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Naegeli Syndrome (Melanophoric Nevus Syndrome; Franceschetti-Jadassohn Syndrome; Naegeli Incontinentia Pigmenti; Reticular Pigmented Dermatosis) 333f52d 333f52d 333f52d
General: Autosomal dominant; separate entity from incontinentia pigmenti (Bloch-Sulzberger syndrome) based on mode of inheritance; blisters and inflammatory lesions of skin as seen in incontinentia pigmenti not present in Naegeli syndrome; skin pigmentation appears at age 2 years.
Ocular: Nystagmus; strabismus; pseudoglioma; papillitis; optic atrophy.
Clinical: Reticular pigmentary skin changes; hypohidrosis; defective teeth with yellow spotting; keratoderma of palms and soles; disturbed regulation of temperature by reduction of number of sweat glands.
Geeraets WJ. Ocular Syndromes, 3rd ed. Philadelphia: Lea & Febiger, 1976.
Greither A, Haensch R. Anhidrotic reticular pigment dermatosis with a pustular erythematous initial phase. Transitional cases, failing between Bloch-Sulzberger incontinentia pigmenti and the reticular pigment dermatosis of Naegeli-Franceschetti-Jadassohn. Schweiz Med Wochenschr 1970; 100:228.
Naegeli O. Familiarer Chromatophorennaevus. Schweiz Med Wochenschr 1927; 8:48.
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