eMedicine Specialties > Dermatology > Papulosquamous Diseases
Vohwinkel Syndrome: Follow-up
Updated: Feb 19, 2009
Follow-up
Further Inpatient Care
- Regular follow-up by a dermatologist is recommended.
- Refer patient to a surgeon if pseudo-ainhum develops.
- Further care and laboratory testing or radiography depend on the treatment selected, course, and complications.
- Audiologists and speech therapists may be referred on an individual basis.
Complications
- Loss of digit(s) with functional impairment of the limb
- Hearing loss
Prognosis
- The prognosis is good as long as medications are used.
Patient Education
- Tailor patient education to the course and complications.
Miscellaneous
Medicolegal Pitfalls
- Medical pitfalls
- Failure to offer symptomatic treatment
- Failure to discuss the adverse effects of treatment
- Failure to offer surgical interventions and evaluation by specialists
- Failure to detect the potential adverse effects of retinoids
- Legal pitfalls (No specific legal actions are known.)
- Failure to discuss adverse effects of treatment options
- Disregarding contraindications
- Possibly not offering genetic counseling to affected family members
Special Concerns
- Pregnancy
- Genetic counseling is advisable to discuss risk of the offspring being affected by the syndrome
- If topical retinoids are used in women with childbearing potential, inform patients about teratogenicity and apply current guidelines
More on Vohwinkel Syndrome |
| Overview: Vohwinkel Syndrome |
| Differential Diagnoses & Workup: Vohwinkel Syndrome |
| Treatment & Medication: Vohwinkel Syndrome |
Follow-up: Vohwinkel Syndrome |
| Multimedia: Vohwinkel Syndrome |
| References |
| « Previous Page | Next Page » |
References
Lucker GP, Van de Kerkhof PC, Steijlen PM. The hereditary palmoplantar keratoses: an updated review and classification. Br J Dermatol. Jul 1994;131(1):1-14. [Medline].
Bondeson ML, Nyström AM, Gunnarsson U, Vahlquist A. Connexin 26 (GJB2) mutations in two Swedish patients with atypical Vohwinkel (mutilating keratoderma plus deafness) and KID syndrome both extensively treated with acitretin. Acta Derm Venereol. 2006;86(6):503-8. [Medline].
Kelsell DP, Wilgoss AL, Richard G, Stevens HP, Munro CS, Leigh IM. Connexin mutations associated with palmoplantar keratoderma and profound deafness in a single family. Eur J Hum Genet. Jun 2000;8(6):469-72. [Medline].
Solis RR, Diven DG, Trizna Z. Vohwinkel's syndrome in three generations. J Am Acad Dermatol. Feb 2001;44(2 Suppl):376-8. [Medline].
Drera B, Tadini G, Balbo F, Marchese L, Barlati S, Colombi M. De novo occurrence of the 730insG recurrent mutation in an Italian family with the ichthyotic variant of Vohwinkel syndrome, loricrin keratoderma. Clin Genet. Jan 2008;73(1):85-8. [Medline].
White TW. Functional analysis of human Cx26 mutations associated with deafness. Brain Res Brain Res Rev. Apr 2000;32(1):181-3. [Medline].
Camisa C, Rossana C. Variant of keratoderma hereditaria mutilans (Vohwinkel's syndrome). Treatment with orally administered isotretinoin. Arch Dermatol. Oct 1984;120(10):1323-8. [Medline].
Spitz JL. Genodermatoses: A Clinical Guide to Genetic Skin Disorders. 2nd ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2004.
Further Reading
Keywords
Vohwinkel syndrome, keratoderma hereditaria mutilans, palmoplantar keratoderma mutilans, autoamputation, palmar keratosis, plantar keratosis, pseudo-ainhum, hearing loss
Follow-up: Vohwinkel Syndrome