A four-year old-child with widespread pyoderma gangrenosum resistant to topical treatment

ARTICLE

Auteur(s) : Matteo Auriemma¹, Angelo Carbone¹, Annalisa Blasetti², Anna Maria Tocco², Concetta Di Giulio², Antonello Tulli¹, Paolo Amerio¹

¹Dermatology Clinic G. D'Annunzio University, Via dei Vestini 1, 66100 Chieti, Italy
²Catholic University of the Sacred Heart, Largo Gemelli 8, 00168 Rome, Italy

Pyoderma Gangrenosum (PG) is an uncommon inflammatory disease preferentially affecting middle-aged women; children account for 4-5% of reported cases [1]. It usually presents as painful pustules, blisters or nodules that enlarge and evolve into deep ulcers, eventually healing with cribriform scars. Lesions generally arise on the legs in adults and older children and in the perineum or face in younger patients. The differential diagnosis includes vasculitis, infections, malignancies and factitious ulcers. Nearly half of cases are associated with inflammatory bowel disease (IBD), neoplasms, rheumatic or haematological disorders. The course of the disease can be malignant, chronic or relapsing, usually receding with treatment of the associated disease [2]. We report the case of a child with PG lesions at multiple sites but no associated disease, who responded to systemic treatment with steroid boluses.

A 4-year-old Caucasian girl presented with vesiculo-bullous lesions on the legs and abdomen (figures 1A-D) that had quickly evolved into 3 to 8 cm painful ulcers with undermined purple borders, impairing her daily activities and sleep. Previous systemic antibiotic treatment had failed. Swab cultures were negative for bacterial or mycotic infection. The clinical features suggested PG, which was confirmed by a biopsy with typical histological findings. The work-up for haematological and rheumatological disorders, neoplastic diseases and IBD, including urine and serum protein electrophoresis, complete blood count, peripheral blood lymphocyte phenotyping and a blood smear, disclosed only mild leukocytosis; Ca 19-9, CEA, alpha-foetoprotein, rheumatoid factor, anti-citrulline, ANCA, ANA and ENA antibodies were all in the normal ranges. Although faecal calprotectin was 414 I/U, a colonoscopy disclosed no pathological lesions. A total body CT scan was negative. Treatment with high-potency local steroids (clobetasol propionate 0.05%) and a calcineurin inhibitor cream (tacrolimus 0.03%), according to the literature [1, 3], was ineffective, and new lesions arose on the face and buttocks two weeks into this treatment. The patient was therefore started on methylprednisolone bolus therapy 10 mg/kg/day on 3 consecutive days. Improvement of the pain and of the skin lesions was noted after two cycles, administered 20 days apart. Oral methylprednisolone 1 mg/kg/day, administered between cycles, led to complete resolution of the cutaneous lesions in 30 days (figures 1D-G). The oral steroids were therefore tapered over 5 months, at a rate of 2.5 mg every other week. Glycaemia, blood pressure and body weight showed no significant changes. The patient developed hypertrichosis and striae distensae; her PG lesions healed with cribriform scars. At 17-months follow-up the patient still has no signs or symptoms of a rheumatic condition or IBD.

Childhood PG is uncommon and often starts with vesiculo-bullous lesions that evolve as chronic ulcers. The face and genital area are the commonest sites involved in young children, but our patient's legs were also affected. PG is most frequently associated with ulcerative colitis, but it may precede or accompany Crohn's disease, myeloproliferative disorders, IgA monoclonal gammopathy, leukaemia and rheumatoid arthritis. Nevertheless the work-up failed to disclose any associated disorder and none arose over the 17-month follow-up. PG treatment includes steroids, dapsone, cyclosporine, azathioprine, mycophenolate mofetil, and tumour necrosis factor-alpha antagonists [4, 5], although systemic steroids and cyclosporine, alone or combined, should be considered as the first-line therapy in disseminated PG [6], due to their effectiveness and fast response. This case, where complete and stable remission was achieved after a month, demonstrates that systemic steroids in pulsed doses can be an effective alternative regimen. In conclusion, despite the increasingly frequent reports of treatment with new drugs, we feel that the classic PG medications should be used, especially in children, where a rapid resolution is needed.

Disclosure

References

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3 Chiba T, Isomura I, Suzuki A, Morita A. Topical tacrolimus therapy for pyoderma gangrenosum. J Dermatol 2005; 32: 199-203.

4 Brooklyn TN, Dunnill MG, Shetty A, et al. Infliximab for the treatment of pyoderma gangrenosum: a randomised, double blind, placebo controlled trial. Gut 2006; 55: 505-9.

5 Marzano AV, Tourlaki A, Alessi E, Caputo R. Widespread idiopathic pyoderma gangrenosum evolved from ulcerative to vegetative type: a 10-year history with a recent response to infliximab. Clin Exp Dermato 2008; 33: 156-9.

6 Reichrath J, Bens G, Bonowitz A, Tilgen W. Treatment recommendations for pyoderma gangrenosum: an evidence-based review of the literature based on more than 350 patients. J Am Acad Dermatol 2005; 53: 273-83.