Oral tofacitinib for generalized Dowling-Degos disease with refractory pruritus: a case report

To evaluate the efficacy and safety of the Janus kinase (JAK) inhibitor tofacitinib in generalized Dowling-Degos disease (DDD) with refractory pruritus and to comparatively analyze its advantages and limitations versus existing therapies.

We report a 61-year-old male with a 20-year history of generalized DDD presenting with pruritic erythematous-to-brown papules and macules refractory to conventional therapies. Multimodal diagnostic evaluations, including dermoscopy and histopathology confirmed the diagnosis, with pruritus severity quantified using the Numerical Rating Scale (NRS). The patient was initially treated with oral acitretin (20 mg/day) combined with prednisone (20 mg/day) for 1 month, followed by maintenance monotherapy with tofacitinib (5 mg twice daily).

Oral acitretin exacerbated the pruritus. Following transition to tofacitinib, pruritus was improved from a baseline NRS score of 9 to 5-6 within 1 month. At four-month follow-up, pruritus scores further stabilized at 2-3, accompanied by lesion darkening without recurrence. No adverse events occurred.

Tofacitinib represents a promising therapeutic option for DDD with refractory pruritus.