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LETTER TO THE EDITOR
Year : 2014  |  Volume : 51  |  Issue : 4  |  Page : 598-599
 

Acute leukemia presenting as primary ecthyma gangrenosum


1 Department of Dermatology, North Bengal Medical College, Darjeeling, West Bengal, India
2 Department of Pediatrics, North Bengal Medical College, Darjeeling, West Bengal, India
3 Department of Pathology, North Bengal Medical College, Darjeeling, West Bengal, India
4 Department of Microbiology, North Bengal Medical College, Darjeeling, West Bengal, India
5 Department of Dermatology, Katihar Medical College, Bihar, India

Date of Web Publication1-Feb-2016

Correspondence Address:
Dr. S Koley
Department of Dermatology, North Bengal Medical College, Darjeeling, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-509X.175355

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How to cite this article:
Koley S, Das G, Mandal R K, Barman D C, Mallick S, Kumar P. Acute leukemia presenting as primary ecthyma gangrenosum. Indian J Cancer 2014;51:598-9

How to cite this URL:
Koley S, Das G, Mandal R K, Barman D C, Mallick S, Kumar P. Acute leukemia presenting as primary ecthyma gangrenosum. Indian J Cancer [serial online] 2014 [cited 2019 Dec 6];51:598-9. Available from: http://www.indianjcancer.com/text.asp?2014/51/4/598/175355


Sir,

Ecthyma gangrenosum (EG), generally associated with pseudomonal sepsis, usually occurs in critically ill and immunocompromised patients. EG might be the first manifestation of an underlying immunosuppression. [1],[2] We present an unsuspected child where primary EG was the presenting manifestation of acute leukemia.

A 5-year-old boy with intermittent fever since last 12 days, two non-healing lesions on the face since last 7 days and progressive pain on bilateral thighs, was referred to dermatology outpatients department. Before 5 days (i.e. 7th day of fever), he visited a general physician who prescribed a third generation broad spectrum cephalosporin after sending his blood for widal test and culture. His fever continued with no improvement, widal test was negative and there was no growth in culture. The sores were initially painless, small and red. Then he had pus-filled-lesions and finally hemorrhagic crusts. He denied the presence of similar lesion in family. Physical examination revealed an irritated child with two painless gangrenous plaques (1.5-2 cm in diameter) located on the right malleolus and the left cheek. Each had a black eschar with the rim of surrounding erythema [Figure 1]. Patient was provisionally diagnosed as EG and put on piperacillin. The laboratory values were significant for WBC 900/mm 3, hemoglobin and hematocrit 5.3 g/dl and 12.5%, respectively, platelets 21,000/mm 3, lymphocytes 60%, blasts 2.8%. The coagulation profile revealed elevated prothrombin time, international normalized ratio, partial thromboplastin time and fibrinogen at 15.5 s, 1.26, 40.5 s and 532 mg %. There was no growth in blood culture. The eschar was elevated and scrapping was taken from the underlying tissue and it grew Pseudomonas after subculture in blood agar [Figure 2]. Bone marrow biopsy was diagnostic of B cell acute leukemia (L2 type) [Figure 3] and [Figure 4]. Six days after admission his cutaneous lesions have almost healed [Figure 5]. Treatment for acute lymphoblastic leukemia (ALL) was started simultaneously with antibiotics. There was no recurrence of lesion.
Figure 1: Ecthyma gangrenosum affecting right mallar area and left chin

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Figure 2: gram stain after subculture of scraping (taken after removing eschar) showing gram negative bacilli (×10)

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Figure 3: Bone marrow smears showing lymphoblasts (Leishman's stain, ×40)

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Figure 4: Lymphoblasts having large round nuclei, inconspicuous nucleoli and scanty cytoplasm (Leishman's stain, ×100)

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Figure 5: healed lesions of ecthyma gangrenosum on 6th day after starting piperacillin

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Specimen collected after removing the eschar grew colonies of Pseudomonas aeruginosa after subculture in blood agar, but blood cultures on two different occasions never showed any growth. We believe that the infection was primary EG without associated bacteremia. This was supported by quick healing of lesion after piperacillin. In the literature, there is an association between ALL and primary EG.[3] It is likely that primary EG is associated with skin changes such as shifts in skin flora, alterations in the stratum corneum, increased hydration and humidity on the epidermal surface.[2] It is hypothesized that transient neutropenia, known to manifest prior to lymphoblastosis, facilitates infection with opportunistic pathogens.[3]

Primary EG has a better prognosis than EG with Pseudomonas bacteremia. The mortality rates vary 0-28% in cases of skin lesions without bacteremia [2],[3] and between 38% and 70% in cases of P. aeruginosa septicemia.[4],[5]

Musculoskeletal manifestations in the form of vague pain in legs probably suggested ALL. B-cell acute lymphocytic leukemia is the most common type of ALL and patients with this disease must begin chemotherapy immediately after diagnosis. Few cases of EG have been reported as the first manifestation of leukemia in a child.[5],[6],[7] It is important to recognize EG, start appropriate antimicrobial agents and investigate the patient for underlying immunodeficiencies.

 
 » References Top

1.
Mull CC, Scarfone RJ, Conway D. Ecthyma gangrenosum as a manifestation of Pseudomonas sepsis in a previously healthy child. Ann Emerg Med 2000;36:383-7.  Back to cited text no. 1
    
2.
Chan YH, Chong CY, Puthucheary J, Loh TF. Ecthyma gangrenosum: A manifestation of Pseudomonas sepsis in three paediatric patients. Singapore Med J 2006;47:1080-3.  Back to cited text no. 2
    
3.
Gençer S, Ozer S, Ege Gül A, Do an M, Ak O. Ecthyma gangrenosum without bacteremia in a previously healthy man: A case report. J Med Case Rep 2008;2:14.  Back to cited text no. 3
    
4.
Fergie JE, Patrick CC, Lott L. Pseudomonas aeruginosa cellulitis and ecthyma gangrenosum in immunocompromised children. Pediatr Infect Dis J 1991;10:496-500.  Back to cited text no. 4
    
5.
Pouryousefi A, Foland J, Michie C, Cummins M. Ecthyma gangrenosum as a very early herald of acute lymphoblastic leukaemia. J Paediatr Child Health 1999;35:505-6.  Back to cited text no. 5
    
6.
Singh N, Devi M, Devi S. Ecthyma gangrenosum: A rare cutaneous manifestation caused by Pseudomonas aeruginosa without bacteremia in a leukemic patient. Indian J Dermatol Venereol Leprol 2005;71:128-9.  Back to cited text no. 6
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7.
Fink M, Conrad D, Matthews M, Browning JC. Primary ecthyma gangrenosum as a presenting sign of leukemia in a child. Dermatol Online J 2012;18:3.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

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