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July 22, 2020

Early Features of Progressive Hemifacial Atrophy–Clinical and Imaging Findings

Author Affiliations
  • 1Dermatology Program, Division of Immunology, Boston Children’s Hospital and Harvard Medical School, Boston, Massachusetts
  • 2Department of Dermatology, Massachusetts General Hospital and Harvard Medical School, Boston
  • 3Division of Vascular and Interventional Radiology, Boston Children’s Hospital and Harvard Medical School, Boston, Massachusetts
  • 4Rheumatology Program, Division of Immunology, Boston Children’s Hospital and Harvard Medical School, Boston, Massachusetts
  • 5Department of Dermatology, Brigham and Women’s Hospital and Harvard Medical School, Boston, Massachusetts
  • 6Department of Plastic and Oral Surgery, Boston Children’s Hospital and Harvard Medical School, Boston, Massachusetts
JAMA Dermatol. Published online July 22, 2020. doi:10.1001/jamadermatol.2020.2593

Progressive hemifacial atrophy (HFA) is a rare craniofacial disorder characterized by progressive atrophy of the skin, subcutis, muscles, and bone. Classified on a spectrum with frontoparietal linear morphea/en coup de sabre (ECDS), HFA occurs deep, with atrophy of the subcutis, muscle, and bone. Features of ECDS and HFA often coexist,1 and the 2 entities are considered variants of craniofacial localized scleroderma.2

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    1 Comment for this article
    Preventing avoidable loss of vision in children with progressive hemifacial atrophy
    Peter Shah, BSc MA FRCOphth FRCP Edin | University Hospitals Birmingham NHS Foundation Trust
    Jones et al provide an excellent overview of progressive hemifacial atrophy in children. An important clinical point is to look for ophthalmic features that can be associated with progressive loss of vision, that are often asymptomatic in the early stages.

    Iris anomalies and asymmetry, such as congenital ectropion uveae, and glaucoma can be detected by a careful ophthalmic examination. Close teamwork between the the dermatology and ophthalmology teams can prevent avoidable, irreversible loss of vision.
    CONFLICT OF INTEREST: None Reported
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