Grzybowski's generalized eruptive keratoacanthoma: remission with cyclophosphamide

A 57-year-old woman presented with intensely pruritic generalized eruptive keratoacanthomas affecting cutaneous and mucosal surfaces (Grzybowski syndrome). She derived marginal benefit from anti-pruritic agents, acitretin and methotrexate. However, cyclophosphamide 100 mg daily for 1 month followed by 3 months at 200 mg daily resulted in remarkable improvement and eventual remission without further treatment. The disease resulted in severe ectropion of upper and lower eyelids bilaterally. Two years after the onset of her disease, the lower lid ectropions were repaired using skin grafting. There was no evidence for papillomavirus infection.