Parry Romberg syndrome

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What is Parry Romberg syndrome?

Parry–Romberg syndrome is the term given to progressive hemifacial atrophy. It typically affects children and adolescents, with tissue atrophy involving layers that can extend from the skin down to subcutaneous fat, muscle, and bone.

What is Parry Romberg syndrome potential relation to linear scleroderma?

Skin fibrosis may be absent among many patients with progressive hemifacial atrophy, but up to 40% have concurrent linear scleroderma ( en coup de sabre variant), thus raising speculation that the two conditions may represent a spectrum of the same disease. This is a controversial area as data is limited. The two conditions share a similar age of onset, can be associated with similar neurologic and ophthalmologic sequelae, and coexist in 40% of patients with hemifacial atrophy. Furthermore, there are case reports of patients with en coup de sabre progressing to hemifacial atrophy, and 30% to 40% of patients with Parry–Romberg syndrome will have findings of localized scleroderma outside the face. Despite these commonalities, the majority of patients with Parry–Romberg have no history of cutaneous sclerosis, and the extent of atrophy often extends to the maxillary, oral, and mandibular areas (sites less commonly involved by en coup de sabre ).

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