Hemiatrophia faciei progressiva G51.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Atrophy of the half face; hemifacial atrophy; Hemifacial progressive atrophy; HFPC; Parry-Romberg syndrome; Romberg-Parry syndromes; Romberg Syndrome; Romberg Trophoneurosis

History
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Parry, 1825; Romberg, 1846

Definition
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This disease is characterized by a primarily atrophic transformation of the affected subcutaneous tissue, muscle and bone. Fibrosis is not or only rarely observed. It often begins in adolescence and childhood in the head region (see section Special Features in Childhood) and then increasingly affects the skin, subcutis, cheek muscles, bones, lips and tongue. This results in a pronounced asymmetry of the face.

Etiopathogenesis
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Not yet finally clarified. Probably variant of circumscribed scleroderma. The combination of Parry-Romberg syndrome with linear morphea is found in 30% of cases (see below), as is the joint occurrence with other autoimmune diseases. The consequences of trigeminal damage (toxic, traumatic, after infection) in childhood with chronic irritation of the cranial nerve are also discussed.

Manifestation
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Occurs in early adolescence or peripubertal, in 80% of patients before the age of 20 LJ.

Localization
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One half of the face or parts of one half of the face.

Clinical features
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Hemiplegic atrophy of skin, muscles and bones in the face, enophthalmos, possibly Horner's syndrome, formation of an asymmetrical face. Tightly atrophic, thinned, occasionally spotty hyper- or depigmented skin. Reduced sweat secretion. Additional occurrence of neurological symptoms possible (epilepsy, migraine). Depression and impaired vision may manifest clinically.

Differential diagnosis
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Therapy
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A therapy trial with penicillin is possible but not very promising. The course of the disease can hardly be influenced by therapy. In severe, progressive cases a systemic therapy with methotrexate should be discussed, possibly in combination with a steroid pulse therapy (see below circumscribed scleroderma). A balneo-phototherapy in the sense of a PUVA-bath therapy can have positive effects. In the case of burnt-out findings, plastic surgery corrections (e.g. fat implantations) are in the foreground.

Progression/forecast
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Spontaneous standstill is possible. There may be severe deformities involving the neck-shoulder region and the trunk. Central nervous disorders occur rarely.

Note(s)
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In a study with 54 volunteers, about 30% had hemiatrophia faciei progressiva and scléroderma en coup de sabre simultaneously. This suggests that there is a pathogenetic connection between both diseases.

Case report(s)
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This disease is characterized by a primarily atrophic transformation of the affected subcutaneous tissue, muscle and bone. Fibrosis is not or only rarely observed. This disease often begins in adolescence and childhood in the head region (see section on special features in childhood) and then increasingly affects cheek muscles, bones and also the tongue (Sommer et al, 2006; Tollefson 2007). This results in a partly pronounced asymmetry of the face. The simultaneous occurrence of linear circumscript scleroderma of the type en coup de sabre and progressive facial hemiatrophy is relatively frequently described with a coincidence of up to 40 % (Jablonska 1975a). In the classification proposed by the authors, it is therefore also listed under the linear forms, although a classification as a deep form is also possible in the case of pure involvement of extracutaneous structures. It is not uncommon for the central nervous system to be involved (see section on apparatus diagnostics). Antinuclear antibodies are detected in this subform in up to 50 % of patients.

Literature
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  1. Henoch E, Romberg HM (1846) Clinical results. A. Forstner, Berlin, S. 75-81
  2. Kaya M et al (2014) Chronologic presentation of a severe case of progressive hemifacial atrophy (parry-romberg syndrome) with the loss of an eye. Case Rep Otolaryngol doi: 10.1155/2014/703017
  3. Khan M et al (2014) Parry Romberg syndrome with localized scleroderma: A case report. J Clin Exp Dent 6:e313-316

  4. Kumar S et al (2014) Parry-romberg syndrome: a case of late onset with rapid progression. J Clin Diagn Res 8:ZD27-28

  5. Moko SB et al (2003) Parry-Romberg syndrome: intracranial MRI appearances. J Craniomaxillofac Surgery 31: 321-324
  6. Nair PA et al (2014) Parry-romberg syndrome: a linear variant of scleroderma with discoid lupus erythematosus on scalp - an association. Int J Trichology 6:71-74

  7. Parry CH (1825) Facial hemiatrophy. In: Collections from the unpublished medical writings of the late Caleb Hillier Parry. Vol. 1, Underwoods, Fleet Street, London, pp. 478-480
  8. Sandhu K et al (2004) Subdural hygroma in a patient with Parry-Romberg syndrome. Pediatric dermatol 21: 48-50
  9. Romberg HM (1846) Trophoneuroses. Clinical results. A Forstner, Berlin, S. 75-81
  10. Stone J (2003) Parry-Romberg syndrome: a global survey of 205 patients using the Internet. Neurology 61: 674-676
  11. Tollefson MM et al (2006) En coup de sabre morphea and Parry-Romberg syndrome: a retrospective review of 54 patients. J Am Acad Dermatol 56: 257-263

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Last updated on: 29.10.2020