Successful treatment of cutaneous collagenous vasculopathy with cyclophosphamide in a patient with granulomatosis with polyangiitis

Cutaneous collagenous vasculopathy (CCV) is a rare primary form of acquired idiopathic microangiopathy characterized by multiple cutaneous telangiectasias over the trunk and limbs. It is usually benign and is not known to be associated with any systemic complications. We present the case of a Caucasian woman with newly diagnosed granulomatosis with polyangiitis (GPA) who presented with diffuse alveolar hemorrhage associated with elevated anti-proteinase 3 (anti-PR3) levels. She reported having a telangiectatic rash over both forearms and lower limbs since the age of 14. Although initially thought to be a form of cutaneous vasculitis related to GPA, a subsequent skin biopsy confirmed the diagnosis of CCV. She received intravenous cyclophosphamide and corticosteroids (CS) as induction therapy for GPA and noted a complete resolution of CCV after completion of treatment with cyclophosphamide. To our knowledge, this is the first case of CCV being treated successfully with cyclophosphamide and CS.