Diffuse cutaneous neurofibromatosis of the hand

Case contributed by Dr Alexandra Stanislavsky

Presentation

Hand pain in a patient with a known neurocutaneous disorder.

Patient Data

Age: 45 years
Gender: Female

There is diffuse, enhancing thickening of the skin and subcutaneous soft tissues, over the entire dorsum of the hand extending into the dorsal aspect index finger and thumb base.  There is also a somewhat more focal enhancing subcutaneous palmar lesion centered just distal to the thenar eminence (distal metacarpal and MCP level), and extending as thickened folds into the first webspace. It contacts the third and fourth flexor digitorum tendons, and partially encases the second flexor digitorum tendons.

There is a low signal, non enhancing septation through the central part of the lesion, possibly a previous surgical scar.

No mass along the median or ulnar nerves.  

Case Discussion

Extensive subcutaneous soft tissue mass, in keeping with diffuse cutaneous neurofibromatosis in a patient with known neurofibromatosis type 1.

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