Ross' syndrome

The Ross syndrome, also familial anhidrosis or anhidrosis Syndrome is a neurological- dermatological disorder defined by the simultaneous occurrence of reduced or aufgehobender sweating ( hypo-or anhidrosis ), tonic pupil contraction ( tonic pupil ) and attenuated or extinguished tendon reflexes ( hyporeflexia or areflexia ). The Ross syndrome is a rare disease. By 1992 only 18 medical case reports have been published. In the Anglo- American world and the General -acquired synonyms are sudomotor denervation or selective progressive anhidrosis used.

The first description of the syndrome was carried out by AT Ross in 1958.

Disease

The Ross syndrome is characterized by a unilateral, usually on the trunk occurring and progressive, diminished sweating (progressive segmental hypohidrosis ) connected to a Adie syndrome, ie, a tonic pupil contraction ( tonic pupil ) and attenuated tendon reflexes ( hyporeflexia ), characterized. The Schweißsekretionsstörung result in impaired heat tolerance. The hypohidrosis on one side of the body is compensated on the opposite side by an increased sweating ( hyperhidrosis). The hyperhidrosis is usually felt by patients as more disturbing than the existing on the opposite side hypohidrosis.

In addition to the Schweißsekretionsstörung it can lead to further disturbances of the autonomic nervous system are such as orthosthatische hypotension and vasovagal syncope. Also shortness of breath, headache, reflux esophagitis, irritable colon and psychiatric changes associated with the disease.

Pathogenesis

The cause of the disease is not precisely known. Hypohidrosis and tonic pupil due to a disturbance of cholinergic postganglionic parasympathetic and sympathetic nerve fibers. The attenuated tendon reflexes can not be explained. Number and morphology of sweat glands are unchanged.

Diagnostics

The Schweißsekretionsstörung can be detected by various Schweißsekretiontests.

Therapy

Because of the heat intolerance, the avoidance of extreme temperatures is recommended. Hyperhidrotic areas can be treated with tap water using iontophoresis, intradermal botulinum toxin injections with or with aluminum chloride solution.