Congenital alopecia areata: another case.
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Biomedical subjects
Publications and source records attributed to B Raone.
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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.
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INTRODUCTION: In 1989 a new type of calcinosis cutis has been described in association with Down's syndrome. This is the milia-like idiopathic calcinosis cutis, which is characterized by milia-like papulae generally located on the limbs (especially hands and feet) and sometimes associated with syringomas around lesions or on the eyelids. OBSERVATION: A 6 year old trisomic girl had about ten round shaped hard white-yellowish papules with a diameter of 2-3 mm on both palms of her hands. The biological balance and immunologic tests gave normal values. The histopathologic pattern was compatible with calcinosis cutis circumscripta associated with the transepidermal elimination phenomenon. Calcified sweat ducts were not observed at the von Kossa staining. Moreover, histology did not evidence any syringomas around the lesions. DISCUSSION: Our observation does not sustain the presently more spread pathogenetic interpretation, according to which eccrine ductal structures could have an active role in the formation of calcium deposits, since histology did not show any calcified eccrine ducts. Therefore, in our opinion, milia-like calcinosis associated with Down's syndrome should be classified among the idiopathic forms.
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