Medical Care

Tailor medical care to individual defects or functional impairment of the limbs or hearing.

Surgical Care

Surgical release of the constriction bands is used to preserve the digits (eg, Z-plasty, other methods for relaxing scars).

Consultations

Consultation with the following specialists may be needed:

Surgeon - If any deformities of the fingers or craniofacial features are present
Audiologist and speech therapist - If hearing loss is noted
Other specialists - As indicated by clinical abnormalities
Genetic counselor - Advisable to discuss risk of the offspring being affected by the syndrome

Diet

No dietary interventions are indicated for treatment. Oral bioavailability of retinoids is enhanced with food intake.

Activity

Activity is unrestricted.

Long-Term Monitoring

Regular follow-up by a dermatologist is recommended. Refer the patient to a surgeon if pseudoainhum 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.

Medication

Lucker GP, Van de Kerkhof PC, Steijlen PM. The hereditary palmoplantar keratoses: an updated review and classification. Br J Dermatol. 1994 Jul. 131(1):1-14. [QxMD MEDLINE Link].
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Seirafi H, Khezri S, Morowati S, Kamyabhesari K, Mirzaeipour M, Khezri F. A new variant of Vohwinkel syndrome: a case report. Dermatol Online J. 2011 Mar 15. 17(3):3. [QxMD MEDLINE Link].
Avshalumova L, Fabrikant J, Koriakos A. Overview of skin diseases linked to connexin gene mutations. Int J Dermatol. 2014 Feb. 53 (2):192-205. [QxMD MEDLINE Link].
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Nico MMS, Fernandes JD. Low-dose isotretinoin prevents digital amputation in loricrin keratoderma (Vohwinkel syndrome with ichthyosis). J Dtsch Dermatol Ges. 2017 Jun. 15 (6):665-667. [QxMD MEDLINE Link].
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Media Gallery

Starfish-shaped plaques.
Palmar hyperkeratosis.
Pseudoainhum.

of 3

Author

Zoltan Trizna, MD, PhD Private Practice

Zoltan Trizna, MD, PhD is a member of the following medical societies: Texas Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Renée R Snyder, MD Dermatologist, Private Practice

Renée R Snyder, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, Texas Medical Association, Texas Dermatological Society

Disclosure: Nothing to disclose.

Dayna Diven, MD Professor, Department of Dermatology, University of Texas Southwestern Austin Programs

Dayna Diven, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Idaho Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Abby S Van Voorhees, MD Assistant Professor, Director of Psoriasis Services and Phototherapy Units, Department of Dermatology, University of Pennsylvania School of Medicine, Hospital of the University of Pennsylvania

Abby S Van Voorhees, MD is a member of the following medical societies: American Academy of Dermatology, Women's Dermatologic Society, National Psoriasis Foundation, American Medical Association, Phi Beta Kappa, Sigma Xi, The Scientific Research Honor Society

Disclosure: Received honoraria from Amgen for consulting; Received honoraria from Abbott for consulting; Partner received salary from Merck for management position; Received honoraria from Abbott for speaking and teaching; Received honoraria from Amgen for review panel membership; Received honoraria from Centocor for consulting; Received honoraria from Leo for consulting; Received none from Merck for other.

Acknowledgements

Mary Farley, MD Dermatologic Surgeon/Mohs Surgeon, Anne Arundel Surgery Center

Disclosure: Nothing to disclose.