Plexiform Neurofibromatosis with Abnormal Pachydermatocele: A Rare Case Report
AK Agarwal, Dileep Kumar, Anil Kumar Gupta, Ganesh Yadav
Citation Information :
Agarwal A, Kumar D, Gupta AK, Yadav G. Plexiform Neurofibromatosis with Abnormal Pachydermatocele: A Rare Case Report. Indian J Phy Med Rehab 2013; 24 (4):110-113.
Plexiform neurofibromatosis, also known as pachydermatocele, is a rare type of neurofibromatosis, caused by excessive growth of the neural tissue in the subcutaneous fat. This condition is rare in Indian subcontinent but when it occurs, it causes cosmetic as well as functional disability. The optimal management of plexiform neurofibroma is not well defined and surgery is often delayed. We report a rare case of neurofibromatosis with pachydermatocele involving the nerves of the left thigh and leg with disfigurement and associated disability.
Von Recklinghausen neurofibromatosis: a clinical and population study in south-east Wales. Brain 1988;111:1355-81.
Tumours and Cysts of Skin and subcutaneous tissue. In: Principles and Practice of Modern Surgery. 5th ed. Calcutta: PS Publishers, 1985:70-81.
Case report 828: Plexiform neurofibroma of the tibial nerve invading the medial and lateral gastrocnemius muscles and plantaris muscle. Skeletal Radiol 1994;23:149-52.
Conference statement. National Institutes of Health Consensus Development Conference. Arch Neurol 1988; 45: 575-8.
Plexiform neurofibromas. Am J Med Genet 1999; 89: 31-7.