|Year : 2017 | Volume
| Issue : 1 | Page : 55-57
Childhood pyoderma gangrenosum
Mayur Ranu Bhobe, Shylaja Someshwar, Hemangi R Jerajani, Dedhia Ami
Department of Skin and VD, MGM Medical College, Navi Mumbai, Mumbai, Maharashtra, India
|Date of Web Publication||2-Feb-2017|
Mayur Ranu Bhobe
House No. 613/E/D/C, Cupangal, Behind Chowgule College, Fatorda, Salcete - 403 602, Goa
Source of Support: None, Conflict of Interest: None
Pyoderma gangrenosum (PG) is rare in children. We report a case of PG in a 4-year-old male who presented with ulcers over face, axillae, and gluteal area. The ulcers healed with cribriform scarring after starting systemic steroids and azathioprine. The patient, however, was unable to follow-up regularly. He had a recurrence of lesions developed sepsis and septic shock and unfortunately expired.
Keywords: Child, cribriform scars, pyoderma gangrenosum, ulcer
|How to cite this article:|
Bhobe MR, Someshwar S, Jerajani HR, Ami D. Childhood pyoderma gangrenosum. Muller J Med Sci Res 2017;8:55-7
| Introduction|| |
First described in 1930 by Brunsting et al., pyoderma gangrenosum (PG) is an idiopathic neutrophilic dermatosis that was once considered pathognomonic of idiopathic ulcerative colitis. The histopathologic distinction of PG from other ulcerative processes with dermal neutrophilia is challenging and at times impossible.
| Case Report|| |
A 4-year-old male child presented with ulcers over axillae, face, gluteal area, and legs for 15 days. Lesions reportedly began as blisters over both axillae which ruptured oozing clear fluid leaving ulcers that gradually increased to the current size. Similar lesions developed over buttocks, cheeks, and back of knees 5 days later. The ulcers were punched out with slough over the floor and violaceous rim [Figure 1],[Figure 2],[Figure 3]. The base was formed by muscle. There was no history of altered bowel habits or abdominal pain. There was no history of joint pains. There was no history of tiredness, spontaneous bleeding. The patient was investigated with a differential diagnosis of ecthyma gangrenosum, ecthyma, PG, pyoderma vegetans, chancriform pyoderma, and tuberculous gumma. Biopsy taken from the edge of the ulcer revealed focal loss in continuity of the epidermis with the center showing homogeneous eosinophilic material with neutrophilic microabscesses [Figure 4] and [Figure 5]. After clinicopathological correlation, diagnosis of PG was made.
|Figure 1: Ulcers over axilla with punched out edges and slough over floor|
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|Figure 4: Biopsy from the edge of ulcer showing loss in continuity of epidermis with homogeneous eosinophilic material (H and E, ×10)|
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|Figure 5: Neutrophilic infiltrates in the floor of the ulcer (H and E, ×40)|
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There was an increase in total count with neutrophilia (absolute neutrophil count - 15,960). Tuberculin test was negative. Pathergy test was also negative. Culture from pus grew Escherichia coli and Pseudomonas. Stool examination was normal.
The patient was put on intravenous antibiotics and dressings with colloidal silver. However, there was no significant improvement. He was later started on systemic steroids at 1 mg/kg and azathioprine 20 mg at 1 mg/kg. Lesions healed with cribriform scars but would recur on attempting to taper steroids. The patient had started on pulse methylprednisolone 500 mg on 3 days/month. Unfortunately, the patient did not follow-up and developed a flare and died of septic shock.
| Conclusion|| |
PG is a rare dermatosis. It usually occurs between 25 and 54 years of age and occurs rarely in children. In a study carried out at the Mayo Clinic, in a review of 180 cases diagnosed between 1930 and 1982, it was seen that only eight cases (4%) involved children younger than 15 years. In a separate study of 86 patients seen at the Mayo clinic from 1970 to 1983, four cases (4.6%) were aged <14 years of age. In adults, the lesions are commonly seen on the lower extremities; whereas in children, the lesions are also seen on the head and face, buttocks, and perianal and genital regions. The above distribution of lesions was also seen in our case. In children, lesions begin as pustules rather than macules or papules.,, Unlike in adults where the legs are more commonly involved, in children, the distribution is more generalized and the general prognosis is better than in adults.
PG is known to be associated with arthritis, inflammatory bowel disease, hematologic malignancy, monoclonal gammopathy, and myeloma. In adults, PG is most frequently associated with arthritis; whereas in children, it is associated with ulcerative colitis,,, followed by leukemia  and Crohn's disease. We could not find any associated systemic disease despite thorough evaluation.
Systemic steroids are considered the drug of choice. Sulfa drugs such as sulfasalazine, sulfapyridine, and sulfamethoxypyridine have also been used. Refractory lesions have been successfully treated with the addition of clofazimine, dapsone, rifampicin, azathioprine, cyclophosphamide, and cyclosporine to the steroid regimen.,, Although pediatric PG has a good prognosis, this patient ultimately died of septic shock despite initial response to immunosuppressive treatment.
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.
Financial Support and Sponsorship
Conflicts of Interest
There are no conflicts of interest.
| References|| |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]