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Table of Contents 
Year : 2012  |  Volume : 57  |  Issue : 1  |  Page : 48-49
Progressive and extensive ulcerations in a girl since 4 months of age: The difficulty in diagnosis of pyoderma gangrenosum

1 Department of Dermatology, NRS Medical College, Kolkata, West Bengal, India
2 Department of Pediatrics, NRS Medical College, Kolkata, West Bengal, India
3 Department of Pathology, NRS Medical College, Kolkata, West Bengal, India

Date of Web Publication10-Mar-2012

Correspondence Address:
Nilendu Sarma
P.N. Colony, Sapuipara, Bally, Howrah-711 227, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-5154.92678

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A female child developed multiple, progressive, therapy-resistant, painful large ulcers, vesicles, and pustules since her 4 months of age. The ulcers were large, some even measured more than 8 cm; most had violaceous undermined margin with surrounding erythematous halo, raw and crusted surface and were distributed extensively over scalp, face, ear, trunk, buttocks, thigh, legs, dorsum of hands, and feet without any mucosal involvement. After detail clinical examination and investigation, it was diagnosed as a case of pyoderma gangrenosum. Extensive search did not reveal any systemic abnormality or detect any infective etiology. The case highlights the problems of diagnosis of multiple ulcers at very early age.

Keywords: Children, diagnosis, pyoderma gangrenosum

How to cite this article:
Sarma N, Bandyopadhyay SK, Boler AK, Barman M. Progressive and extensive ulcerations in a girl since 4 months of age: The difficulty in diagnosis of pyoderma gangrenosum. Indian J Dermatol 2012;57:48-9

How to cite this URL:
Sarma N, Bandyopadhyay SK, Boler AK, Barman M. Progressive and extensive ulcerations in a girl since 4 months of age: The difficulty in diagnosis of pyoderma gangrenosum. Indian J Dermatol [serial online] 2012 [cited 2023 Sep 24];57:48-9. Available from:

   Introduction Top

Pyoderma gangrenosusm (PG) is a chronic, rare, inflammatory cutaneous disorder and is manifested by the development of painful ulcers, bulla, pustules and rarely vegetating lesions and histologically by predominantly neutrophilic infiltration. Onset before 6 months of age is extremely rare. We report a case of severe PG in a girl since her 4 months of age. The difficulty in diagnosis and methodical approach in such cases is highlighted.

   Case Report Top

A 6-month-old Hindu girl presented with progressive, extensive, and painful ulcers since her 4 months of age.

Ulcers developed from erythematous papules and vesicles, had purple red undermined margin, raw or crusted surface, merged with other ulcers in the neighborhood and formed large (even >8 cm) lesions with annular, polycyclic or crescent shape. Smaller vesico-pustular lesions were seen surrounding the larger ulcer. They were distributed over scalp, face, ear, trunk (less over anterior trunk), buttocks, thigh, legs, dorsum of hands and feet without any mucosal involvement [Figure 1].
Figure 1: The clinical photographs showing extensive ulcerations almost all over the body. (a) Face, (b) arm, (c) dorsum of hand, (d) leg, (e) thigh, (f) back, (g) encircled site where intradermal normal saline was injected, (h) same site after 48 h showing pustulation (positive pathergy test)

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The child was playful with normal physical and intellectual growth. There was mild fever, moderate pallor without any cyanosis, clubbing, organomegaly, lymphadenopathy, or joint abnormality. There was no family history of similar illness and consanguinity in the parents. Repeated course of broad-spectrum systemic antibiotics was ineffective.

Pustule developed following intradermal injection of normal saline on normal skin (positive pathergy test) [Figure 1] (g, h).

There was low hemoglobin (8.9 gm/dl), leucocytosis (22,600/cmm), neutrophilia (70%), and elevated sedimentation rate (69 mmHg).

Chest X-ray, Mantoux test, DNA-PCR for tuberculosis, immunoglobulin level, RA factor, ANA (Hep-2 cell), VDRL, c-ANCA, HIV- ELISA, pus culture, and colonoscopy of rectal mucosa revealed no abnormality.

Histopathology of the skin biopsies with H and E stain from the margin of the ulcer from two different sites showed epidermal ulceration and dense dermoepidermal collection of inflammatory exudates consisting predominantly of neutrophils with occasional multinucleated giant cells [Figure 2]. One of the biopsies also showed foci of granulomatous reaction [[Figure 2], inset]. Z-N stain (for AFB) and PAS stain (for fungus) were negative.
Figure 2: Photomicrograph showing ulceration of epidermis and collection of inflammatory exudates along with occasional giant cells (H and E, ×100). Inset showing ill formed granuloma with giant cells (H and E, ×400)

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It was diagnosed as a case of pyoderma gangrenosum. The patient responded satisfactorily to oral prednisolone (2 mg/kg/day). However, there were signs of relapse with dose less than 1 mg/kg/day. At that time, the patient became irregular in follow-up and finally lost to follow up.

   Discussion Top

In most of the cases, PG is associated with some systemic diseases like inflammatory bowel diseases (IBD), rheumatoid arthritis, myeloproliferative disease, etc. [1] No systemic abnormality was however detected in this case.

Extensive distribution of the disease was unusual for an infant with PG who generally have ulcers on head and buttocks. Only 16 cases of infantile PG have been reported so far. Onset at 4 months of age was extremely rare and possibly not reported so far.

PG is very rare and lacks any confirmatory test. So misdiagnosis is frequent. [2],[3] Due to much atypicality, diagnosis was difficult and many possibilities were considered like PG, tuberculous, atypical mycobacterial infection, fungal, malignant and vasculitic ulcers, Wegener's granulomatosis, sweet's syndrome, and epidermolysis bullosa.

Violaceous undermined margin with erythematous halo, pain, characteristic histological appearance and positive pathergy test were helpful for diagnosis.

The case highlighted few important things. Granuloma and giant cells may be present in PG. [4] Systemic involvement may not be present or apparent in all cases. Multiple site colonoscopic biopsies from even normal rectal mucosa could have increased the diagnostic yield for any subclinical evidence of IBD.

The case is reported because it had so many unique and atypical features. It highlighted the diagnostic difficulty of persistent ulcers in infancy.

   References Top

1.van den Driesch P. Pyoderma gangrenosum: A report of 44 cases with follow-up. Br J Dermatol 1997;137:1000-5.  Back to cited text no. 1
2.Contreras-Ruiz J, Kresch-Tronik NS, de la Cruz-Garcia MI, Mercado-Ceja S, Lozano-Platonoff A. Delayed diagnosis of pyoderma gangrenosum: A case study. Ostomy Wound Manage 2008;54:32-6.  Back to cited text no. 2
3.Weenig RH, Davis MD, Dahl PR, Su WP. Skin ulcers misdiagnosed as pyoderma gangrenosum. N Engl J Med 2002;347:1412-8.  Back to cited text no. 3
4.Sanders S, Tahan SR, Kwan T, Magro CM. Giant cell in pyoderma gangrenosum. J Cutan Pathol 2001;28:97-100.  Back to cited text no. 4


  [Figure 1], [Figure 2]

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