English

Abstract

Pyoderma Gangrenosum (PG) rare neutrophilic dermatoses (1/100,000), of which Bullous Pyoderma gangrenosum is an atypical form, which is very rare. Bullous PG is usually associated with haematological disorders like myeloproliferative disorders, haematological malignancies specially AML and several other haematological disorders. It presents as a superficial haemorrhagic bulla which ulcerates, ulcers increase in size and heal with scarring. Treatment is mainly to identify and treat the cause. Pyoderma Gangrenosum shows rapid response to oral corticosteroid therapy. 1, 3 . Clinical presentation: A 32yr old female presented with fever, multiple vesicles on face, upper limb, lower limb and trunk, and these early lesions looked like chicken pox lesions. Lesions increased in size to form haemorrhagic bullas which eroded to form ulcers, ulcers rapidly increased in size with necrotic base and erythematous to violaceous border. Investigation: Haemoglobin: 5.7gm%, Peripheral smear: normocytic and normochromic anaemia. Skin Biopsy: Sub corneal blisters with dermal and perifollicular neutrophilic infiltrate. A diagnosis of Bullous Pyoderma gangrenosum was made. Patient's anaemia was treated; oral prednisolone and topical steroids were started. Patient showed marked improvement to treatment. INTRODUCTION: Pyoderma gangrenosum (PG) is a rare, non-infectious neutrophilic dermatoses commonly associated with underlying systemic disease. Diagnosis is based on typical clinical features and exclusion of other cutaneous ulcerating diseases. It is associated with ulcerative colitis, Crohn's disease, Rheumatoid arthritis, Connective tissue disease like SLE, certain infections and drugs. Bullous PG is a rare atypical variant, which presents with superficial haemorrhagic bulla and is usually associated with haematological disorders like haematological malignancies, myeloproliferative disorders and other haematological disorders. Skin biopsy shows intra epidermal bulla with dermal and perifollicular neutrophilic infiltrate. Treatment is to identify and treat the cause. Good clinical improvement is seen with systemic steroids and topical steroids. This article describes a rare case of Bullous PG, the early lesions of which mimicked chicken pox and patient showed marked response to systemic steroids. CASE REPORT: A 32 year old female presented with fever, multiple vesicles and bulla on face, upper limb, lower limb and trunk, burning sensation on lesions. Many vesicles were present on an erythematous base, like a dew drop on rose petal appearance, at this point a clinical diagnosis of chicken pox was made and treated was started for the same. Vesicles increased in size to form a large haemorrhagic bulla, bulla eroded to form ulcers. The ulcers rapidly increased in size by more than 1 cm/day. These ulcers had a necrotic base and erythematous to violaceous border and central black eschar.