Naegeli-Franceschetti-Jadassohn Syndrome Treatment & Management

  • Author: Rebekah H Clifford, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Apr 29, 2010
 

Medical Care

No treatment is effective for Naegeli-Franceschetti-Jadassohn (NFJ) syndrome. As with other ectodermal dysplasias, exposure to heat should be limited and sufficient hydration is recommended. Tooth care to prevent early caries is indicated. Doxycycline has been found to interfere with tumor necrosis factor-alpha (TNF-alpha)–mediated signaling and apoptosis in vitro and may have a role in future treatment.[1]

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Activity

Sports-related exercise should be restricted to only nonexhausting activities. In particular, patients with Naegeli-Franceschetti-Jadassohn (NFJ) syndrome can be allowed to swim.

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Contributor Information and Disclosures
Author

Rebekah H Clifford, MD  Resident Physician, Department of Dermatology, Walter Reed Army Medical Center

Rebekah H Clifford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Medical Women's Association, and Women's Dermatologic Society

Disclosure: Nothing to disclose.

Coauthor(s)

Kenneth J Galeckas, MD  Assistant Professor, Department of Dermatology, Uniformed Services University of the Health Sciences; Staff Dermatologist, Director, Laser and Cosmetic Clinic, Intern and Medical Student Coordinator, Department of Dermatology, National Naval Medical Center

Kenneth J Galeckas, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society for Dermatologic Surgery, and Association of Military Dermatologists

Disclosure: Nothing to disclose.

Specialty Editor Board

James Fulton Jr, MD, PhD  Center for Cosmetic Dermatology; Consultant, Vivant Pharmaceuticals, LLC

James Fulton Jr, MD, PhD is a member of the following medical societies: American Academy of Cosmetic Surgery, American Academy of Dermatology, American Society for Laser Medicine and Surgery, Dermatology Foundation, International Society of Cosmetic and Laser Surgeons, and Skin Cancer Foundation

Disclosure: vivant pharmaceuticals Ownership interest Consulting

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.

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

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, Department of Dermatology, Geisinger Medical Center

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

Disclosure: Nothing to disclose.

References

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  22. Dereure O. [Naegeli-Franceschetti-Jadassohn syndrome and dermatopathia pigmentosa reticularis. Two allelic ectodermal dysplasias related to mutations of dominant gene coding for keratin 14]. Ann Dermatol Venereol. Jun-Jul 2007;134(6-7):595. [Medline].

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Lack of dermatoglyphics in a patient with Naegeli-Franceschetti-Jadassohn syndrome.
Reticulated pigmentation on the trunk in a patient with Naegeli-Franceschetti-Jadassohn syndrome.
Periorbital reticulated pigmentation in a patient with Naegeli-Franceschetti-Jadassohn syndrome.
Spotty palmoplantar hyperkeratosis in Naegeli-Franceschetti-Jadassohn syndrome.
Defective denture with yellow spots on the enamel in a patient with Naegeli-Franceschetti-Jadassohn syndrome.
 
 
 
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