Pyoderma Gangrenosum in a 10-year-old boy

presented by

Henry Foong FRCP

Ipoh, Malaysia

December 31, 2006

Consultant Dermatologist, Foong Skin Specialist Clinic, Ipoh, Malaysia

Abstract Reported here is a 10-year-old boy who presented with a painful necrotic leg ulcer. Toilet and debridement of the ulcer aggravated the lesion and triggered off new lesions. This feature (pathergy) should alert the clinician the diagnosis of pyoderma gangrenosum. He was treated with oral prednisolone and cyclosporin which showed good response.
Y.C.H., 10-year-old boy
2 years
Lower limbs

The ulcer started as a mosquito bite on the left calf which gradually increased into an ulcerative lesion despite treatment. He had seen a pediatrician, a dermatologist and orthopedic surgeons. 2 years ago, an orthopedic surgeon did a skin graft for him for a leg ulcer but the graft did not take.

About a month ago, he developed a rapidly progressing painful ulcer on the left leg again. An orthopedic surgeon did a toilet and debridement for him and this triggered off new lesions just above it and also on the gluteal areas.

He had no symptoms of diarrrhea. No mouth ulcers. No bleeding tendency.

Physical Examination

Pertinent findings were a deep ulcer 8cm x 6cm on the lateral aspect of the lower 1/3 of the left leg. It has an irregular, violaceous and undermining border surrounding th ulcer. Just superior to it, was another similar but smaller necrotic ulcer. Multiple smaller necrotic ulcers were also noted on the gluteal areas.

Abdominal examination was normal. Rest of examination was unremarkable.


Pyoderma gangrenosum showing the irregular, violaceous and undermined border surrounding the deep ulcer.

Similar necrotic ulcers on the gluteal areas.

Pyoderma gangrenosum gradually resolving after treatment with prednisolone, cyclosporine and antibiotics at 3 weeks.

Laboratory Data

Hb 11.3gm% TWBC 18,000 (N88, L10,M1, E1) Platelets 370 000

Urea 10 mg/dl Creatinine 0.5 mg/dl

ANA negative

Rh Factor negative

HIV negative

Culture: Heavy growth of pseudomonas aeruginosa sensitive to ciprofloxacin but resistant to cefuroxime, ceftazidime, gentamicin and amikacin.


Non-specific ulcer with neutrophilic infiltrates in the dermis. No granuloma seen. No malignancy.

Diagnosis Pyoderma Gangrenosum
Reasons Presented

The child was treated with oral prednisolone 45mg daily, cyclosporine 100mg daily and ciprofloxacin 250mg bd. The blood counts and renal profile was monitored closely. His ulcer gradually resolved with healing. Evaluation for inflammatory bowel disease by paediatrics was negative.

The rapid progression of a painful necrotic ulcer with an irregular undermined border is typical of pyoderma gangrenosum (PG). PG is believed to be a reactive inflammatory dermatoses and is part of the spectrum of neutrophilic dermatoses.

PG is essentially a diagnosis of exclusion. PG has a characteristic clinical appearance but other ulcerative conditions may have similar appearance. Indeed in a study by Weenig GH et al in Mayo clinic, about 10% of the cases were found not to have PG.(1)

The misdiagnosis of pyoderma gangrenosum is not uncommon and exposes patients to risks associated with its treatment. A thorough evaluation is required in all patients suspected of having pyoderma gangrenosum in order to rule out alternative diagnoses.

Proposed diagnostic criteria of PG (Daniel Wu et al).(2) To be considered a definite case of PG, the condition should meet both major and at least two minor citeria. Major Criteria: (i) Rapid progression of a painful necrolytic cutaneous ulcer with an irregular violaceous and undermined border (ii) Other causes of cutaneous ulceration have been excluded. Minor Criteria: (i) History suggestive of pathergy (ii) systemic diseases associated with PG (iii) Histopathologic findings(sterile dermal neutrophilia, +/- mixed inflammation +/- lymphocytic vasculitis (iv) Treatment response (rapid response to systemic steroid treatment)

The introduction of new treatment modalities has improved and broadened therapeutical options for PG. Because the number of other published studies are very low, recommendations have to be interpreted with great care. (4)


What is the experience of other members in these childhood cases?

Has anyone any experience treating pyodema gangrenosum with infliximab or IV immunoglobulins?(3)

  1. Roger H. Weenig et al. Skin Ulcers Misdiagnosed as Pyoderma Gangrenosum.NEJM 2002; 347: 1412-1418 (view abstract)
  2. W P Wu et al. Pyoderma gangrenosum: clinicopathological correlation and proposed diagnostic criteria. Int J Dermatol 2004;43:790-800 ( view abstract)
  3. Brooklyne TN et al. Infliximab for the treatment of pyoderma gangrenosum: a
    randomised, double blind, placebo controlled trial. Gut. 2006 Apr;55(4):505-9 (view abstract)
  4. Treatment recommendations for pyoderma gangrenosum: An evidence-based review of the literature based on more than 350 patients. J Am Acad Dermatol 2005; 53: 273-283. (view abstract)
Key Words pyoderma gangrenosum, childhood, cyclosporine
Comments from Faculty and Members

Jeffrey Callen MD, Professor of Medicine (Dermatology), Chief, Division of Dermatology, Department of Medicine, University of Louisville School of Medicine, Louisville, KY, USA on Dec 31, 2006

The child should be evaluated for inflammatory bowel disease. In addition, if not already done, cultures for AFB and fungi should be performed. Treatment with infliximab is useful, even in the absence of IBD. IVIG in my opinion is unlikely to be effective.
I have a child with chronic and recurrent PG since age 4 - both cyclosporin and infliximab have been helpful, but we have not identified an associated process.

Shahbaz Janjua MD, Specialist Dermatologist, Ayza Skin & Research Center, Lalamusa, Pakistan on December 31, 2006

This is really an impressive case of PG.

I recently treated PG in a 12-year-old boy. He primarily had untreated scabies which got complicated by superaded bacterial infection over a period of one month. He presented with very painful large ulcerated oozing lesions with undermined borders on the extremities. Treatment with topical and oral antibiotics had been unhelpful. I treated this patient with oral prednisolone, ciprofloxacillin, and topical fusidic acid cream. I also prescribed topical permethrin to the patient and all the contacts. A dramatic recovery was seen in one week.

In my opinion many patients would respond to treatment with oral steroids without any need to prescribe other immunosuppressive agents.

Robert I. Rudolph, M.D., FACP, Clinical Professor of Dermatology, University of Pennsylvania, Philadelphia. PA, USA on December 31, 2006

Wow! I tend to agree with the diagnosis, but the ferocity and widespread nature of the lesions makes me very worried about an underlying systemic disease - especially hematologic - despite negative studies at this time. This kid needs very close followup.

I'd also consider a halogenoderma, or atypical erosive Sweet's. I would have said, incidentally, that the buttock lesions were artefactual if they had been the only spots present. Good Luck.

Bushan Kumar MD, Professor of Dermatology, Departtment of Dermatology,
Postgraduate Institute of Medical Education and Research, Chandigarh, India
on Janaury 4, 2007

Interesting case report. The diagnosis is by exclusion as the disease is very uncommon in children - but the clinical picture is quite characteristic. Just a little query in the history of 2 years. Has the lesions been persisting all along? From the line “developed a rapidly progressing painful ulcer on the right leg ‘again’ it seems to indicate that there were no previous lesions.

Why did the patient report to the orthopedic surgeon on both occasions - some joint symptoms as well? Or some other reason?

Histopathological changes in the gut can be present even in the absence of clinical symptoms. In the present episode course of the disease seems to be rather fulminant - any systemic symptoms?

I feel steroids give good results. Immunosuppressives could be added if the response was inadequate with steroids alone.

Henry Foong FRCP, Ipoh, Malaysia on January 21, 2007

This present episode is a relapse of the disease which started about 2 years ago. He was then treated with oral prednisolone but defaulted partly because he developed cushigoid features as a result of the oral corticosteroids treatment.

For this present episode, he was successfully treated with cyclosporin and oral prednisolone with minimal cushigoid features and the dose has been reduced to cyclosporin 100mg daily and prednisolone 5 mg daily. The rest of the perianal lesions has healed completely.

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