Treatment of systemic sclerosis complicated with pyoderma gangrenosum with adalimumab: A case report of a rare disease

Abstract

Pyoderma gangrenosum (PG) is a rare noninfectious neutrophilic dermatosis characterized by recurrent, painful ulcers that commonly affect the lower extremities but can also involve other parts of the body. Over half of patients with PG have concomitant systemic immune diseases, with the association of PG with systemic sclerosis (SSc) being extremely rare. Treatment of PG primarily involves local therapy, steroids, and immunosuppressants, with an increasing emphasis on biologic agents. Among these, tumor necrosis factor-alpha (TNF-α) antagonists are considered effective. The patient in this report was an elderly female with a history of systemic sclerosis for many years and initially presented with gangrenous ulcers on the fingertips. After inconclusive conventional treatment, adalimumab was added for 5 weeks, resulting in disease suppression, a reduction in ulcer size, and re-epithelialization of the skin lesions after 6 months.

Keywords: adalimumab; pyoderma gangrenosum; systemic sclerosis.

Figure 1.

(a) The patient presented with ulcers on both lower limbs, with deep ulcers with irregular edges on all four limbs. The edges of the lower limb ulcers are purplish-red, which is consistent with the clinical manifestations of pyoderma gangrenosum (PG). (b) Photos of the patient’s lower limbs at discharge. (c) Follow-up visit in June shows the condition of the patient’s lower limbs; as seen in the images, the ulcers have completely healed, forming scar changes resembling “cigarette paper.” (d) Gulliver’s sign in pyoderma gangrenosum. Note the new epithelial growth connecting the ulcer bed to the surrounding normal skin.

Figure 2.

(a) ×20 magnification and (b) ×200 magnification skin biopsy of the lower limbs showing neutrophil infiltration, pyogenic dermatitis, and epidermal vesicles.