Australas J. Dermatol 1992; 33: 43-44
INFANTILE PYODERMA GANGRENOSUM JAIDEEP SOOD, MOHAN SINGH AND PUSHPA CHATURVEDI
Sevagram, India SUMMARY
A six month old female infant with pyoderma gangrenosum is reported. Pyoderma gangrenosum in an infant is rare. The child responded to pulse therapy with intravenous dexamethasone and intralesional triamcinolone acetonide. Key words: pyodertna gangrenosum, corticosteroid treattnent, pulse therapy. Chest radiograph revealed right-sided patchy consolidation. Blood cultures grew coagulasepositive staphylococci and pus from lesions showed a heavy growth of Klebsiella. A skin pathergy test was performed with intradermal saline injection over the forearm and was highly reactive with subsequent ulceration. The histopathology from a preulcerative nodule revealed central areas of acute inflammatory infiltrate in the mid-dermis extending down to subcutaneous fat, with predominant neutrophils and Iymphomononuclear cells. The blood vessels showed endothelial proliferation and invasion of walls of the blood vessels with the infiltrate.
INTRODUCTION
Pyoderma gangrenosum is an uncommon chronic painful ulcerative skin disease originally described by Brunsting et al in 1930.' The primary lesion is a pustule, papulovesicic, nodule or bulla with a necrotizing inflammatory process extending peripherally resulting in ulcer formation.^ The average age of onset is 40 years and the condition has rarely been reported in children.^ We are reporting pyoderma gangrenosum in an infant with an excellent response to intralesional and systemic suprapharmacologic doses of corticosteroids. CASE REPORT
Her pneumonia responded well to 17 days treatment with gentamicin, cephalexin and penicillin but as fever persisted the patient was given sisomicin and cloxacillin. After one month of antibiotic therapy fever subsided and repeated blood cultures were sterile; during this time she also received a 200 ml blood transfusion. Topical management included potassium permanganate baths and alternate applications of soframycin, neomycin and sodium fusidate, but the lesions continued to extend with small-sized satellite papulonodules which later fused into ulcers. Similar lesions were also noted at sites of intramuscular injections indicating the heightened pathergic response. After one and a half months therapy with topical and systemic antibiotics, IV pulse therapy was given daily with 2.6 mg/kg body weight of dexamethasone into a slow running saline infusion. Oral dapsone was added in a dose of 25 mg daily. Within two weeks the lesions started healing, with healthy granulation tissue in the base of ulcers. To sustain the clinical response, intralesional injection of triamcinolone acetonide was given at the periphery and base of the ulcers. After two months the lesions healed
A six months old female infant was admitted with rapidly progressing necrotic ulcers over the abdomen and later over the buttocks and extremities commencing 15 days before admission. She also had fever and a cough. There was neither colitis nor arthritis. The mother had been receiving streptomycin, isoniazid and rifampicin for pulmonary tuberculosis for six weeks. On examination the patient was febrile and toxic. Large (15 X 9 cms) well-circumscribed ulcers with a necrotic floor and undermined edges were present over the front of chest, abdomen and buttocks. Multiple similar small ulcers were present on the thighs, legs and upper limbs (Fig 1). Significant investigative findings were as follows. Hb 85g/l, total WCC count 14.5 x lOVl with polymorphs 81%, lymphocytes 17% and eosinophils 2%, and ESR 135 mm in one hour. Jaideep Sood, MD. Department of Medicine. Mohan Singh, MD. Department of Dermatology. Pushpa Chaturvedi, MD. Department of Paediatrics. Mahatma Gandhi Institute of Medical Sciences, Sevagram, Wardha District, India. Address for correspondence: Dr J Sood, 88 Celtic Cres, Ellerslie, Auckland, New Zealand.
43
JAIDEEP SOOD, MOHAN SINGH AND PUSHPA CHATURVEDI
FIGURE 1—Large circumscribed ulcers of pyoderma gangrenosum,
completely. At three months follow up the patient remained well and asymptomatic. DISCUSSION
Pyoderma gangrenosum has been associated with a number of systemic diseases. Foremost are ulcerative colitis, rheumatoid arthritis, monoclonal gammopathy and myeloproliferative diseases, but importantly pyoderma gangrenosum can occur in absence of any identifiable disease in 50% of cases." The lesions can be initiated by trauma and may evolve rapidly. The development of initial lesions at the site of typhoid vaccination has been reported,' and the development of lesions at venepucture sites as in our case has also been noted.* Only 4% of a large series of cases of pyoderma gangrenosum occurred before the age of 15 years.^ Ohara et al reported disseminated lesions typical of pyoderma gangrenosum in a 16 month old boy.' Cutaneous lesions af pyoderma gangrenosum have been reported in infants," but our case probably remains the youngest reported. Many of the cases reported in children have posed difficult management problems. Some lesions may heal spontaneously but corticosteroid treatment is normally required. Johnson and Lazarus, in a report of three cases of longstanding resistant pyoderma gangrenosum, administered suprapharmacologic doses of intravenous prednisolone during a five day pulse therapy regimen." More recently clofazimine and 44
thalidomide have been found to be effective in some recalcitrant cases.'" The disease is often difficult to treat and usually lasts several months. Skin grafting of the ulcers is not justified in the active phase of the disease as new lesions may be induced at the donor sites.' REFERENCES ' Brunsting LA, Goeckerman WH, O'Leary P. Pyoderma gangrenosum: Clinical and experimental observations in five cases occuring in adults. Arch Dermatol 193; 22: 655-680. ' Holt PJA, Davies MG, Saunders KC et al, Pyoderma gangrenosum: Clinical and laboratory findings in 15 patients with special reference to polyarthritis. Medicine 1980; 59: 114-133. ' Powell FC, Perry HO, Pyoderma gangrenosum in childhood. Arch Dermalol 1984; 120: 757-761, " Hickman JG, Lazarus GS. Pyoderma gangrenosum: A reappraisal of associated systemic disease, Br J Dermatol 1980; 102: 235-237, ' Jablonska S, Zur pathogenese des Pyoderma gangrenosum, Haulartz 1964; 15: 584-591, ' Ohara K: Two cases of pyoderma gangrenosum, J Kans Med School 1965; 17: 88-101, ' Glass AT, Bancila E, Milgraum S, Pyoderma gangrenosum in infancy: The youngest reported patient, J An^ Acad Dermalol 1991; 25: 109-110, ' Dick DC, Mackie RM, Patrick WJA et al: Pyoderma gangrenosum in infancy, Ada Derm Venereol (Stockh) 1981; 62: 348-350, ' Johnson RB, Lazarus GS, Pulse therapy: Therapeutic efficacy in the treatment of pyoderma gangrenosum. Arch Dermatol 1982; 118: 76-84, '° Venencie PY, Saurat JH. Pyoderma gangrenosum chez un enfant: Traitement par la thalidomide, Ann Pediatr 1982; 29: 67-69,
INFANTILE PYODERMA GANGRENOSUM JAIDEEP SOOD, MOHAN SINGH AND PUSHPA CHATURVEDI
Sevagram, India SUMMARY
A six month old female infant with pyoderma gangrenosum is reported. Pyoderma gangrenosum in an infant is rare. The child responded to pulse therapy with intravenous dexamethasone and intralesional triamcinolone acetonide. Key words: pyodertna gangrenosum, corticosteroid treattnent, pulse therapy. Chest radiograph revealed right-sided patchy consolidation. Blood cultures grew coagulasepositive staphylococci and pus from lesions showed a heavy growth of Klebsiella. A skin pathergy test was performed with intradermal saline injection over the forearm and was highly reactive with subsequent ulceration. The histopathology from a preulcerative nodule revealed central areas of acute inflammatory infiltrate in the mid-dermis extending down to subcutaneous fat, with predominant neutrophils and Iymphomononuclear cells. The blood vessels showed endothelial proliferation and invasion of walls of the blood vessels with the infiltrate.
INTRODUCTION
Pyoderma gangrenosum is an uncommon chronic painful ulcerative skin disease originally described by Brunsting et al in 1930.' The primary lesion is a pustule, papulovesicic, nodule or bulla with a necrotizing inflammatory process extending peripherally resulting in ulcer formation.^ The average age of onset is 40 years and the condition has rarely been reported in children.^ We are reporting pyoderma gangrenosum in an infant with an excellent response to intralesional and systemic suprapharmacologic doses of corticosteroids. CASE REPORT
Her pneumonia responded well to 17 days treatment with gentamicin, cephalexin and penicillin but as fever persisted the patient was given sisomicin and cloxacillin. After one month of antibiotic therapy fever subsided and repeated blood cultures were sterile; during this time she also received a 200 ml blood transfusion. Topical management included potassium permanganate baths and alternate applications of soframycin, neomycin and sodium fusidate, but the lesions continued to extend with small-sized satellite papulonodules which later fused into ulcers. Similar lesions were also noted at sites of intramuscular injections indicating the heightened pathergic response. After one and a half months therapy with topical and systemic antibiotics, IV pulse therapy was given daily with 2.6 mg/kg body weight of dexamethasone into a slow running saline infusion. Oral dapsone was added in a dose of 25 mg daily. Within two weeks the lesions started healing, with healthy granulation tissue in the base of ulcers. To sustain the clinical response, intralesional injection of triamcinolone acetonide was given at the periphery and base of the ulcers. After two months the lesions healed
A six months old female infant was admitted with rapidly progressing necrotic ulcers over the abdomen and later over the buttocks and extremities commencing 15 days before admission. She also had fever and a cough. There was neither colitis nor arthritis. The mother had been receiving streptomycin, isoniazid and rifampicin for pulmonary tuberculosis for six weeks. On examination the patient was febrile and toxic. Large (15 X 9 cms) well-circumscribed ulcers with a necrotic floor and undermined edges were present over the front of chest, abdomen and buttocks. Multiple similar small ulcers were present on the thighs, legs and upper limbs (Fig 1). Significant investigative findings were as follows. Hb 85g/l, total WCC count 14.5 x lOVl with polymorphs 81%, lymphocytes 17% and eosinophils 2%, and ESR 135 mm in one hour. Jaideep Sood, MD. Department of Medicine. Mohan Singh, MD. Department of Dermatology. Pushpa Chaturvedi, MD. Department of Paediatrics. Mahatma Gandhi Institute of Medical Sciences, Sevagram, Wardha District, India. Address for correspondence: Dr J Sood, 88 Celtic Cres, Ellerslie, Auckland, New Zealand.
43
JAIDEEP SOOD, MOHAN SINGH AND PUSHPA CHATURVEDI
FIGURE 1—Large circumscribed ulcers of pyoderma gangrenosum,
completely. At three months follow up the patient remained well and asymptomatic. DISCUSSION
Pyoderma gangrenosum has been associated with a number of systemic diseases. Foremost are ulcerative colitis, rheumatoid arthritis, monoclonal gammopathy and myeloproliferative diseases, but importantly pyoderma gangrenosum can occur in absence of any identifiable disease in 50% of cases." The lesions can be initiated by trauma and may evolve rapidly. The development of initial lesions at the site of typhoid vaccination has been reported,' and the development of lesions at venepucture sites as in our case has also been noted.* Only 4% of a large series of cases of pyoderma gangrenosum occurred before the age of 15 years.^ Ohara et al reported disseminated lesions typical of pyoderma gangrenosum in a 16 month old boy.' Cutaneous lesions af pyoderma gangrenosum have been reported in infants," but our case probably remains the youngest reported. Many of the cases reported in children have posed difficult management problems. Some lesions may heal spontaneously but corticosteroid treatment is normally required. Johnson and Lazarus, in a report of three cases of longstanding resistant pyoderma gangrenosum, administered suprapharmacologic doses of intravenous prednisolone during a five day pulse therapy regimen." More recently clofazimine and 44
thalidomide have been found to be effective in some recalcitrant cases.'" The disease is often difficult to treat and usually lasts several months. Skin grafting of the ulcers is not justified in the active phase of the disease as new lesions may be induced at the donor sites.' REFERENCES ' Brunsting LA, Goeckerman WH, O'Leary P. Pyoderma gangrenosum: Clinical and experimental observations in five cases occuring in adults. Arch Dermatol 193; 22: 655-680. ' Holt PJA, Davies MG, Saunders KC et al, Pyoderma gangrenosum: Clinical and laboratory findings in 15 patients with special reference to polyarthritis. Medicine 1980; 59: 114-133. ' Powell FC, Perry HO, Pyoderma gangrenosum in childhood. Arch Dermalol 1984; 120: 757-761, " Hickman JG, Lazarus GS. Pyoderma gangrenosum: A reappraisal of associated systemic disease, Br J Dermatol 1980; 102: 235-237, ' Jablonska S, Zur pathogenese des Pyoderma gangrenosum, Haulartz 1964; 15: 584-591, ' Ohara K: Two cases of pyoderma gangrenosum, J Kans Med School 1965; 17: 88-101, ' Glass AT, Bancila E, Milgraum S, Pyoderma gangrenosum in infancy: The youngest reported patient, J An^ Acad Dermalol 1991; 25: 109-110, ' Dick DC, Mackie RM, Patrick WJA et al: Pyoderma gangrenosum in infancy, Ada Derm Venereol (Stockh) 1981; 62: 348-350, ' Johnson RB, Lazarus GS, Pulse therapy: Therapeutic efficacy in the treatment of pyoderma gangrenosum. Arch Dermatol 1982; 118: 76-84, '° Venencie PY, Saurat JH. Pyoderma gangrenosum chez un enfant: Traitement par la thalidomide, Ann Pediatr 1982; 29: 67-69,
