Lymphedema in patients with hidradenitis suppurativa: a systematic review of published literature.

BACKGROUND: Lymphedema is a debilitating complication of hidradenitis suppurativa (HS) that can be therapeutically challenging. Systematic evaluation of the clinical presentation and management of this condition has yet to be performed.

METHODS: OVID MEDLINE and EMBASE databases were searched along with a review of the references of searched articles for all English reports discussing lymphedema secondary to HS.

RESULTS: Two case series and 15 case reports met inclusion criteria, representing 27 patients. On average, these patients had a history of HS for 18 years (range 4-30 years). Lymphedema was found in the following areas in order of frequency: scrotum (16 patients, 59%), penis (12 patients, 44%), labia majora (4 patients, 15%), perineum (3 patients, 11%), groin (3 patients, 11%), buttocks (2 patients, 7%), and abdomen (1 patient, 4%). In six patients (22%), lymphedema occurred in two or more of the aforementioned locations. Lymphedema most commonly presented not only as swelling with induration but also as verrucous papules or nodules. The majority of cases (14 patients, 52%) were treated with radical surgical excision combined with medical therapy and obtained favorable outcomes. Surgical defect was most frequently covered with split thickness skin grafts 7 patients, 54%) followed by skin flaps (3 patients, 11%) and healing by secondary intention (3 patients, 11%).

CONCLUSIONS: Lymphedema appears to be a relatively rare complication of chronic HS that most commonly affects the anogenital region. Based on the evidence available, dermatologists should pursue a collaborative approach with surgery in the management of this condition at early stages.