Clinical vignette

A 15-year-old boy presents with a history of multiple lumps since the age of 3 which are slowly progressing. He was the second child of non-consanguineous parents with no past medical history. The lumps were first noted on his head, and subsequently on his neck and back. By the age of 6, the lumps on his upper back had increased in size to the point where he became permanently kyphotic. By the time of presentation at age 13, his neck lumps had limited neck rotation to 20-30 degrees to either side. Upon examination, there were multiple lumps along the right axilla, posterior neck, back and extremities. He had kyphosis and levoscoliosis as well as limitation of movement, particularly in the axial skeleton. A series of radiographs taken are shown in Figures 1A-1C, illustrating the constellations of findings typical of FOP. Fibrodysplasia ossificans progressiva is a rare and disabling genetic condition characterized by congenital malformation of the great toes and by progressive heterotopic ossification in specific anatomic patterns [1]. The worldwide prevalence is approximately 1 in 2 million people with no ethnic, racial, gender or geographic predilection.


Book reviews
The Rheumatological Physical Examination.Edited by H Little. Pp. 149.US$39)50.Grune and Stratton:  Florida.1986.This book is aimed at students, interns, and trainees in rheumatology.The introduction correctIx states that physical examination is paramount in rheumatological management and that it is a bedside activity.It follows that it can only be properly learnt at the bedside.A written text will serve for reference and for revision.This particular volume will need to be extensively revised and rearranged before it becomes useful for its purpose.
It opens with separate chapters on the general features of normal and of abnormal joints.This is followed by the longest chapter.which deals with the three minute complete examination of the normal co-operative patient.lavishly illustrated with photographs.Then.separate chapters deal with the detailed examination of individual joints or regions.Each of these chapters begins with an outline of embryonal development, which is sketchv and not quite relevant, followed by an anatomical description of each joint which is not quite detailed enough for the serious student.
Many illustrations appear twice and there are separate numbering sequences for diagrams and for photographs.
As a result the numbering system is totally confusing.I would have preferred to see a single sequence of chapters and illustrations with differential typeface for the 'rapid' and the 'in depth' examinations.
The last chapter describes a method of measuring muscle strength by means of a modified sphygmomanometer device.This method is widely used for measuring grip strength.Curiously.this application is not described.Normal values are not given and validation of the method is not mentioned in the text or the references.
The editor decided to exclude features of spinal cord.nerve root, and peripheral nerve lesions (apart from carpal tunnel), though such conditions frequently enter the differential diagnosis of rheumatological cases and form an integral part of the management of such patients in this country.In view of the wide possible variability of normal joints in different individuals there is insufficient emphaisis on the assessment of unilateral lesions by comparison with the other side.
Several common features and procedures have been omitted, notabiv the isometric testing of muscles to detect rotator cutf lesions in the shoulder and in the hip and epicondylar Icsions at the elbow.Heberden's and Bouchard's nodes, Baker's cyst, and hallux rigidus are not mentioned.The differentiation of a knee effusion from synovial proliferation by means of the patellar tiap and the method of testing tor plantar fasciitis are ni)t described.
Neither are mid-tarsal axial rotation ot the foot and the window' sion ot svnovitis in the metatarsophalangeal joints.
Various features are poorly described or misleading.Twice, the mechanism involved in 'cracking' joints is explained wronglvy and pure testing of hip rotation is omitted.I would also disagree with the statement that thoracic spinal rotation caln be tested in the standingo subject.
This hook could torm the basis of an excellent seconid edition.Consultant Rheumatologist.
Rheumatology 85 comprises a series of articles coverino a broad range of rheumatological topics.including much related basic science.The book is looselv based on the series ot review lectures given at the 1985 ILAR conference in Sydney and is thus more a 'state of the art' reviews than a collation of the multitude of original reports presented at that meeting.It has been published barely a vear after the event and includes some articles w ith references to work published or 'in press' in 1985 tribute indeed to editorial tenacity!The price of such speed is the photolithographic reproduction of authors' original manuscripts which differ in format and tvpeface and are often difficult to read rapidly.The overall impression is unattrac- tive.
The articles vary in approach and style: none is outstanding, though I was interested to read those dealing with repetitive strain injury, a topic more ot us will have to grapple with in the near future, I fear.The inclusion of a few brief chairman's comments adds little except to remind the reader that the book purports to be a congress report.I approached this 5f10 page.£65-(00 tome with some trepidation and emerge from the experience not really convinced that it has added much original information to the already vast rheumatological literature.If someone is looking tor an update it bears consideration, but I would not recommend its automatic purchase either by indi- viduals or for departmental libraries.