occurs when there is interruption of sympathetic innervation to the sweat glands, resulting in a lack of sweat production.
She was diagnosed as having CIPA because of recurrent episodes of unexplained fever, anhidrosis
, burns, and bone fractures after birth.
The etiology of PEODDN is currently unknown, however, studies have alluded to a genetic contribution through genetic mosaicism and a possible eccrine or circumscribed epidermal keratinization abnormality.9,10 PEODDN is also associated with many conditions including scoliosis, anhidrosis
, seizure disorder, sensory polyneuropathy, deafness, left hemiparesis, developmental delay, breast hypoplasia, alopecia, onychodysplasia, Bowen's disease, and squamous cell carcinoma.11,12 Our patient was clinically healthy, without any of the aforementioned associated disorders.
In cases of Horner's syndrome, there may be partial ptosis, miosis, anhidrosis
, with or without enophthalmos.
The medical condition, anhidrosis
, is the inability to do what?
The patient subsequently developed clinical features of Horner syndrome, such as ptosis, miosis, and anhidrosis
of the face.
Differential diagnosis includes Werner syndrome, focal dermal hypoplasia, alopecia areata, aplasia cutis congenita, incontinentia pigmenti, dyskeratosis congenital, familial simple anhidrosis
, Naegeli-Franceschetti-Jadassohn syndrome, and pachyonychia congenita.
Unusual changes in sweating - either excessive perspiration (hyperhidrosis) or little or no perspiration (anhidrosis
) - can be cause for concern.
Patients usually have pathological fractures and sacral anhidrosis
. Deep tendon reflexes are not present.
For instance, Horner's syndrome is a condition in which damage affects the function of the upper segments (cervicothoracic) of the paravertebral sympathetic chain, leading to dilated pupil on the ipsilateral side in addition to ipsilateral eyelid ptosis and hemifacial anhidrosis
On neurological examination the patient was noticed to have left-sided ptosis, miosis, enophthalmos (Figure 1), and reported facial anhidrosis
on the left side, alongside ipsilateral weakness predominantly involving his intrinsic hand muscles, long finger flexors, and numbness involving the medial border of his arm, hand, and little finger (see Video 1 in Supplementary Material available online at http://dx.doi.org/10.1155/2016/3015947).
Iris heterochromia, dilation lag, anhidrosis
, and harlequin sign may concurrently be present [2, 3].
Horner syndrome, presenting as a combination of ptosis, miosis, and anhidrosis
of the face, could be caused by thermal injury to the middle cervical sympathetic ganglion (mCSG) .
Absent innervation of skin and sweat glands in congenital insensitivity to pain with anhidrosis
. Clin Neurophysiol 2000;111(9):1596-1601.