Parry-Romberg syndrome

Parry–Romberg syndrome (PRS), or progressive hemifacial atrophy (PHA), is a rare neurocutaneous disorder characterized by slow, unilateral wasting of the skin, subcutaneous tissue, and sometimes muscle, cartilage, and bone of the face. Onset usually occurs in childhood or adolescence (5–15 years) and progresses over 2–10 years before stabilizing.

Clinical features:

  • Facial changes: Hemifacial atrophy involving the cheek, periorbital region, lips, and mandible; overlying skin often hyperpigmented or hypopigmented.
  • Oral findings: Dental root resorption, delayed eruption, and unilateral tongue atrophy.
  • Ocular involvement: Enophthalmos from orbital fat loss; uveitis, keratitis, and Horner’s syndrome have been described.
  • Neurological associations: Migraines, trigeminal neuralgia, seizures (10–15%), and contralateral Jacksonian epilepsy are common.

The cause remains uncertain. Leading hypotheses include autoimmunity, localized sympathetic dysfunction, and trauma-triggered inflammation. Parry–Romberg syndrome is now considered part of a spectrum with linear scleroderma (morphea en coup de sabre).


History of Parry-Romberg syndrome

1825 – Charles Henry Parry included an account by his father Caleb Hillier Parry in his Collections from Unpublished Papers 3 years after his death

Miss F., aged twenty-eight, tall, thin, and with a somewhat florid complexion, thirteen or fourteen years ago, when at school, was rather suddenly seized with some degree of hemiplegia of the left side, accompanied with transient confusion of intellects…from the period of the attack the left side of the face began to grow more thin than the right, and the eye to become less prominent, and therefore to appearance smaller…when she protrudes her tongue, it turns to the left ; but there is no dropping of the eyelids, or corner of the mouth, nor any weakness or numbness of the limbs on the affected side.

Parry 1825: 478-480

1846Romberg gave a complete description of the disorder Trophoneurosen in Klinische Ergebnisse 1846: 75-81

Schon beim ersten Anblick zeigte sich ein wesentlicher Unterschied beider Gesichtshälften: die rechte hatte das normale Ansehn eines 28jährigen Mädchens, die linke das einer alten Frau… Die Mittellinie des Gesichts bildete eine scharfe Grenze, auf deren linken Seite das Alter, auf deren rechten die Jugend den Beschauer anblickte

…welcher dieser Klasse von Krankheiten, wo durch aufgehobenen Nerveneinfluss mangelhafte Ernährung bedingt wird, anzugehören scheint

Romberg 1846: 75-81

At first glance, there was a striking difference between the two halves of the face: the right had the normal appearance of a 28-year-old woman, the left that of an old woman… The midline of the face formed a sharp boundary, on the left age gazed at the observer, on the right youth

…this seems to belong to this class of diseases, where insufficient nutrition results from the withdrawal of nervous influence

Romberg 1846: 75-81

1871 – Albert Eulenburg (1840–1917), a German neurologist, was the first to use the descriptive term ‘Hemiatrophia facialis progressiva‘ [Progressive Hemifacial Atrophy (PHA)]

1871 – Albert Eulenburg (1840–1917)
Coins the term Hemiatrophia facialis progressiva to describe the syndrome.

1945Wartenberg reviews 300 cases, linking hemifacial atrophy and scleroderma under “heredodegeneration” hypothesis.

1983 – Lewkonia and Lowry demonstrate immunologic overlap with localized scleroderma (antinuclear antibodies, linear morphea).

2015Tolkachjov et al. comprehensive review supports PRS and morphea as part of a common disease spectrum.


Associated Persons

Alternative names
  • Progressive hemifacial atrophy (PHA)
  • Hemiatrophia facialis progressiva
  • Romberg syndrome
  • Romberg disease

References

Original Articles

  • Parry CH. Hemiplegia Miss F. In: Collections from the unpublished medical writings of the late Caleb Hillier Parry. London. 1825; 1: 478-480.
  • Romberg MH. Trophoneurosen. In: Klinische Ergebnisse. 1846: 75-81

Review Articles

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BA MA (Oxon) MBChB (Edin) FACEM FFSEM. Emergency physician, Sir Charles Gairdner Hospital. Passion for rugby; medical history; medical education; and asynchronous learning #FOAMed evangelist. Co-founder and CTO of Life in the Fast lane | Eponyms | Books |

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