|LETTERS TO EDITOR
|Year : 2016 | Volume
| Issue : 3 | Page : 248-250
Recalcitrant pyoderma gangrenosum treated with cyclosporine in an infant
Teki Satya Sri, Geeta A Kiran, B Udayakumar
Department of DVL, Gandhi Medical College, Hyderabad, Telangana, India
|Date of Web Publication||5-Jul-2016|
Teki Satya Sri
101, Srinidhi Arcade, Behind Axis Bank, SR Nagar, Hyderabad - 500038, Telangana
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Sri TS, Kiran GA, Udayakumar B. Recalcitrant pyoderma gangrenosum treated with cyclosporine in an infant. Indian J Paediatr Dermatol 2016;17:248-50
|How to cite this URL:|
Sri TS, Kiran GA, Udayakumar B. Recalcitrant pyoderma gangrenosum treated with cyclosporine in an infant. Indian J Paediatr Dermatol [serial online] 2016 [cited 2019 Oct 20];17:248-50. Available from: http://www.ijpd.in/text.asp?2016/17/3/248/179478
Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis of uncertain etiology with an estimated incidence of 3–10/million/year. Only about 4% of the patients are infants and children. Systemic corticosteroids are effective in acute, rapidly progressive form of disease. We report an infant with multiple PG lesions which were refractory to treatment by corticosteroids but showed a significant improvement with cyclosporine.
An 11-month-old male infant presented with spontaneous eruption of painful skin lesions along with a fever of 2 months duration. Cutaneous examination showed multiple papulopustules, plaques and ulcers with central crusting, violaceous edges, rolled borders involving face, scalp, trunk, and extremities [Figure 1]. Complete blood picture showed anemia. erythrocyte sedimentation rate was raised. His renal function test and liver function test were normal. Serological parameters like cytoplasmic antineutrophil cytoplasmic antibody (C-ANCA), perinuclear ANCA, antinuclear antibody, hepatitis B, and hepatitis C serology were negative. Pathergy test was positive. HIV ELISA was nonreactive. Chest X-ray, ultrasound abdomen, and colonoscopies were normal. Blood culture was negative. Bone marrow aspiration ruled out hematological malignancy. Peripheral smear showed iron deficiency anemia and neutrophilic leukemoid reaction. Biopsy showed epidermis with ulceration, undermined edges, and dense neutrophilic infiltrate in the dermis forming a microabscess and dermal adenexae with focal infiltrate of mixed cells, features, suggestive of PG [Figure 2]. Direct immunofluorescence showed insignificant deposits of IgM and C3 along the basement membrane. Serum protein electrophoresis ruled out multiple myelomas. Coagulation panel showed no evidence of coagulopathy. Fungal, bacterial, and mycobacterial tissue cultures were negative. With the above clinical picture and laboratory findings, a final diagnosis of PG was made.
|Figure 2: Histopathological examination showing neutrophilic microabscesses in the dermis|
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The patient was treated with oral steroids (1 mg/kg/day) for 6 weeks besides local wound care and topical medications. As the recovery was very slow, cyclosporine was introduced in a dose of 3 mg/kg/day and gradually increased to 5 mg/kg/day over a period of 1 month with the dramatic healing of skin lesions with cribriform scarring and no development of new lesions until date [Figure 3].
|Figure 3: Complete remission of pyoderma gangrenosum lesions after cyclosporine therapy|
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| Discussion|| |
PG is a destructive, necrotizing, noninflammatory neutrophilic dermatosis. PG is secondary to an underlying disease in >50% of cases.
PG is classified into ulcerative, pustular, bullous, and vegetative forms. The ulcerative form is the most common form in children in association with ulcerative colitis followed by leukemia and Crohn's disease. But in our case, there was no systemic disease. Ulcerative PG is often treated with high-dose corticosteroids (prednisone 1–2 mg/kg/day). A potent immunosuppressive agent especially cyclosporine is a useful substitute in patients resistant to corticosteroid therapy, and it has the advantage of not causing any myelosuppression. Its use is usually restricted to idiopathic variant because of its own side effects profile like hypertension, hirsutism, hepatotoxicity, central nervous system abnormalities, nephrotoxicity, and a potential for lymphoma development. Its mechanism of action is by binding intracellular protein cyclophilin and forming a complex that inhibits phosphatase calcineurin. This activity prevents transcription of interleukin-2 by T-cells. This breaks the vicious cycle of T-cell, proliferation, autoimmune inflammation, and destruction.
| Conclusion|| |
We conclude that cyclosporine can be considered in infants with severe PG refractory to systemic corticosteroids.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Graham JA, Hansen KK, Rabinowitz LG, Esterly NB. Pyoderma gangrenosum in infants and children. Pediatr Dermatol 1994;11:10-7.
Wollina U. Pyoderma gangrenosum – A review. Orphanet J Rare Dis 2007;2:19.
Powell FC, Schroeter AL, Su WP, Perry HO. Pyoderma gangrenosum: A review of 86 patients. Q J Med 1985;55:173-86.
Crowson AN, Mihm MC Jr, Magro C. Pyoderma gangrenosum: A review. J Cutan Pathol 2003;30:97-107.
Brynskov J, Freund L, Rasmussen SN, Lauritsen K, de Muckadell OS, Williams N, et al
. A placebo-controlled, double blind, randomized trial of cyclosporine therapy in chronic Crohn's disease. Engl J Med 1989;321:845-50.
[Figure 1], [Figure 2], [Figure 3]