DS14 Acquired capillary malformation (Fegeler syndrome) following basal cell carcinoma excision with glabellar flap reconstruction: a rare complication

Abstract

Port-wine stains (PWS) are cutaneous capillary malformations that usually present as congenital vascular birthmarks. They can present typically over the distribution of the trigeminal nerve branches, over the forehead and nose. There are only a handful of reports on acquired PWS, and we report one of the first following cutaneous surgery. A 55-year-old woman was referred for Mohs micrographic surgery (MMS) for a biopsy-proven nodular, micronodular and infiltrative basal cell carcinoma on the right nasal canthus measuring 10 mm. This was removed with two stages of MMS, and the defect was repaired using a glabellar flap. At review 2 months after, the patient reported an asymptomatic well-­defined erythematous area on the forehead with sharp cutoffs. The patient was systemically well, with no other notable medical or drug history, and no childhood history of vascular malformations. She reported the area would become more noticeable on exercise, and denied any improvement with topical corticosteroids. On examination there was a well-defined erythematous area overlying the V1 distribution of the trigeminal nerve, blanching under pressure, consistent with a PWS. The patient was subsequently referred for a trial of pulsed-dye laser treatment. PWSs are vascular malformations characterized by a progressive dilation and ectasia of the superficial cutaneous vascular plexus. Acquired PWSs are very rare, with only limited reports in the literature. Most of these have been due to the trauma, with the first reported case in 1949 by Fegeler (Fegeler syndrome), although since then, nontraumatic acquired PWSs have been reported following medication initiation. The aetiological factors triggering their development remain to be identified, although proposed mechanisms include trauma inducing perivascular atrophy leading to vessel dilation, or impaired reparative processes in vessels resulting in dilated vessel walls. Our case serves to highlight a very rare complication following cutaneous surgery.