Hutchinson-Gilford progeria is a rare genetic disorder resulting from mutations 
in the LMNA gene encoding lamin A/C. In addition to the classical phenotype 
usually caused by the 1824C>T mutation of LMNA, a number of atypical progeroid 
syndromes have been described. They have some distinct features, such as 
skeletal deformities or scleroderma-like skin changes. The underlying defect is 
usually a homozygous mutation of LMNA, or a combined defect of LMNA and another 
gene, for example, ZMPSTE-24. We present a 2-year-old girl born to 
consanguineous parents affected by progeroid syndrome with scleroderma-like skin 
changes. Genetic analysis revealed the homozygous LMNA mutation 1303C>T (R435C). 
The same heterozygous mutation was found in the patient's parents and 11 other 
family members. The progeroid syndrome in our patient shares the signs of two 
laminopathies: progeria and restrictive dermatopathy. Two other children in the 
family died at the age of 2 due to a disease similar to that in the proposita. 
On the basis of the family pedigree we presume that these children probably had 
the same homozygous LMNA mutation. Scleroderma-like skin changes in infants, 
associated with growth retardation and dysmorphic features, suggest premature 
aging syndrome, requiring genetic testing and counseling of asymptomatic 
carriers of LMNA mutations.