Familial anhidrosis L74.84

Last updated on: 29.10.2020

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Synonym(s)

Anhidrosis Syndrome; Familial anhidrosis; general acquired sudomotor denervation; progressive selective anhidrosis; Ross's syndrome; Ross Syndrome

History
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Ross, 1958

Definition
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Disease with a typical symptom triad of tonic pupillary disturbances, loss of autonomic and extraneous reflexes, and bi- or unilateral progressive segmental anhidrosis, which reflexively leads to hyperhidrosis in unaffected segments and to heat intolerance.

Etiopathogenesis
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Unknown, a polytopic disseminated neuropathy in the autonomic nervous system is assumed. Association with autonomic dysfunction is possible.

Laboratory
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Sweat secretion tests (thermoregulatory, cholinergic, emotional, gustatory irritation and during physical exertion) show a lack of reactive hydrosis.

Histology
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Unchanged number and morphology of sweat glands.

Differential diagnosis
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Ectodermal dysplasia with hypoanidrosis; Pancoast tumor; hypoanidrosis after sympathectomy, in diabetes mellitus, anemia, intoxications, brain or kidney diseases. Differentiation from Adie syndrome is difficult.

Therapy
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By ophthalmologists and neurologists. Avoid extreme temperatures and exsiccative measures such as long and hot showers, hot baths, etc. Bland care with moisturising external agents such as Ungt. emulsif. aq., if necessary with urea additive.

Hyperhidrotic body areas can often be successfully treated with tap water iontophoresis (direct current, 15 Volt, 25 mA) (for exact procedure see there). The commercially available plastic tubs (with electrode connection) are usually designed for hands and feet and must be handmade to fit the affected body area if necessary.

Alternatively, intradermal botulinum injections can be used, which are distributed fan-shaped under hyperhidrotic areas.

Literature
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  1. Chakravarty A et al (2003) Ross syndrome--a case documentation. Acta Neurol Scand 107: 72-73
  2. Itin P et al (1992) The Ross syndrome. Dermatologist 43: 359-360
  3. Reinauer S et al. (1992) Ross syndrome: redefinition and therapy of associated hyperhidrosis by a modified iontophoresis technique. Z Hautkr 67: 615-621
  4. Ross AT (1958) Progressive selective sudomotor denervation. A case with coexisting Adie's syndrome. Neurology 8: 809-817
  5. Weller M, Wilhelm H, Sommer N et al (1992) Tonic pupil, areflexia, and segmental anhidrosis: two additional cases of Ross syndrome and review of the literature. J Neurol 239: 231-234
  6. Yasar S et a. (2010) Ross syndrome: Unilateral hyperhidrosis, adie-pupils and diffuse areflexia. JDDG 12: 1004-1006

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020