Abstract

CASE REPORT OF VON RECKLINGHAUSENS�?¢�?�?��?�?� DISEASE

Author(s):Archana S , Shefali Singhal, Muruganandam TV, Hamedullah, Thilak S, Meera G, Shilpa Reddy, Chandramani

Neurofibromatosis type -1 is a familial tumour syndrome belonging to genodermatosis group of disorders. It is genetically transmitted by autosomal doiminance with variable penetrance. The disorder is characterized by benign growths of the peripheral nerve sheaths,neurofibromas and café au lait macules. A45 year old male came with complaint of multiple asymptomatic swelling all over his body since birth. They were continuously progressing in size,one of which on the back, weighed 2.5 kgs , causing dragging pain. No constitutional on systemic symptoms were present. Patient was completely evaluated. Neuofibroma was confirmed with a biopsy. The results being normal. The plexiform lesion was excised. The patient is on regular follow up.

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