Surgical treatment of keratosis palmaris in Mal de Meleda

Mal de Meleda (MDM, OMIM #248300) is a rare congenital palmoplantar keratosis. Deep fissures cause pain and limit extension of the metacarpo-phalangeal joints. We report the case of a patient operated on both hands with a 29-year interval between each hand. A 53-year-old patient with MDM demonstrated severe keratosis of the left hand. The same surgeon operated on the right hand. Both hands were operated using the same technique. Skin of the palm, the palmar side of the index, and the first phalangeal of third, fourth and fifth fingers were excised with a sharp rugine. The hand was immediately covered by a full thickness-skin graft (FTSG) harvested on groin. The excision of all keratosis on the palm can lead to complete cure of MDM symptoms. FTSG is essential, especially on the fingers, in order to minimize secondary retraction. At the palm, FTSG offers better mechanical resistance then a split-thickness skin graft. A large groin graft, with closure of the donor site in a Y fashion, can cover the entire hand. Long term follow-up (29 years) demonstrates no recurrence of keratosis on surgically treated areas.