Ann Rheum Dis: first published as 10.1136/ard.8.1.1 on 1 March 1949. Downloaded from A VARIANT OF RHEUMATOID ARTHRITIS CHARACTERIZED BY RECURRENT DIGITAL PAD NODULES AND PALMAR FASCIITIS, CLOSELY RESEMBLING PALINDROMIC RHEUMATISM BY E. G. L. BYWATERS From the Department of Medicine, Postgraduate Medical School of London, and the Special Unit for Juvenile Rheumatism, Canadian Red Cross Memorial Hospttal, Taplow, Buckinghamshire INDEX Page Page INTRODUCTION .. 1 Relation to lupus erythematosus . 26 CASE HISTORIEs (1-6) .. 2 Relation to lipoidosis 26 HISTOLOGY Relation to palindromic rheumatism 27 Synovial membrane 22 Relation to gout .. 28 Nodules .... 23 Relation to rheumatoid arthritis .29 DISCUSSION SUMMARY .. 29 copyright. Cutaneous nodules .. 23 REFERENCES .. ..t 29 Palmar and Digital contractures 24 Introduction (c) psoriatic arthritis, characterized by ter- Observations culninating in the work of Nichols minal interphalangeal joint involvement and http://ard.bmj.com/ and Richardson in 1909 clearly differentiated association of activity with skin exacerbations; rheumatoid arthritis from degenerative joint disease, (d) joint disorders associated with ulcerative a distinction which has been universally accepted colitis and other intestinal diseases (for example and has done more to clarify our ideas on chronic Whipple's syndrome), characterized by a rheumatism than any other single concept. Rheu- relatively mild course with remissions and matoid arthritis itself, however, has tended since exacerbations dependent on activity of the then to become an unwieldy nosological hotch- primary disease; on September 28, 2021 by guest. Protected potch, including almost any chronic joint affliction (e) spondylitis ankylopoietica or, as some which is not obviously exogenous (for example prefer to call it, rheumatoid arthritis of the traumatic or bacterial) in origin. Many 'have sug- spine, diffeiing in the earlier age and prepon- gested that the term includes a variety of diseases, derantly male incidence: its peripheral joint and attempts have been made to separate out such manifestations are different neither clinically clinical entities as: nor pathologically from those of rheumatoid (a) infective arthritis, characterized by the arthritis "proper "; involvement of a few large joihts and the (f) intermittent hydrarthrosis and palin- presence of a focus of infection, but no recover- dromic rheumatism (Hench) which are thought able metastatic organism and by a tendency by some, but not by Hench, to be variants to improve or even to heal following the removal of the rheumatoid arthritis syndrome; certainly of the focus; some cases of intermittent hydrarthrosis ulti- (b) classical rheumatoid arthritis, occurring mately develop the same type of permanent in middle-aged women and involving, charac- joint involvement as in rheumatoid arthritis: teristically, multiple finger joints; see later discussion; 2 Ann Rheum Dis: first published as 10.1136/ard.8.1.1 on 1 March 1949. Downloaded from 2- ANNALS OF THE RHEUMATIC DISEASES (g) Still's disease. This is rheumatoid arth- others do not-but to " account " for the variant ritis occurring in children and the so-called we postulate some extra factor, operative before or distinguishing features (for example late radio- after the onset of the main disease, either deter- logical involvement) are due only to the mined by it or actually determining the main increased cartilage protection of epiphyseal disease. bone at this age. Other features, such as A further characteristic of such variants is that a pericarditis, enlarged glands, and spleen, are gradation exists between the type and its variant. seen in adults as well; Thus we do not include rheumatic fever as a variant (h) arthritis associated with visceral dis- of rheumatoid arthritis because we do not see cases seminated lupus erythematosus (see later which are pathologically halfway between rheumatic discussion); fever and rheumatoid arthritis; our patients turn out (i) Felty's syndrome with arthritis, spleno- ultimately to have either permanent joint or per- megaly, anaemia, leukopenia, and pigmefltation manent heart disease or neither (in contra-distinc- of the skin is now generally agreed (for example tion to those of other clinics, for example, Talkov and others, 1942), to be rheumatoid Baggenstoss and Rosenberg, 1941; Bayles, 1943; arthritis, occasionally complicated by coincident Young and Schwedel, 1944). Thus, to prove a disease such as cirrhosis of the liver: in the syndrome to be a variant, it is necessary to establish classical rheumatoid case, any or rarely all of the existence of lesser and variable degrees of these extra signs may be present; variation from type, that is, transitionaf forms. (rheumatoid arthritis It is the object ofthis paper to detail such a variant. (j) Sjogren's syndrome A case will first be described (in full detail since it with kerato-conjunctivitis sicca). appears to be unique) which shows the variation Thus, we have a number of loosely conceived at a maximum, so much so that the correct diagnosis nosological entities of whose life course we know was not reached for nine years: following this, other as yet little and whose pathological changes, still ill- cases will be detailed rather more shortly, showing understood, appear superficially to resemble each transition to the more usual type of rheumatoid other very closely. These various syndromes are arthritis. copyright. statistically determined rather than true entities, Case Histories based on clinical or anatomical data rather than a knowledge of aetiology. The chief reason for CASE 1 distinguishing between them is the practical useful- E.P., a man aged 60, on his first admission (Feb. 2, ness ofsuch distinctions for prognosis and treatment. 1943) with no family history or relevant disease, had been in good health until the age of 51, except for a minor For scientific purposes, the resemblances between http://ard.bmj.com/ these various conditions are, from many angles, degree of silicosis contracted during service as a mining more important than their differences; it seems engineer inf South Africa, for which he was invalided at the age of 45. He had contracted no tropical' disease. probable that the basic (and unknown) pathological The present complaint started in 1934 at the age of 51* processes are, in all, similar in nature. This means following an exposure to damp and cold: the hands that these conditions may be considered as variants became swollen and painful in attacks lasting some two on a basic theme, or from a basic type. Such or three days. At this period he had perhaps two attacks variants fall into several possible categories. The every month, affecting only the hands and feet. These variation-producing factor may be constitutional are described as swellings and pain in the neighbourhood on September 28, 2021 by guest. Protected (for example, the congenital ductus arteriosus variant of the proximal interphalangeal joints, one or two being of subacute bacterial endocarditis); it may be age affected at each incident, and then perhaps others in of onset (for example, Still's disease); it may be succeeding incidents. These attacks continued to 1935, such as being always more frequent during the winter months, modification by environmental factors, but completely absent in a warm climate (South Africa nutritional state, etc., or it may be the presence of in December 1935 and January 1936) and returning some other disease producing a pseudovariant on the voyage home. (for example, cirrhosis ofliver complicating rheuma- In October 1935 the -patient saw Consultant I toid and producing Felty's syndrome). There might (Medicine) complaining at that time of " superficial be variation in original exogenous stimulus (for painful swellings on the toes, fingers, wrists, knees, and example, antigen)-or variation in the type or soles of feet, especially marked in cold weather". location of tissue originally stimulated. Of such * The patient's wife was medically qualified and kept a detailed vagaries we know very little-why some patients diary of her husband's condition and copies of the medical reports lesions and others from the numerous specialists whom he consulted; I sm much indebted with rheumatoid develop eye to her for allowing me to see and use these documents, as well as to the do not, why some psoriatics develop arthritis and consultants themselves. Ann Rheum Dis: first published as 10.1136/ard.8.1.1 on 1 March 1949. Downloaded from A VARIANT OF RHEUMATOID ARTHRITIS 3 Examination showed nothing abnormal except a small swelling arising in two to three hours, sometimes taking oedematous area appearing during examination between days to subside. This was brought on by cold weather the index and middle knuckles of the left hand, which but left no permanent change. A tentative diagnosis disappeared in a few minutes. In between these attacks of gout was made and the patient was treated with the patient was perfectly well. For example; aspirin and colchicum. No improvement having followed a holiday in Bermuda in April "Aug. 2.-Swelling of right little finger. 1937, he was sent Aug. 6.-Finger better. into a private clinic under the care of Consultant V Aug. 7-8.-Right wrist painful. (Gastro-enterology), where the-condition was thought to Aug. 9.-Wrist free of pain. Got slightly chilled: be gout. Blood uric acid was 3-6 and later. 3-2 mg. by evening the right index finger was considerably swollen per 100 c.cm. Atophan at first seemed to be helpful, with clear distal demarcation, swelling covering one inch causing the swellings to disappear for a few days, but above and below middle phalangeal joint; middle finger subsequently it had much less obvious effect. The also swollen. Left' hand: middle finger swollen and erythrocyte sedimentation rate was recorded as 62 mm, similar to right index. Feet: uncomfortable but not definitely swvollen. and 4 mm. per hour (Westergren) one month later. Aug. 10.-Swelling of fingers improved. Haemoglobin was 96 per cent., red cell count 4,900,000 Aug.