A rare disorder characterized by progressive atrophy of some or all tissues on one side of the face, occasionally extending to other parts of the body. The process may be bilateral in 5% to 10% of cases. Tissues involved include the skin, tongue, gingiva, soft palate, the cartilage of the nose, ear, subcutaneous fat, larynx, muscle, and bone. It may be associated with a localized patch of scleroderma in the frontal region of the scalp, causing a patch of cicatrical alopecia (called a 'coup de sabre' because it resembles the scar of a wound made by a sabre).
Onset occurs most often before puberty or during adolescence, but occasionally begins in middle or later years. Either sex may be affected. It is believed to be related to scleroderma. A few familial instances are suggestive of autosomal dominant inheritance.
The first mention of this condition was made by Parry, whose medical writings were published posthumously in 1825. After a stroke in 1816 Parry was unable to continue with his medical practice. Despite his disability, his intellect was unimpaired, and with the assistance of his dutiful daughter, he accumulated a collection of his writings and observations. These include an account of progressive hemifacial atrophy. They were published three years after his death by his eldest son, the physician Charles henry Parry.
The disorder was again described by Romberg in 1846, and the descriptive title " progressive hemifacial atrophy" was coined in 1871 by the German neurologist Albert Eulenburg (1840-1917). In addition to the descriptive designation, the names of Romberg and Parry have been used together and separately. The disorder is not uncommon, and in 1963 Rogers was able to review 772 cases. A definitive review was given by Lewkonia and Lowry in 1983.
- C. H. Parry:
In his: Collections from the Unpublished Medical Writings of the Late Caleb Hillier Parry. Volume I.
London, Underwood, Fleet Street, 1825, pp. 478-480.
- M. H. von Romberg:
In Romberg: Klinische Ergebnisse. Berlin, Förstner, 1846¸ pp 75-81.
- A. Eulenburg:
Lehrbuch der functionellen Nervenkrankheiten auf Physiologischer Basis.
Berlin, A. Hirschwald, 1871. Page 712.
- B. O. Rogers:
Progressive facial hemiatrophy: Romberg's disease: a review of 772 cases.
Proceedings of the 3rd International Conference on Plastic Surgery. Excerpta Medica. International Congress Series, 1963, 66: 681-686.
- R. M. Lewkonia, R. B. Lowry:
Progressive hemifacial atrophy (Parry-Romberg syndrome) report with review of genetics and nosology. American Journal of Medical Genetics, New York, 1983, 14: 385-390.