Abstract
BackgroundProgressive Hemifacial Atrophy (PHA) is an acquired, typically unilateral, facial distortion with unknown etiology. The true incidence of this disorder has not been reported, but it is often regarded as a subtype of localized scleroderma. Historically, a debate existed whether PHA is a form of linear scleroderma, called morphea en coup de sabre (ECDS), or whether these conditions are inherently different processes or appear on a spectrum (; Adv Exp Med Biol 455:101–4, 1999; J Eur Acad Dermatol Venereol 19:403–4, 2005). Currently, it is generally accepted that both diseases exist on a spectrum of localized scleroderma and often coexist.The pathogenesis of PHA has not been delineated, but trauma, autoimmunity, infection, and autonomic dysregulation have all been suggested. The majority of patients have initial manifestations in the first two decades of life; however, late presentations in 6th and 7th decades are also described [J Am Acad Dermatol 56:257–63, 2007; J Postgrad Med 51:135–6, 2005; Neurology 61:674–6, 2003]. The typical course of PHA is slow progression over 2-20 years and eventually reaching quiescence.Systemic associations of PHA are protean, but neurological manifestations of seizures and headaches are common [J Am Acad Dermatol 56:257–63, 2007; Neurology 48:1013–8, 1997; Semin Arthritis Rheum 43:335–47, 2013]. As in many rare diseases, standard guidelines for imaging, treatment, and follow-up are not defined.MethodsThis review is based on a literature search using PubMed including original articles, reviews, cases and clinical guidelines. The search terms were “idiopathic hemifacial atrophy”, “Parry-Romberg syndrome”, “Romberg’s syndrome”, “progressive hemifacial atrophy”, “progressive facial hemiatrophy”, “juvenile localized scleroderma”, “linear scleroderma”, and “morphea en coup de sabre”. The goal of this review is to summarize clinical findings, theories of pathogenesis, diagnosis, clinical course, and proposed treatments of progressive hemifacial atrophy using a detailed review of literature.Inclusion- and exclusion criteriaReview articles were used to identify primary papers of interest while retrospective cohort studies, case series, case reports, and treatment analyses in the English language literature or available translations of international literature were included.
Highlights
Progressive Hemifacial Atrophy (PHA) is an acquired, typically unilateral, facial distortion with unknown etiology
History and nomenclature First described by Parry in 1825, and Romberg in 1846, this constellation of craniofacial findings was labeled as progressive hemifacial atrophy by Eulenberg in 1871
While a majority of patients experience halting of the facial atrophy, in the aforementioned global internet survey, 26% of patients reported disease acceleration. 68% of these cases were women and experienced worsening of facial hemiatrophy during pregnancy (9 women) or after childbirth (8 women)
Summary
This review is based on a literature search using PubMed including original articles, reviews, cases and clinical guidelines. Adnexal atrophy was seen in 11/13 of ECDS and 3/9 of PHA while mononuclear cell infiltrates was present in all ECDS biopsies and 6/9 PHA Another case series that followed 71 ECDS and PHA patients over a 20 year timespan, found that the clinical morphological pattern of the disease presentation changed progressively such that clear distinctions between the two entities often disappeared over time [3] (Table 1). Progression or transition of ECDS into PHA in the same physical location has been illustrated in a handful of cases [2,3] In another large series of ECDS and PHA patients, the authors concluded that their findings support these two entities as being on a spectrum of disease. Z-plasty, lip repair, nasal reconstruction, eyebrow repair, face-lift, lip augmentation, hair transplant, and other adjuvant procedures can be used to create a better cosmetic outcome [144,145,155]
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