[Perforating milia-like idiopathic calcinosis of the extremities in a patient with Down syndrome].

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.