[Cutaneous manifestations in the dysproteinemias. A case of atypical macroglobulinemia of Waldenstrom (author's transl)]. / Les manifestations cutanées des dysglobulinémies.

Cutaneous involvement in the dysproteinemias are of unusual occurrence. The authors report a case of Waldenstrom disease evolving to a biclonal dysglobulinemia with cutaneous involvement. The different possibilities of association between cutaneous manifestations and dysproteinemias are reviewed.

[Dyskeratosis congenita Zinsser-Cole-Engman form. Report of two affected brothers (author's transl)]. / Dyskératose congénitale de Zinsser-Cole-Engman Chez deus frères.

Cutaneous pigmentation, lingual leukoplasia and dystrophic changes of nails are present in the two cases. The other clinical manifestations are dental alterations, epiphora, loss of dermal ridges of the pulp with hyperhidrosis, atrophic skin of the dorsum of the hands. Dysphagia and bone marrow hypoplasia are present in one case. The proband (case 1) has normal values for the following: hemoglobin electrophoresis, pyruvate kinase, marrow and blood chromosome analysis. Biopsy of pigmented skin showed an atrophic epidermis with orthokeratotic-hyperkeratosis; in the higher dermis there were several melanophores. Multiple layers of vasal lamina are seen under electron microscopy. The parents and the two daughters are free of clinical or hematologic manifestations. The mother and her two affected sons have A1-BW 27 HLA haplotype. X-linked transmission is discussed.