Oral and maxillofacial complications associated with congenital sensory neuropathy with anhydrosis: Report of two cases

Pedigree data suggest that congenital peripheral sensory neuropathies are a group of disorders having an autosomal recessive mode of inheritance. They are manifested by an absence of pain sensation and are separated by clinical, laboratory, and neuropathological studies.’ Congenital sensory neuropathy with anhydrosis, first described by Swanson et al.,* has been referred to as type II familial dysautonomia and hereditary sensory neuropathy, type IV. To date, thirteen cases of this rare disorder have been reported. The disorder is characterized clinically by recurrent unexplained fevers, failure to thrive, absence of perspiration despite the presence of normal sweat glands, insensitivity to pain, self-mutilation, mild mental retardation and aplasia of the dental ename1.3*4 The autonomic dysfunction is apparently confined to the parasympathetic system since esophageal motility and serum catecholamine levels are within normal limits and neither erythematous skin blotching nor orthostatic hypotension occur. Histologic examination of the sural nerve shows a severe lack of nonmyelinated axons.