[Pachydermodactyly: seven new cases].

INTRODUCTION

Pachydermodactyly is a superficial benign digital fibromatosis usually involving the proximal portions of the fingers. It is clinically characterized by an asymptomatic, bulbous, soft-tissue swelling around proximal phalanges and interphalangeal joints. We report here seven new cases of pachydermodactyly.

CASE REPORTS

Seven patients (4 F, 3 M) ranging in age from 14 to 63 years were studied. Two of them were affected by tuberous sclerosis; two other patients were sisters, one of whom was affected by the transgrediens form of pachydermodactyly. The personal history of two male patients revealed the compulsive habit of interlacing the fingers. Finally a 23-year-old patient was affected by the localized pachydermodactyly. In all the patients roentgenogram and echography of the affected fingers as well as histological and ultrastructural of a cutaneous biopsy examination were carried out. The results of the tests confirmed the diagnosis of pachydermodactyly.

DISCUSSION

Our data suggest that pachydermodactyly is underestimated rather than rare and more frequent in females than in males as until now reported in the literature. We suggest classifying pachydermodactyly into five types: classic pachydermodactyly frequently associated with mechanical trauma, monopachydermodactyly or localized pachydermodactyly, transgrediens pachydermodactyly in which the cutaneous thickness extends to the metacarpophalangeal areas, familial pachydermodactyly which may by transgrediens and pachydermodactyly associated with tuberous sclerosis.