4.4 Review

Pyoderma gangrenosum associated with limited cutaneous systemic sclerosis: a rare case with literature review

Journal

CLINICAL RHEUMATOLOGY
Volume 40, Issue 3, Pages 1141-1145

Publisher

SPRINGER LONDON LTD
DOI: 10.1007/s10067-020-05285-z

Keywords

Autoimmune disease; Neutrophilic dermatoses; Pyoderma gangrenosum; Rheumatic disease; Scleroderma; Systemic sclerosis; T cell

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Pyoderma gangrenosum is a skin disease characterized by painful ulcers that can lead to permanent disfigurement if not appropriately treated. It is often observed in various autoimmune disorders but rarely associated with systemic sclerosis. Treatment with intravenous methylprednisolone and topical clobetasol can lead to complete resolution of the ulcerated lesions.
Pyoderma gangrenosum (PG) is a skin disease characterized by painful ulcers that, when not appropriately treated, can lead to permanent disfigurement. Pyoderma gangrenosum has been observed in a multitude of autoimmune disorders such as rheumatoid arthritis, inflammatory bowel disease (IBD), and sarcoidosis (Feld et al. J Rheumatol. 39(1):197,2012; Herrero et al. J Rheumatol. 36:7:1557-1558,2009). It is rarely associated with autoimmune disorders such as systemic sclerosis. We report a case of a patient with known limited cutaneous systemic sclerosis who developed an ulcerated lesion on the 2nd digit of the left hand. The lesion was initially thought to be cellulitis and the patient underwent superficial wound debridement. Postoperatively, the patient's lesions worsened. The patient was treated with intravenous (IV) methylprednisolone and 0.05% topical clobetasol due to high suspicion for PG with complete resolution of ulcerated lesions and minimal scarring.

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