Generalised anhidrosis: different lesion sites demonstrated by microneurography and skin biopsy
- V Donadio1,
- P Montagna1,
- M Nolano2,
- P Cortelli3,
- C Misciali4,
- G Pierangeli1,
- V Provitera2,
- A Casano1,
- A Baruzzi1,
- R Liguori1
- 1Department of Neurological Sciences, University of Bologna, Via U. Foscolo 7, 40123 Bologna, Italy
- 2Department of Neurology, Salvatore Maugeri Foundation, IRCCS, Via Bagni Vecchi, 82037 Telese Terme (BN), Italy
- 3Department of Neurology, University of Modena and Reggio Emilia, Via Dal Pozzo, 41100 Modena, Italy
- 4Department of Dermatology, S.Orsola Hospital, Via Massarenti 9, 40135 Bologna, Italy
- Correspondence to: Dr V Donadio Dipartimento di Scienze Neurologiche dell’Università di Bologna, Via Ugo Foscolo 7, 40123 Bologna, Italy; donadioneuro.unibo.it
- Received 13 February 2004
- Accepted 4 August 2004
- Revised 12 July 2004
Abstract
Generalised anhidrosis (GA) shows a uniform clinical picture whether the pathogenesis involves intrinsic abnormalities of sweat glands or postganglionic sympathetic cholinergic nerve dysfunction. We describe two patients who presented intolerance to heat and anhidrosis. In the first patient, symptoms started at 33 years of age, and were associated with absent tendon reflexes and a mydriatic right pupil unreactive to light. The other patient had been unable to sweat since birth. GA was diagnosed on the basis of clinical findings and thermoregulatory tests. Microneurography and morphological analysis of the skin and its innervation disclosed a different lesion site underlying GA in the two patients, and distinguished between a postganglionic autonomic nerve fibre lesion and sweat gland dysfunction.
- AIGA, acquired idiopathic generalised anhidrosis
- CED, congenital ectodermal dysplasia
- CRT, circadian rhythm of body core temperature
- ENF, epidermal nerve fibre
- GA, generalised anhidrosis
- IPSF, idiopathic pure sudomotor failure
- MSNA, muscle sympathetic nerve activity
- RS, Ross syndrome
- SGF, sweat gland failure
- SSNA, skin sympathetic nerve activity
- TST, thermoregulatory test
- VIP, vasoactive intestinal peptide
- generalised anhidrosis
- skin biopsy
- microneurography
- postganglionic sudomotor lesions
- sweat gland abnormalities
Footnotes
-
Competing interests: none declared
