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CORRESPONDENCE
Year : 2011  |  Volume : 56  |  Issue : 3  |  Page : 355-357
Pyoderma gangrenosum: Variation in clinical presentation at different ages


1 Department of Dermatology, KS Hegde Medical Academy, Deralakatte, Mangalore, India
2 Department of Pathology, KS Hegde Medical Academy, Deralakatte, Mangalore, India
3 Department of Paedidatrics, KS Hegde Medical Academy, Deralakatte, Mangalore, India

Date of Web Publication30-Jun-2011

Correspondence Address:
Banavasi S Girisha
Department of Dermatology, KS Hegde Medical Academy, Deralakatte, Mangalore
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.82494

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How to cite this article:
Girisha BS, Shenoy MM, Mathias M, Shenoy V. Pyoderma gangrenosum: Variation in clinical presentation at different ages. Indian J Dermatol 2011;56:355-7

How to cite this URL:
Girisha BS, Shenoy MM, Mathias M, Shenoy V. Pyoderma gangrenosum: Variation in clinical presentation at different ages. Indian J Dermatol [serial online] 2011 [cited 2020 Jul 13];56:355-7. Available from: http://www.e-ijd.org/text.asp?2011/56/3/355/82494


Sir,

Pyoderma gangrenosum (PG) is an inflammatory condition of the skin, which was first described by Brunsting et al, in 1930. The etiology of PG is unknown. Initially, it was thought to be a cutaneous gangrene complicating streptococcal infection. It was found to be irrelevant and several theories have been postulated since then. However, none are consistent in all patients. PG is more frequent in adults, but children may be affected on rare occasions. Reported incidence in children is only 4% in the largest series of PG. [1] We report two cases of PG in a child and an adult patient, with variations in clinical presentation.

Case 1: A 3-year-old female child with a history of recurrent ulcerations over the extremities and back since 5 months was referred by a paediatrician. It started as a pustule and then enlarged and ruptured to form an ulcer. It refused to heal despite the antibiotic therapy and similar fresh ulcers also appeared. No other significant medical or surgical history was recorded. On examination, the child had multiple irregular-to-round ulcers of varying sizes along with few pustular lesions distributed over the thighs, gluteal region and back [Figure 1] and [Figure 2]. Mucous membranes were not involved and there was no lymphadenopathy. Patient was hospitalized and investigated. She had mild anemia and her total count was high (16,500 cells/cm 3 ). Her erythrocyte sedimentation rate (ESR) was also high (45 mm at the end of one hour) and platelet count was also above normal limits (4,50,000/cm 3 ). The peripheral smear report of the patient showed normocytic hypochromic anemia, neutrophilia with a mild shift to the left with thrombocythemia. Abdomino-pelvic ultrasonogram showed normal results. The results for Venereal Disease Research Laboratory (VDRL), antinuclear antibody (ANA), enzyme-linked immunosorbent assay (ELISA) for HIV 1 and 2 were negative. During the stay in hospital, the child developed similar pustular lesions at the site of intravenous cannula, representing the phenomenon of pathergy. A biopsy was taken from the edge of a lesion. Histopathology revealed dense mixed cell infiltration in the dermis with areas of haemorrhage and necrosis [Figure 3]. The child was treated with methyl prednisolone pulse (30 mg/kg) and systemic antibiotics. Following the initiation of the treatment, ulcers showed signs of improvement.
Figure 1: Irregular to round ulcers over the thigh and leg

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Figure 2: Irregular to round ulcers over the gluteal region and back

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Figure 3: Histopathology shows mixed cellular infiltrate (10×)

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Case 2: A 29-year-old female patient with a history of ulceration in the right lower leg since 6 months, was referred from the surgery department. Ulcer was refractory to treatment in spite of antibiotics and antiseptic therapy. On detailed history, it was noted that patient was diagnosed as having ulcerative colitis 9 years ago. She was started on oral mesalazine tablets, which she took for 2 years and stopped on her own choice. She was having bouts of loose stools, with blood and mucous during the last 7 years. She developed an ulcer over the right lower leg 6 months back. The ulcer started spontaneously as a small erosion and gradually increased in size. No other significant medical or surgical history was noted. On examination, she was poorly nourished, and had anemia (7.5 g/dl) and clubbing. On her right leg, she had a single ulcer of size 12×8cm. Oozing and crusting was present and, on closer examination, a violaceous hue was noted on the edge of the ulcer [Figure 4]. Her blood investigations revealed that she had normal leucocyte and platelet counts. Liver and renal parameters were within normal limits. Serology results for HIV, HBs Antigen and HCV were negative. The patient was hospitalised and a skin biopsy was taken from the edge of the lesion. Histopathological examination revealed features of vasculitis and mixed cell infiltrate in the dermis [Figure 5]. Correction of anemia was done by blood transfusion and she was started on prednisolone (20mg OD) and mesalazine (800mg TID). During her hospital stay, her condition improved and ulcer healed.
Figure 4: Large ulcer over the right lower leg

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Figure 5: Features of vasculitis and mixed cell infiltrate in the dermis (10×)

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PG commonly occurs between 25 and 54 years of age and occurs rarely in children. The estimated incidence of PG is approximately 3-10 patients per million population per year. [2] Lower extremities are predominantly affected in both adults and children, but preferential involvement of buttocks, perianal and genital region is seen in the latter. Head involvement can also occur in children, which is rare in adults. [1] Both the cases discussed here had the involvement of legs and case 1 had involvement of buttocks, perianal region, and back as well.

Common disease associations are inflammatory bowel disease, arthritis, mono clonal gammopathy, leukemia, immune deficiency state, and HIV infection. [3],[4],[5] The type as well as severity of associated disorders are of prognostic importance. [6] Arthritis is the most common associated disorder in adults, whereas, in children, it is ulcerative colitis. [1],[4] No underlying disease associations could be found in case 1, which can occur in about 25%-50% of PG. [2],[6],[7] Case 2 had ulcerative colitis, and it preceded the onset of PG. In ulcerative colitis, PG can occur at any stage of colitis and even after total colectomy. [2]

Clinical course of case 1 was one of rapid progression, with spontaneous occurrence of new lesions and after trauma, the latter representing the phenomenon of pathergy. It can occur in about 20% of patients. [2] It is believed to be mediated by a host-mediated cellular immune response to skin antigens altered by trauma or surgery and results in the formation of new ulcers. [8] Case 2 had gradually spreading indolent course. Both cases represent the unpredictable nature of the disease.

The diagnosis is mainly clinical, with recognition of evolving clinical features, as histopathology is nonspecific. Six broad disease categories that may simulate PG are vascular occlusive or venous disease, vasculitis, malignancies, infection, exogenous tissue injury, and other inflammatory disorders. [7],[9] These should be specifically ruled out before a diagnosis of PG is made.

Treatment may vary according to the severity of the disease, and is mostly empirical, as the exact pathogenesis is unknown. Goals of treatment are to stop the disease process and promote healing of cutaneous lesions. [2] Systemic steroids and cyclosporine A either alone or in combination are considered to be the first line of treatment. [6] Other agents used include dapsone, clofazimine, thalidomide, azathioprine, and mycophenolate mofetil. Surgery has a limited role to play; however, local debridement and skin grafting may be attempted under immune-suppression to hasten healing, decrease patient morbidity, and hospital stay. [8],[10],[11] Both of our patients exhibited a fairly good response to systemic steroids.

Long-term outcome of PG is not exactly known, as there are no long-term follow-up data available. A high index of suspicion for diagnosis, continuous monitoring, and constant surveillance for associated disorders makes PG both interesting and challenging.

 
   References Top

1.Graham JA, Hansen KK, Rabinowitz LG, Esterly NB. Pyoderma gangrenosum in infants and children. Paediatr Dermatol 1994;11:10-7.  Back to cited text no. 1
    
2.Ruocco E, Sangiuliano S, Gravina AG, Miranda A, Nicoletti G. Pyoderma gangrenosum: An updated review. J Eur Acad Dermatol Venereol 2009;23:1008-17.  Back to cited text no. 2
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3.Powell FC, Su WP, Perry HO. Pyoderma gangrenosum. Classification and management. J Am Acad Dermatol 1996;34:395-412.  Back to cited text no. 3
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4.Bhat RM. Management of pyoderma gangrenosum - An update. Indian J Dermatol Venereol Leprol 2004;70:329-35.  Back to cited text no. 4
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5.Callen JP, Jackson JM. Pyoderma gangrenosum: An Update. Rheum Dis Clin N Am 2007;33:787-802.  Back to cited text no. 5
    
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.  Back to cited text no. 6
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7.Su WP, Davis MD, Weenig RH, Powel FC, Perry HO. Pyoderma gangrenosum: Clinicopathologic correlation and proposed diagnostic criteria. Int J Dermatol 2004;43:790-800.  Back to cited text no. 7
    
8.Rozen SM, Nahabedian MY, Manson PN. Management strategies for pyoderma gangrenosum: Case studies and Review of literature. Ann Plast Surg 2001;47:310-5.  Back to cited text no. 8
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9.Weenig RH, Davis MD, Dahl PR, Su WPD. Skin ulcers misdiagnosed as pyoderma gangrenosum. N Engl J Med 2002;347:1412-8.  Back to cited text no. 9
    
10.Kaddoura IL, Amm C. A Rationale for adjuvant surgical intervention in pyoderma gangrenosum. Ann Plast Surg 2001;46:23-8.  Back to cited text no. 10
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11.Wollina U. Clinical management of pyoderma gangrenosum. Am J Clin Dermatol 2002;3:149-58.  Back to cited text no. 11
[PUBMED]  [FULLTEXT]  


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

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