Pachyonychia Congenita Medication

  • Author: Saira J George, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Aug 9, 2010
 

Medication Summary

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

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Retinoids

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.

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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.

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Keratolytics

Class Summary

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

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

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Urea (Ureacin-40)

 

Promotes hydration and removal of excess keratin in conditions of hyperkeratosis.

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

 

Compounded in the pharmacy. Promotes hydration and removal of excess keratin in conditions of hyperkeratosis.

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Contributor Information and Disclosures
Author

Saira J George, MD  Staff Dermatologist, Dermatological Association of Texas

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

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

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

Disclosure: Nothing to disclose.

Glen H Crawford, MD  Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital

Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

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

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous Chief Editor, William D. James, MD, and previous author, Aleksej Kansky, MD, PhD, to the development and writing of this article.

References

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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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