot Fr Dermatol Syphil 1969;76:727-728. [4] Grob JJ, Gabriel B, Horchowski N, Bonerandi JJ. Localisation cutanee bulleuse avec epidermotropisme au tours d’une leucemie monoblastique. Ann Dermatol Venereol 1988;115:59-61. [5] Epstein E, McEarchen K. Dermatologic manifestations of the lymphoblastoma leukemia group. Arch Intern Med 1937; 60: 867-875.
Journal of the European Academy of Dermatology and Venereology 6 (1996) 76-79
Case report
Acute myelomonocytic leukemia revealed by a chickenpox-like rash Bruno Hoen a~* , Anne C Neidhardt a, Catherine Aghassian a, VCronique Dorvaux b, Francis Witz b, Philippe Canton a a Department of Infectious and Tropical Diseases, University of Nancy Medical Center, 54511 Vandoeuure, France b Department of Internal Medicine, Division of Clinical Hematology, University of Nancy Medical Center, Nancy, France
Abstract An unusual case of leukemia cutis is presented. A 42-year-old man presented with a vesicular skin rash mimicking chickenpox. Skin biopsy firmly ruled out the diagnosis of varicella and revealed an infiltration of the superficial derma by myeloblasts. Bone marrow aspirates confirmed the diagnosis of acute myelomonocytic leukemia (type M4). Keywords: Leukemia cutis; Myelomonocytic leukemia; Varicella
1. Introduction Leukemia cutis may sometimes complicate the course of or even reveal acute myelogenous leukemia. The following case-report illustrates how a vesicular skin rash mimicking chickenpox may be a very unusual presentation of leukemia cutis. 2.
Case report
A 42 year-old male patient with no medical past history was referred to hospital for a vesicular skin rash associated with fever and dyspnea. * Corresponding author. Tel.: (33) 83 15 41 12; fax: (33) 83 15 35 34.
He had been febrile (39°C) for 3 weeks. Cutaneous eruption had started at the same time as fever. It was initially located to the upper limbs and then spread to the trunk, lower limbs and scalp. The patient developed dyspnea progressively over the last week before admission. At that time he had lost 3 kg of body weight and still had high-grade fever. Skin rash consisted in erythematous vesiculo-papular plaques with crusted lesions (Fig. 1A). All lesions seemed to be of identical age. They were located on the torso, scalp and the four limbs. Some of them formed small clusters (Fig. 1B). The patient complained of mild pruritus and had orthopnea. Chest examination showed no auscultatory change. Chest Xray disclosed bilateral interstitial opacities (Fig. 2). Arterial blood partial pressure of oxygen was 62 torr.
0926-9959/96/$15.00 0 1996 Elsevier Science B.V. All rights reserved SSDZO926-9959(95)00143-3
B. Hoen et al. /J. Eur. Acad. Dermatol. Venereal. 6 (1996) 76-79
Fig. 1. (A) Vesicular rash of the trunk. (B) Eruption in the forearm; some lesions form small clusters.
77
B. Hoen et al. /J. Eur. Acad. Dermatol. Venereal. 6 (1996) 76-79
pathic effect of Herpesviridae. Bronchoalveolar lavage fluid contained 8% myeloblasts and 40% basophil granulocytes. Viral cultures of bronchoalveolar lavage fluid remained negative. Eventually the diagnosis of acute myelomonocytic leukemia beginning with manifestations of cutaneous and pulmonary infiltration was retained. After chemotherapy was initiated, skin lesions healed and chest X-ray returned to normal within three weeks.
3. Discussion
Fig. 2. Chest X-ray on patient’s admission.
Three diagnoseswere discussed: . severe chickenpox in an adult who could not recollect having developed varicella during childhood. The long-lasting evolution together with the fact that all vesicles had the same age did not support this hypothesis. However chickenpox in an immunocompromised host could not be ruled out firmly and the patient was given intravenous acyclovir 10 mg/kg body weight 3 times daily . dermatitis herpetiformis was unlikely becauseof the high-grade fever and the absence of severe pruritus . leukemia cutis revealing an hematologic malignancy. White blood cell count showed 7220 leukocytes/mm3 (5% pqlymorphonuclear and 77% atypical cells). Hemoglobin concentration was 9.1 g/d1 and platelet count was 100000/mm3. Bone marrow examination confirmed the diagnosis of acute myelomonocytic leukemia, with bone marrow eosinophilia, type M4 according to the French-American-British (FAB) classification. Skin biopsy showed a monomorphic infiltration of the superficial derma by myeloblasts and no cyto-
When understanding leukemia cutis as a specific lesion secondary to leukemic infiltration of the skin, it appears that this entity is rather rare. Baer et al. [ll identified biopsy-proven leukemia cutis in 18 out of 877 patients (2%) with acute myelogenous leukemia. Ten of these patients had FAB type M4 leukemia. None of the 18 patients presented with vesicular lesions. In a series of 42 casesof leukemia cutis, the distribution of associated leukemia included 3 casesof acute lymphocytic leukemia, 16 of chronic lymphocytic leukemia, 12 of acute granulocytic leukemia, 3 of chronic granulocytic leukemia, 5 of acute monocytic leukemia and 3 of acute myelomonocytic leukemia [2]. In none of these caseswere vesicles described as part of skin lesions. Vesicles have been described in few cases of chronic lymphocytic leukemia [2,31 and a unique bullous lesion has been reported during the course of an acute monocytic leukemia (FAB type M.5) [4]. However vesicles are considered to be quite exceptional in acute myelogenous leukemia. In an old series, Epstein et al. 151reported 2 cases of “myeloid leukemia” with vesicles or bullae. However in these two cases,leukemia was not acute and skin lesions were considered as leukemids rather than leukemia cutis. In acute myelogenous leukemia, beyond its clinical aspects, leukemia cutis seems to be frequently associated with the development of extramedullary disease at other sites El], as was the case in our observation. This occurrence might also worsen the prognosis of leukemia.
B. Hoen et al. /J. Eur. Acad. Dermatol. Venereol. 6 (1996) 76-79
References [l] Baer MR, Barcos M, Fare11H, Preisler HD. Acute myelogenous leukemia with leukemia cutis. Eighteen cases between 1969 and 1986. Cancer 1989;63:2192-2200. [2] Su WPD, Buechner SA, Li CY. Clinicopathologic correlations in leukemia cutis. J Am Acad Dermatol 1984;11:121-128. [3] Bureau Y, Barr&e H, Litoux P, Bureau B. Leucose
19
lymphoide avec des lesions cutanees visiculobulleuses. Bull Sot Fr Dermatol Syphil 1969;76:727-728. [4] Grob JJ, Gabriel B, Horchowski N, Bonerandi JJ. Localisation cutanee bulleuse avec epidermotropisme au tours d’une leucemie monoblastique. Ann Dermatol Venereol 1988;115:59-61. [5] Epstein E, McEarchen K. Dermatologic manifestations of the lymphoblastoma leukemia group. Arch Intern Med 1937; 60: 867-875.