Congenital sensory neuropathy with anhidrosis (hereditary sensory neuropathy type IV)
| Autor: | D. Koumelas, A. Varhlzidis, John Stratigos, G. Makaronis, K. Gourgiotou, John Hatzis |
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| Rok vydání: | 1992 |
| Předmět: |
Male
medicine.medical_specialty Hereditary Sensory Neuropathies Pain Insensitivity Congenital Dermatology Thermal stimulation Tongue Medicine Humans Anhidrosis Hereditary Sensory and Autonomic Neuropathies Hypohidrosis business.industry medicine.disease Pain Insensitivity Congenital/diagnosis Hypohidrosis/complications/*diagnosis Surgery Peripheral neuropathy medicine.anatomical_structure El Niño Child Preschool Sensory neuropathy Hereditary Sensory and Autonomic Neuropathies/complications/*diagnosis medicine.symptom business Sensory nerve Follow-Up Studies |
| Zdroj: | The Australasian journal of dermatology. 33(2) |
| ISSN: | 0004-8380 |
| Popis: | Hereditary sensory neuropathies comprise a group of rare childhood diseases which are classified into four types. We present a Greek boy 11 years old with hereditary sensory neuropathy type IV (congenital sensory neuropathy with anhidrosis) whom we have followed up and studied during the last seven years. Our patient presented for the first time with recurrent hyperthermic episodes without sweating, and lack of pain sensation from the first months of life. Insensitivity to pain and thermal stimuli had resulted in burns on the extremities and self-mutilation of the tongue, lips and fingertips. When he was five and seven years old respectively he had two painless fractures of the ankles which led to insoluble orthopedic problems. He also suffered from mental retardation, which was obvious from his first years of life. Sweat gland investigations showed significant hypohidrosis or anhidrosis although the sweat glands were normal microscopically. Hereditary sensory neuropathy type IV, although rare, is important for dermatologists because it must be differentiated from other anhidrotic syndromes, and in view of the poor prognosis of the condition. Australas J Dermatol |
| Databáze: | OpenAIRE |
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