Ross syndrome

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Classification according to ICD-10
L74.8 Other diseases of the eccrine sweat glands
ICD-10 online (WHO version 2019)

The Ross syndrome , also familiar anhidrosis or anhidrosis syndrome is a neurological dermatological disease defined by the simultaneous occurrence of reduced or aufgehobender sweating (hypo- or anhidrosis), tonic pupil contraction ( tonic pupil ) and attenuated or extinct tendon reflexes ( hyporeflexia or areflexia ). Ross syndrome is a rare condition. As of 1992, only 18 medical case reports had been published. In the Anglo-American language area, the synonyms General acquired sudomotor denervation or Progressive selective anhidrosis are also used.

The syndrome was first described by AT Ross in 1958.

Clinical picture

Ross syndrome is characterized by a unilateral, mostly on the trunk occurring and progressive, reduced sweat secretion (progressive segmental hypohidrosis) combined with Adie syndrome , i.e. a tonic pupillary contraction ( pupillotonia ) and weakened muscle reflexes ( hyporeflexia ). The sweat secretion disorder leads to a restricted heat tolerance. The hypohidrosis on one side of the body is compensated for on the opposite side by increased sweat secretion (hyperhidrosis). The hyperhidrosis is usually perceived by patients as more annoying than the hypohidrosis existing on the opposite side.

In addition to the sweat secretion disorder, other disorders of the autonomic nervous system such as orthostatic hypotension and vasovagal syncope can occur . Also, shortness of breath , headaches , gastroesophageal reflux disease , irritable bowel syndrome and psychiatric changes are associated with the disease in conjunction.

Disease emergence

The exact cause of the disease is not known. Hypohidrosis and pupillotension are due to a disruption of the cholinergic postganglionic parasympathetic and sympathetic nerve fibers. The weakened muscle reflexes cannot be explained. The number and morphology of the sweat glands are unchanged.

Diagnosis

The sweat secretion disorder can be detected by various sweat secretion tests.

therapy

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

Individual evidence

  1. ↑ a b M. Weller et al .: Tonic pupil, areflexia, and segmental anhidrosis: two additional cases of Ross syndrome and review of the literature. In: J Neurol. 1992 Apr; 239 (4), pp. 231-234. PMID 1597691
  2. ↑ a b P. Altmeyer: Therapielexikon Dermatology and Allergology: Therapy compact from AZ. 2nd Edition. Springer-Verlag, 2005, ISBN 3-540-23781-X , p. 65. Restricted preview in Google Book Search .
  3. ^ A b H. Wilhelm et al.: Ross syndrome: Adie syndrome with disorders of sweat and temperature regulation . In: Klin Monatsbl Augenheilkd. 1991; 199 (12), pp. 442-443. doi: 10.1055 / s-2008-1046111 , PMID 1791687 .
  4. ^ AT Ross: Progressive selective sudomotor denervation; a case with coexisting Adie's syndrome . In: Neurology. 1958 Nov; 8 (11), pp. 809-817. PMID 13590391
  5. ↑ Reinhard K. Achenbach: Hyperhidrosis: Physiological and pathological sweating in diagnosis and therapy. 1st edition. Steinkopff-Verlag, 2004, ISBN 3-7985-1475-5 , p. 53.
  6. ^ A b Otto Braun-Falco, Gerd Plewig, Helmut H. Wolff: Dermatology and Venereology . Springer-Verlag, 2005, ISBN 3-540-40525-9 , p. 915.
  7. ^ A b c Peter Altmeyer, Martina Bacharach-Buhles: Springer Encyclopedia Dermatology, Allergology, Environmental Medicine. 1st edition. Springer-Verlag, 2002, ISBN 3-540-41361-8 , p. 111.

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