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Year : 2018  |  Volume : 19  |  Issue : 1  |  Page : 62-64

Ecthyma gangrenosum as the presenting clinical feature of autoimmune lymphoproliferative syndrome

1 Department of Pediatric Medicine, Institute of Child Health, Kolkata, West Bengal, India
2 Department of Pediatric Dermatology, Institute of Child Health, Kolkata, West Bengal, India

Date of Web Publication28-Dec-2017

Correspondence Address:
Aniruddha Ghosh
11, Dr. Biresh Guha Street, Kolkata - 700 017, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpd.IJPD_141_16

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Ecthyma gangrenosum (EG) is a severe invasive cutaneous infection caused classically by Pseudomonas aeruginosa typically affecting immunocompromised patients especially those with neutropenia. We report the case of a 2-year-old boy with autoimmune lymphoproliferative syndrome presenting with solitary EG on the forehead. Blood culture, as well as culture from the lesion both, showed growth of P. aeruginosa. Pseudomonal sepsis responded well to piperacillin-tazobactam and amikacin therapy. Prompt diagnosis of the lesion is crucial as failure to start timely appropriate therapy may lead to fatal outcome.

Keywords: Autoimmune lymphoproliferative syndrome, double negative T-cell, ecthyma gangrenosum, neutropenia, Pseudomonas aeruginosa

How to cite this article:
Ghosh A, Das R, Halder PP, Dhar S. Ecthyma gangrenosum as the presenting clinical feature of autoimmune lymphoproliferative syndrome. Indian J Paediatr Dermatol 2018;19:62-4

How to cite this URL:
Ghosh A, Das R, Halder PP, Dhar S. Ecthyma gangrenosum as the presenting clinical feature of autoimmune lymphoproliferative syndrome. Indian J Paediatr Dermatol [serial online] 2018 [cited 2020 Jul 11];19:62-4. Available from: http://www.ijpd.in/text.asp?2018/19/1/62/206070

  Introduction Top

Ecthyma gangrenosum (EG) is a characteristic skin lesion classically caused by Pseudomonas aeruginosa sepsis but may also be caused by some other bacteria and fungus.[1] Usually, patients with diagnosed hematological malignancy or immunodeficiency disorders develop this kind of severe sepsis.[1] Rarely, previously healthy controls with underlying malignancy or immunodeficiency may present with EG as a first pointer toward the hidden disorder.[2] Autoimmune lymphoproliferative syndrome (ALPS) is a chronic nonmalignant disorder where immune dysregulation occurs due to defect in lymphocyte apoptosis and resultant autoimmune cytopenias especially neutropenia.[3] ALPS presenting for the first time with EG as a part of severe Pseudomonas septicemia especially in pediatric population has rarely been reported.

  Case Report Top

A 2-year-old male patient born out of nonconsanguineous marriage presented with recurrent fever for the past 8 months and a single painless solitary macule of 4 cm × 5 cm size on the left side of forehead for the past 2 days. There was associated abdominal distension for the last few days. Family history, perinatal history was normal.

The patient was very sick looking and was febrile (103.8°F). The general survey revealed moderate pallor, significant generalized lymphadenopathy involving bilateral cervical, axillary, inguinal, and right-sided epitrochlear lymph nodes. All peripheral pulses were palpable. Vitals were within normal range for the age.

Systemic examination revealed hugely enlarged liver (8 cm below right costal margin in midclavicular line) and spleen (7 cm along the splenic axis). His previous medical documents showed a steady trend of enlargement of liver and spleen over the last 8 months.

The macule on the forehead, after admission, gradually turned into edematous papule and then erythematous pustule within 24 h. This pustule on the next day gradually turned into a dark violet colored eschar with surrounding erythematous halo [Figure 1].
Figure 1: Ecthyma gangrenosum on the left side of the forehead - central dark violet colored eschar with perilesional erythematous halo

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Routine investigation showed: Hb: 7.7 g%. Total leukocyte count: 7700/cmm, neutrophil - 6% (absolute neutrophil count-462/cmm), lymphocyte - 86%. Platelet count - 75,000/cmm (anemia with absolute neutropenia and thrombocytopenia). Reticulocyte count: 5.3%. Peripheral blood smear showed hypochromic microcytic picture with anisopoikilocytosis and crenated red blood cells. Direct Coombs test was positive, showed 3+ in quantitative analysis indicating autoimmune hemolysis. C-reactive protein was 213.4 mg/dL (cutoff 5 mg/dL). Liver and renal function tests, electrolytes were normal.

Bone marrow aspiration revealed myeloid hyperplasia, erythroid hyperplasia, and increased megakaryocytes. Ultrasonogram of the whole abdomen showed hepatomegaly, splenomegaly, and presence of several enlarged peripancreatic and retroperitoneal lymph nodes. Blood culture and culture of punch biopsy specimen from the lesion on forehead both gave rise to P. aeruginosa colonies.

Empirically piperacillin-tazobactam and amikacin were started on admission, and as the sensitivity report also showed the organism to be sensitive to these antibiotics, same were continued for 10 days. The patient became afebrile on the day 6 of admission. The lesion on the forehead formed a scab which eventually came off leaving behind a healed ulcer. However, the pancytopenia did not improve even after successful treatment of sepsis.

Cervical lymph node biopsy showed nonspecific reactive hyperplasia. Immunoglobulin levels were within normal ranges. Peripheral blood lymphocytes were analyzed by flow cytometry for double negative T-cells (CD3+ TCR αβ+ CD4 CD8 DNT). Healthy control showed the expected <1% double negative (CD4 and CD8 negative cells) while the patient's sample showed 2.5% of double negative cells. The patient was diagnosed to have ALPS and put on a daily oral steroid (1 mg/kg/day) and weekly oral cotrimoxazole and was referred to a specialized center for hematological disorder for further management.

  Discussion Top

EG is classically considered as pathognomonic of severe sepsis caused by P. aeruginosa, but EG caused by several other microorganisms such as Staphylococcus aureus,  Escherichia More Details coli, Klebsiella pneumonia, Corynebacterium diphtheriae, Candida, and Mucor have also been reported.[1]

Patients who develop EG usually have underlying disorders affecting host immunity such as agammaglobulinemia, hypogammaglobulinemia, aplastic anemia, or AIDS.[1] It has also been described in otherwise healthy pediatric patients with transient risk factors, such as concurrent viral infection and recent antibiotic therapy.[2],[4]

ALPS occurs due to mutations in the Fas ligand pathway of apoptosis.[3] Autosomal recessive and dominant mutations have been described.[5] The most common clinical manifestation of ALPS is chronic lymphadenopathy due to lymphocyte accumulation and/or splenomegaly. There is also an accumulation of phenotypically normal CD3+ CD4 CD8 T-cells (CD3+ DNT).[6] ALPS patients have multilineage autoimmune cytopenias that are chronic and often refractory to treatment. There is also autoimmune dysfunction of other organs including liver, kidneys, and eyes or emergence of lymphoproliferative disorders such as malignant lymphomas involving many different organ systems.[7] Hematopoietic stem cell transplantation is the only curative treatment. Immunosuppressive and immunomodulator drugs such as steroids, immunoglobulin, mycophenolate mofetil, rituximab, and vincristine, are also used.[7]

EG typically appear as small indurated papulovesicular lesion progressing rapidly to necrotic ulcer with surrounding erythematous halo and a central black eschar.[8] EG is the result of bacterial entry into the subcutaneous vessels especially invading the media and adventitia layers, giving rise to hemorrhagic occlusive vasculitis.[8] Lesions are most commonly seen in axilla, buttocks, perineum, and limbs but may be found anywhere in the body.[8]

If EG is clinically suspected, a prompt punch biopsy from the lesion should be sent for culture and sensitivity study along with blood culture.[1],[8] In combination with supportive measures, empiric broad-spectrum systemic antibiotics should be started immediately after obtaining blood cultures and skin biopsy.[8] Typically, a combination of antipseudomonal beta-lactam penicillin such as piperacillin with an aminoglycoside or fluoroquinolone is recommended.[1] Surgical debridement of necrotic tissue and drainage of localized abscesses may be necessary in extensive lesions.[1] Patients with severe neutropenia, hypogammaglobulinemia, or agammaglobulinemia may benefit from treatment with granulocyte-stimulating factor or immunoglobulin.[1]

The presence of EG denotes severe septicemia with a potentially fatal outcome. Even with appropriate therapy the mortality for Pseudomonas septicemia in the immunocompromised remains high.[2] Poor prognostic markers associated with EG include neutropenia, septic shock, inappropriate or delayed antibiotic therapy, pulmonary or abdominal sepsis, and resistant microorganisms.[1] In previously healthy controls a thorough workup may unmask an underlying immune deficiency or malignancy, like in our case.

This case highlights the importance of early diagnosis and aggressive treatment if there is clinical suspicion of EG and also it stresses on a thorough workup to rule out any underlying immunodeficiency state or malignancy. If there is any evidence of autoimmune phenomenon, the diagnosis of ALPS should be considered.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Patel JK, Perez OA, Viera MH, Halem M, Berman B. Ecthyma gangrenosum caused by Escherichia coli bacteremia: A case report and review of the literature. Cutis 2009;84:261-7.  Back to cited text no. 1
Zomorrodi A, Wald ER. Ecthyma gangrenosum: Considerations in a previously healthy child. Pediatr Infect Dis J 2002;21:1161-4.  Back to cited text no. 2
Worth A, Thrasher AJ, Gaspar HB. Autoimmune lymphoproliferative syndrome: Molecular basis of disease and clinical phenotype. Br J Haematol 2006;133:124-40.  Back to cited text no. 3
Huang YC, Lin TY, Wang CH. Community-acquired Pseudomonas aeruginosa sepsis in previously healthy infants and children: Analysis of forty-three episodes. Pediatr Infect Dis J 2002;21:1049-52.  Back to cited text no. 4
Drappa J, Vaishnaw AK, Sullivan KE, Chu JL, Elkon KB. Fas gene mutations in the Canale-Smith syndrome, an inherited lymphoproliferative disorder associated with autoimmunity. N Engl J Med 1996;335:1643-9.  Back to cited text no. 5
Fisher GH, Rosenberg FJ, Straus SE, Dale JK, Middleton LA, Lin AY, et al. Dominant interfering fas gene mutations impair apoptosis in a human autoimmune lymphoproliferative syndrome. Cell 1995;81:935-46.  Back to cited text no. 6
Shah S, Wu E, Rao VK, Tarrant TK. Autoimmune lymphoproliferative syndrome: An update and review of the literature. Curr Allergy Asthma Rep 2014;14:462.  Back to cited text no. 7
Greene SL, Su WP, Muller SA. Ecthyma gangrenosum: Report of clinical, histopathologic, and bacteriologic aspects of eight cases. J Am Acad Dermatol 1984;11:781-7.  Back to cited text no. 8


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