A CASE REPORT ON CONGENITAL PROTEIN S DEFICIENCY PRESENTING AS NEONATAL PURPURA FULMINANS

Abstract

Gupta P, Sehgal SA, Dhull AK

PRESENTATION OF CASE A 33-year young male presented with chief complaints of an ulcerated lesion in right inguinal region for 4-months. Patient was apparently asymptomatic 4-months back when he had swelling the size of a lemon in the right inguinal region, associated with continuous dull aching pain, non-radiating and not associated with bowel or bladder complaints. The swelling gradually progressed in size and subsequently developed an ulcerated irregular lesion involving inner aspect of the entire thigh with necrotic slough and pus discharge. There was no history of weight loss, loss of appetite or breathlessness. There were no other significant systemic illnesses. Patient was a chronic smoker for 15- years, non-alcoholic and directly exposed to sun for prolonged periods due to occupational reasons. Systemic examination was normal. All haematological and biochemical parameters were within normal limits. On examination, there was a confluent asymmetric ulceroproliferative lesion of size 20×15 cm, involving inner aspect of right thigh and groin with serosanguinous discharge. The neoplastic cells illustrated wide spread positivity for cytokeratin (CK), epithelial membrane antigen (EMA) and vimentin immunocytochemical stain, while stain for leucocyte common antigen (LCA) was negative. Magnetic resonance imaging revealed a lesion in right inguinofemoral region of 14×3 cm in transverse and antero-posterior diameters. The lesion is seen in cutis and infiltrates the subcutaneous tissue. Multiple prominent and mildly enlarged bilateral inguinal nodes are seen with lesion seen in right half of sacrum. The histopathological appearance, IHC profile of the biopsy tissue and MRI scan confirmed the diagnosis as spindle cell squamous cell carcinoma, stage IV. Subsequently patient received palliative radiotherapy of 800 cGy single session to the involved site with adequate margins by Co-60 external beam radiotherapy and received pain palliation to some extent. However, given the patient’s poor functional status and very rapid progression of the disease, the patient elected to get discharged and was lost to follow-up.

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