[Simultaneous occurrence of metageria and Gottron's acrogeria in one family]

We report on a 17-year-old patient suffering from generalized mottled hyperpigmentation and multiple telangiectatic lesions of the skin. The subcutaneous fatty tissue was lacking or deficient. The patient showed a bird-like face. The cutaneous changes had been present since birth. X-ray studies revealed acro-osteolysis of the hands and feet. The metabolic and endocrine data were within normal ranges except of latent hypothyroidism. The clinical picture presented all criteria of metageria. The patient's mother and sister showed similar cutaneous changes, however, restricted to the distal parts of the extremities. We diagnosed acrogeria Gottron. The incidence of both metageria and acrogeria in one family makes metageria as an independent entity doubtful.