Abstract

NM-197

Use of Botox injections for treatment of plantar hyperhydrosis in pediatric patient with Pachyonychia Congenita

Willett O, Strickland N
University of Arkansas of Medical Sciences - Arkansas Children's Hospital, Little Rock, Arkansas, United states

Pachyonchia Congenita is a rare congenital dermatological disorder affecting the keratin gene. All patients affected by this disorder have specific changes of the nails and skin. The most common characteristics are hypertrophic nail dystrophy, palmar and plantar keratoderma and plantar pain. The plantar pain is due to formation of blisters in the affected area of hyperkeratosis. This condition is exacerbated in the summer months from hyperhidrosis and increased blister formation due to friction and subsequent trauma.

Our patient is an 11yo female with Pachyonychia Congenita type 1 who was referred to our pain clinic by the dermatology when she was 8 yo due to severe plantar pain. See had extensive blisters and keratoderma on bilateral plantar feet. She was not walking and using a wheelchair to ambulate. She had multiple infections on her feet, which also contributed, to her pain. Our patient was unable to participate in activities of daily living. Usual treatment modalities to treat the hyperkeratosis include emollients, keratolytics, comfortable shoes, debridement of nails and areas of hyperkeratosis however, these are all temporary and only provided her minimal relief.

Botox injections are successful for the treatment of hyperhidrosis in patients with excessive sweating in axillae, palms and soles. In adult patients with Pachyonchia Congenita hyperhydrosis has been successfully treated with Botox injections. There is one pediatric case report published on the use of Botox for hyperhidrosis in Pachyonchia Congenita.

Our patient received treatment with intradermal plantar Botox soon after presentation to the clinic. She reported immediate improvement in plantar pain. She has been followed by our clinic for the last three years with an average of one Botox injection every three months. Her average dose of Botox is 100 mg/kg, which is divided evenly into each foot. Since treatment, her pain has significantly improved, see figure 1,2. She is able to ambulate and participate in activities of daily living.

References:
1. Sancy A. Leachman; et al: Clinical and Pathological Features of Pachyonychia Congenita. J Investig Dermatol Symp Proc. 2005 Oct;10(1):3-17.

2. Sonal Shah, MD; et al. Pachyonychia congenita in pediatric patients: natural history, features, and impact. JAMA Dermatol. 2014 Feb;150(2):146-53. doi: 10.1001/jamadermatol.2013.6448.

3. Swartling C, Karlqvist M; et al. Botulinum toxin in the treatment of sweat-worsened foot problems in patients with epidermolysis bullosa simplex and pachyonychia congenita. Br J Dermatol. 2010 Nov;163(5):1072-6. doi: 10.1111/j.1365-2133.2010.09927.x.