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Pachyonychia Congenita Medication

  • Author: Saira J George, MD; Chief Editor: William D James, MD  more...
Updated: Mar 30, 2016

Medication Summary

Medications are used to reduce the symptoms associated with pachyonychia congenita. Cure is not yet possible.



Class Summary

Retinoids are a family of drugs related to vitamin A. They regulate the differentiation and proliferation of epithelial cells. Some also possess antitumoral activity.

Acitretin (Neotigason, Soriatane)


Retinoic acid analogues such as acitretin and isotretinoin are relatively widely used in dermatology. Etretinate is the main metabolite. The detailed mechanisms of action are still being studied.



Class Summary

These agents cause cornified epithelium to swell, soften, macerate, and then desquamate.

Salicylic acid topical


By dissolving the intercellular cement substance, salicylic acid produces desquamation of the horny layer of skin, while not affecting structure of viable epidermis.

Hydrate skin and enhance the effects of the medication by soaking the affected area in warm water for 5 minutes prior to use. Remove any loose tissue with a brush, washcloth, or emery board and dry thoroughly. Improvement should generally occur in 1-2 weeks.

Urea (Ureacin-40)


Urea promotes hydration and removal of excess keratin in conditions of hyperkeratosis.

Salicylic acid (20%), urea (40%), and hydrophilic ointment compound


This is compounded in the pharmacy. It promotes hydration and removal of excess keratin in conditions of hyperkeratosis.

Contributor Information and Disclosures

Saira J George, MD Assistant Professor of Dermatology, The University of Texas MD Anderson Cancer Center

Saira J George, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Lester F Libow, MD Dermatopathologist, South Texas Dermatopathology Laboratory

Lester F Libow, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Texas Medical Association

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Gregory J Raugi, MD, PhD Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at Seattle School of Medicine; Chief, Dermatology Section, Primary and Specialty Care Service, Veterans Administration Medical Center of Seattle

Gregory J Raugi, MD, PhD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.


The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Aleksej Kansky, MD, PhD, to the development and writing of this article.

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The most prominent feature is a substantially thickened, brownish gray nail plate with a rough surface.
Leukokeratosis of the oral mucosa is a prominent sign. Patchy whitish areas may be seen on the back of the tongue; the buccal mucosa; and sometimes, the gingiva.
Hyperkeratotic lesions of the skin may involve acanthosis, hyperkeratosis, and parakeratosis.
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