Palmoplantar Keratoderma and Skin Grafting: Postsurgical Long‐term Follow‐up of Two Cases with Olmsted Syndrome

Abstract

Olmsted syndrome is a rare congenital mutilating palmoplantar keratoderma associated with periorificial keratotic plaques. Treatment options include topical keratolytics, systemic retinoids, and debulking procedures. Full-thickness excision of hyperkeratotic plaques followed by skin grafting has been reported in the medical literature, although long-term results have not been evaluated. We present two cases of Olmsted syndrome with severe palmoplantar keratoderma treated with excision and skin grafting, along with long-term clinical results 11 years (patient 1) and 6 years (patient 2) following the initial surgery.

Palmoplantar keratoderma and perioral keratotic plaques with hypotrichosis in a child

“…Systemic retinoids have been reported to result in partial improvement but are ineffective in blocking the progressive course . Excision and skin grafting of severe keratoderma showed favorable long‐term clinical results in some patients . Erlotinib, an epidermal growth factor receptor inhibitor, showed thinning of keratoderma and resolution of perioral plaques in one OS patient .…”

“…7 Excision and skin grafting of severe keratoderma showed favorable long-term clinical results in some patients. 8 Erlotinib, an epidermal growth factor receptor inhibitor, showed thinning of keratoderma and resolution of perioral plaques in one OS patient. 9 Therapeutically, our patient achieved partial relief with the oral acitretin 20 mg daily and topical 20% urea cream.…”

Surgical treatment of Papillon–Lefèvre Syndrome with bovine collagen and skin graft

“…Despite complete debridement, recurrences have been reported in the grafted areas and at the periphery of the graft. 3 We recommend excision down to muscle or plantar fascia and bovine collagen with skin graft for the reconstruction. The bovine collagen was used to increase the dermal thickness in the plantar area, which is particularly important in the forefoot and hindfoot.…”

Pain mechanisms in hereditary palmoplantar keratodermas

“…Home remedies, wet soaks, salicylic acid, urea, tar, shale oil, antibacterial treatment, retinoids and corticosteroids have been utilized to treat OS pain . More extreme measures such as complete removal of the affected skin and subsequent skin graft have also been used . Though some individuals have reported initial relief from this procedure, keratoderma often returns in the grafted tissue …”