Hairy cell leukemia presenting with Ecthyma Gangrenosum- a case report
- 156 Downloads
Ecthyma gangrenosum is a cutaneous infectious usually associated with P. aeruginosa. It usually develops In patients with an underlying immunodeficiency.
A 50-year old mentally disabled white male with a history of epilepsy presented with fever and a painless red macule on his right arm which rapidly progressed to a painful ulcer. Blood and lesion cultures revealed P.aeruginosa, confirming our clinical diagnosis of ecthyma gangrenosum. Subsequently an underlying immune deficit was found, namely patient was diagnosed with hairy-cell leukemia. Despite adequate antibiotics no infection control could be achieved. After treating the underlying immune deficit as well, the infection and hairy-cell leukemia resolved completely.
Ecthyma gangrenosum is an important cutaneous infection to recognize, because it is it is typically associated with P.aeruginosa bacteremia. Recognizing this skin leasion should prompt empiric antimicrobial therapy including an agent with antipseudomonal activity. Furthermore, just like in our case, the presence of ecthyma gangrenosum can signal the presence of an occult immune deficit, warranting further investigation.
KeywordsEcthyma gangrenosum Hairy-cell leukemia
Ecthyma gangrenosum is an uncommon cutaneous infectious classically associated with Pseudomonas aeruginosa bacteremia in critically ill and immunocompromised patients. 
The well-recognized leasion of ecthyma gangrenosum results from perivascular bacterial invasion of the media and adventitia of arteries and veins with seconday ischemic necrosis. 
Despite adequate antibiotic treatment our patient continued to have high fever and elevated CRP. Clindamycin and single dose of gentamicin were empirically added to ceftazidim, but no clinical improvement ensued. We decided to start treatment of hairy cell leukaemia with cladribine (0.12 mg/kg during 5 days). After initiation of treatment, the ectyhma gangrenosum resolved completely within 3 months and the patient achieved a complete remission of HCL.
EG is typically caused by Pseudomonas aeruginosa, although EG-like lesions have been observed in patients with other bacterial, viral and fungal infections.  The diagnosis is generally made based on the characteristic clinical appearance of the lesions along with a positive blood or wound cultures.
Ecthyma gangrenosum can signal previously undetected immunodeficiency. After presentation with ecthyma gangrenosum our patient was diagnosed with hairy-cell leukemia. HCL is an uncommon chronic B cell lymphoproliferative disorder accounting for approximately 2% of lymphoid leukemias.  Many patients have symptoms related to splenomegaly or cytopenias inducing fatigue, infection and/or haemorrhagic findings. Approximately one-quarter are asymptomatic, mostly presenting because of the unexpected finding of pancytopenia during routine evaluation. Patients only require treatment when they have significant cytopenia or less severe cytopenias that are symptomatic (eg., repeated infections, bleeding). Hairy cell leukemia is treated with purine analogs (ie,cladribine or pentostatin). Because these drugs are myelosupressive, it is challenging to start treatment in patients with an active infectious.  Since our attempt to control infection failed, we had no other option but to start treatment with the purine nucleoside analog. Treatment of the underlying disease in combination with antibiotics led to complete resolution of ecthyma gangrenosum and complete remission of HCL.
In conclusion, ecthyma gangrenosum is an important cutaneous infection to recognize, because it is it is typically associated with P.aeruginosa bacteremia. Recognizing this skin leasion should prompt empiric antimicrobial therapy including an agent with antipseudomonal activity. Furthermore, just like in our case, the presence of ecthyma gangrenosum can signal the presence of an occult immune deficit, warranting further investigation.
No funding was obtained for the writing of this case report.
Availability of data and materials
Any data (suitably anonymised to maintain patient confidentiality) is available for readers to review if a suitable written request to the Corresponding author is made.
RS, MS-participated in the clinical care of the patient on the ward. MT- advised and supervised the laboratory testing, interpretation and reporting. RS-wrote the first draft of the paper, which was edited for style and accuracy by MS and MT. All authors critically reviewed the manuscript for publication. All authors have read and approved the final version of this manuscript.
Ethics approval and consent to participate
Consent for publication
Written consent to publish this case report and accompanying images was obtained from the patient and his parents/legal guardians. A copy of the written consent is available for review by the Editor of this journal.
All authors declare to have no competing interests.
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
- 3.Weinberg, AN & Swartz M. Gram negative coccal and bacillary infections. Fitzpatrick, TB, Eisen, AZ, Wolf, K al eds Dermatology gen med. 1993; Vol2: 2334 2349.Google Scholar
- 6.Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al. WHO classification of Tumours of Haematopoietic and lymphoid tissues. 2008.Google Scholar
Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.