Naegeli syndrome

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Naegeli syndrome
Classification and external resources
OMIM 161000
DiseasesDB 29767
eMedicine derm/736 

Naegeli syndrome (Naegeli-Franceschetti-Jadassohn syndrome) is a rare autosomal dominant form of ectodermal dysplasia, characterized by reticular skin pigmentation, diminished function of the sweat glands, the absence of teeth and hyperkeratosis of the palms and soles. One of the most striking features is the absence of fingerprint lines on the fingers.

Naegeli syndrome is comparable to Dermatopathia pigmentosa reticularis, both of which is caused by a specific defect in the keratin 14 protein.

It was named after Oskar Naegeli.

References

  • Schnur R, Heymann W (1997). "Reticulate hyperpigmentation.". Semin Cutan Med Surg 16 (1): 72-80. PMID 9125768.

External links

synd/1417 at Who Named It


Acknowledgement and Attribution Regarding Sources of Content

Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .

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