Hereditary sensory neuropathy - type - II
S Chidamabara Murthy
Department of Skin and Sexually Tarnsmitted Diseases, S. N. Medical College & H. S. K. Hospital & Research centre, Bagalkot
|How to cite this article:
Murthy S C, Udagani M M, Patil M N. Hereditary sensory neuropathy - type - II. Indian J Dermatol Venereol Leprol 2002;68:375-376
To The Editor,
A 4-year-old boy, born to consanguinous parents, presented to us with painless ulcers and deep fissures over the sole of the feet for the last three months. It used to begin as a callosity and later ulcerate. Patient was comfortable, despite the presence of ulcers. He was having recurrent painless ulcers over the hands due to mechanical trauma and burns. Patient was delivered at full term. His developmental mile - stones were normal. There was no maternal illness during pregnancy. He was the only son and none of his family members suffered from similar illness.
On examination, peripheral pluses were normal. Lacrimation was normal. A solitary trophic ulcer over sole of right foot and deep fissures over both the soles were present. Multiple healing ulcers and scars were present over the distal upper and lower extremities. There was impairment of sensation for touch, pain and temperature predominantly over the distal upper and lower extremities. Nervous system examination revealed diminished deep tendon reflexes. Spine was normal. There were no hypopigmented patches, nerve thickening, sweating abnormalities, speech and learning difficulties, bowel and bladder incontinence. Other systemic examinations were normal.
Routine investigations and blood sugar were within normal limits. VDRL was non reactive, HIV - 1 and 2 were negative by ELISA. Special investigations like nerve biopsy, nerve conduction studies and electromyogram could not be done due to patient′s refusal and want of facilities. Based on the above findings, a clinical diagnosis of hereditary sensory neuropathy - type II was made. Patient was advised care of hands and feet.
Hereditary sensory neuropathies are divided into five types. They are differentiated based on clinical signs and symptoms, the type of inheritance, the type of neurons affected, distal and cutaneous nerve action potential patterns.
Our patient had an early onset, recessive inheritance, involvement of both upper and lower limbs, suggestive of Hereditary Sensory Neuropathy - type II, without abnormalities found in other types. This case is being reported for its rarity. Early and prompt recognition with care of hands and feet can prevent mutilating acropathy.
Sharma NL, Sharma VK, Sood A. Congenital sensory neuropathy with anhidrosis. Indian J Dermatol Venereol Leprol 1992; 58:257-259.[Google Scholar]
Pavithran K, Nair PR, Mathew PS, et al. Hereditary sensory neuropathy (Type II) ( Congenital absence of pain ). Indian J Dermatol Venereal Leprol 1984;50:217 - 219.[Google Scholar]