Dar N R, Fatema A, Awan Z.
Maffucci`s Syndrome - first case report from Pakistan.
J Pak Med Assoc Jan ;53(10):494-5.

A thirty years old female presented in outpatient department with multiple swellings on upper and lower extremities. These swellings appeared fifteen years back, when she was fifteen years of age. The swellings were soft, asymptomatic and developed from the fingers of left hand followed by appearance of painful bony hard swellings on the wrist. Both soft and hard swellings progressed with age along with appearance of similar swellings on left elbow, left ankle and feet and toes of the right foot. There were no associated systemic complaints. The patient was single with no family history of similar disease. Examination revealed multiple hard and soft nodules on left elbow, left ankle and foot and the toes of the right foot. They were ten to fifteen in number, of variable sizes, ovoid in shape and smooth surfaced. Some of the nodules were hard and whitish in colour and non fluctuant and tender on palpation, while other nodules were soft, bluish, fluctuant and non tender. Both type of nodules were asymmetrical in distribution and not fixed to overlying skin. Systemic examination was unremarkable. Above wrist amputation of the left hand was performed. On basis of clinical features and histopathological appearance, a diagnosis of Maffuci`s syndrome was made.

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