Parry-Romberg Syndrome

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A 17-year-old patient with Parry-Romberg syndrome.
A 3D reconstruction of a CT scan from the same patient. The shrinkage of the fatty tissue and the facial muscles in - in this case - healthy facial bones is clearly visible.
Classification according to ICD-10
G51.8 Other diseases of the facial nerve
ICD-10 online (WHO version 2019)

The Parry-Romberg syndrome also Progressive hemifacial atrophy ( progressive facial hemiatrophy ), half-sided facial atrophy , Neurotic Gesichtsatrophie , Säbelhieb Allah and Prosopodysmorphie called, is a rare disorder of unknown cause, which is typically a progressive unilateral atrophy ( hemiatrophy ) of the face comes. The disease was named after Moritz Heinrich Romberg and Caleb Hillier Parry, who first described it.

Epidemiology

Parry-Romberg syndrome is a very rare condition for which the frequency of the disease is unknown. It occurs preferentially in young women ( gynecotropia ). At first onset, 75% of the predominantly female patients are under 20 years old.

root cause

The cause of the disease is unclear. Infections , especially those with Borrelia , an autoimmune genesis, trauma and genetic factors are discussed as causes . The detection of antinuclear antibodies in more than half of the cases and the detection of rheumatoid factors in about a third of the cases are considered as evidence of an autoimmune genesis .

Clinical picture

The disease is characterized by unilateral progressive atrophy of some or all of the tissues on one side of the face. Initially, there may be hypo- or hyperpigmentation of the affected facial skin. The atrophy of the skin and hyperpigmentation of the affected side are called “coup de saber”. Fat tissue , muscles , cartilage and bones can also be involved. Involvement of the extremities has been described.

It can migraine , trigeminal neuralgia , focal epilepsies occur. Also described depigmentation and hair loss on the affected side.

Diagnosis

The diagnosis is made taking into account the medical history and clinical examination findings. The diagnosis can be supported by imaging of the head ( magnetic resonance imaging (MRI) or computed tomography (CT)) and by histopathological examination of the skin. In the computed tomography, hypodensities and calcifications can be detected if the brain is involved. In magnetic resonance imaging, enhancement of the white matter and the meninges can be detected.

Therapy and prognosis

Parry-Romberg syndrome is an initially progressive and self-limiting disease. On average, disease activity lasts 7 to 9 years. There is no known definitive curative therapy for the active phase of the disease. However, there are numerous individual case reports in which various drugs and other forms of therapy have been used with success. This includes

If disease activity is no longer detectable, that is, if the disease no longer progresses, reconstructive and plastic surgical measures are possible. For example, pedicled free fatty tissue plastic is used, with the help of which the atrophic half of the face can be reconstructed. The use of autologous lipo injections and foreign body injections (hydroxyapatid granules) have also been described.

further reading

  • J. El-Kehdy, O. Abbas, N. Rubeiz: A review of Parry-Romberg syndrome. In: Journal of the American Academy of Dermatology . Volume 67, Number 4, October 2012, pp. 769-784, ISSN  1097-6787 . doi : 10.1016 / j.jaad.2012.01.019 , PMID 22405645 . (Review).
  • Andreas Bolgien: The history of the trophoneuroses with special consideration Moritz Heinrich Rombergs (1795–1873) and hemiatrophy. In: Würzburg medical history reports. Volume 25, 2006, pp. 175-203.
  • A. Kühne, T. Kyburz, G. Burg: The Parry-Romberg Syndrome. In: Switzerland Med Forum. Volume 5, 2005, pp. 615-617. PDF version
  • E. Roller, J. Reifenberger, B. Homey, D. Bruch-Gerharz: Hemiatrophia faciei progressiva (Parry-Romberg syndrome). In: The dermatologist. Volume 57, number 10, pp. 905-906.

Individual evidence

  1. ↑ Alphabetical index for the ICD-10-WHO version 2019, volume 3. German Institute for Medical Documentation and Information (DIMDI), Cologne, 2019, p. 681
  2. ↑ Andreas Bolgien: The history of trophoneuroses with special reference Moritz Heinrich Romberg (1795-1873) and the hemiatrophy. In: Würzburg medical history reports. Volume 25, 2006, pp. 175–203, here: p. 175.
  3. ^ A b c d e f A. Kühne, T. Kyburz, G. Burg: The Parry-Romberg Syndrome. In: Switzerland Med Forum. Volume 5, 2005, p. 616. PDF version
  4. ↑ U. Jappe, E. Hälzle, J. Ring: Parry-Romberg Syndrome. In: The dermatologist . 1996; 47, pp. 599-603.
  5. ^ MT Sahin, S. Baris, A. Karaman: Parry-Romberg syndrome: A possible association with borreliosis. In: Journal of the European Academy of Dermatology and Venereology . 2004; 18 (2), pp. 204-207.
  6. ^ I. Garcia-de la Torre, J. Castello-Sendra, T. Esgleyes-Ribot, G. Martinez-Bonilla, J. Guerrerosantos, MJ Fritzler: Autoantibodies in Parry-Romberg syndrome: a serologic study of 14 patients. In: J Rheumatol . 1995 Jan; 22 (1), pp. 73-77.
  7. ^ MS Chapman, JE Peraza, SK Spencer: Parry-Romberg syndrome with contralateral and ipsilateral extremity involvement. In: Journal of Cutaneous Medicine and Surgery . 1999 Jul; 3 (5), pp. 260-262.
  8. ↑ a b c R. Madasamy, M. Jayanandan, UR Adhavan, S. Gopalakrishnan, L. Mahendra: Parry Romberg syndrome: A case report and discussion. In: Journal of oral and maxillofacial pathology: JOMFP. Volume 16, Number 3, September 2012, pp. 406-410, ISSN  0973-029X , doi : 10.4103 / 0973-029X.102498 , PMID 23248475 , PMC 3519218 (free full text).