CLINICAL CASES   

Giornale Italiano di Dermatologia e Venereologia 2005 February;140(1):69-74

Copyright © 2005 EDIZIONI MINERVA MEDICA

language: English, Italian

Familial livedo and pulmonary fibrosis (familial livedoid pulmonary fibrosis): a new entity?

Scagliusi P. ¹, Bonamonte D. ², Scagliusi A. ², Minenna G. ¹, D’Amore M. ¹, Angelini G. ²

¹ Rheumatology Section Department of Internal Medicine and Public Health University of Bari, Bari, Italy 2 Dermatology Section Department of Clinical Medicine Immunology and Infectious Diseases University of Bari, Bari, Italy

Two cases of livedo racemosa are described, whose onset mani-fested in adolescence in a mother and daughter. They were followed up for 30 and 17 years, respectively. In both patients slow, progressive pulmonary fibrosis developed, requiring a lung transplantation in the mother. A critical, in-depth analysis of the clinical and laboratory findings and lengthy follow-up data has led us to exclude all known causes of livedo, and to propose a new nosographic entity, “Familial Livedoid Pneumofibrosis”, to be included among the vasculitic connective tissue diseases