Ectodermal dysplasia, absent dermatoglyphic pattern,
changes in nails, simian crease, and abnormal sweating.
Unknown. First described by M. Basan in 1965; since then
a limited number of articles describing various patients with features of
the syndrome have been published. However, clear distinction between
epidermal ridge disorders remains difficult because of the heterogeneous
nature of these conditions and the lack of an accepted classification
Autosomal dominant inheritance. Genetic
heterogeneity results in a spectrum of familial absence/dissociation of
dermal ridge patterns.
The precise mechanisms leading to the wide
phenotypic variations of the syndrome remain to be determined.
Based on clinical examination, family history, and
The syndrome is characterized by multiple
congenital milia on the chin and neonatal vesicular/bullous lesions on the
fingers and soles of the feet, which resolve during the first month of life.
Ectodermal dysplasia with absence or abnormality of dermal ridge patterns,
simian creases, and lack of sweat glands on the volar surfaces. The fingertips are tapered
and there is an increased cold sensitivity on hands and feet. The nails are
rough, with transverse groves and longitudinal ridges. The skin is smooth with a
leather-like texture. No other congenital abnormalities are associated with
the condition. Life expectancy appears to be normal.
Premedication with anticholinergic
agents (e.g., atropine) should be avoided in neonates and infants because
the condition is associated with a reduced number of sweat glands,
potentially resulting in pyrexia and abnormal body temperature regulation.
Temperature monitoring should be used in
all cases. Vascular access in the extremities may be difficult as a
consequence of the leather-like skin texture.
Careful use of anticholinergic drugs
Ectodermal Dysplasia: At least 150 other forms of Ectodermal Dysplasia are known.
Reed T, Schreiner RL: Absence of dermal ridge patterns: genetic
heterogeneity. Am J Med Genet
Richards W, Kaplan M: Anhidrotic ectodermal dysplasia. An unusual case of
pyrexia in the newborn. Am J Dis Child
Schaumann B, Alter M: Dermatoglyphics in Medical Disorders. New York, Springer-Verlag,
1976, p 89.