Medical Insider – Dr. Ria P. Maslog
EDITED AND APPROVED
Acleroderma
This is a generalized disorder of the connective tissue and this is otherwise known as Scleroderma.
Clinically, this disease may become severe and progressive. There will be fibrosis and generalized changes in the skin, digital arteries, synovium and a number of internal organs.
The form of scleroderma common among the pediatric population is the Focal Scleroderma.
Clinically, Focal Scleroderma presents as:
– indurations in the skin
– and sometimes atrophy and pigmentary changes of the discrete areas of the skin
Females are more affected than males and the ration is 3:1.
Specifically, the skin lesions are discrete, elevated, firm , waxy-colored or non-colored plaques with a violaceous halo. These lesions are most commonly affecting the neck area and are described as “ encased in paraffin”.
Because of this , there will be muscle atrophy and sometimes bone demineralization with limitation of motion secondary to extensive scarring.