The Extremities



The Extremities






For more than twenty years I have amused my compulsory leisure with collecting these curious physical signatures in this town. At my house I have hundreds and hundreds of them …. I have the finger-prints of the court, the sheriff, and every member of the jury. There is hardly a person in this room…whose natal signature I cannot produce, and not one of them can so disguise himself that I cannot pick him out from a multitude of his fellow-creatures and unerringly identify him by his hands. And if he and I should live to be a hundred I could still do it.

—MARK TWAIN, PUDD’NHEAD WILSON



Upper Extremities


Various Findings in Rheumatic Diseases


Nodules

The extensor surfaces of the upper extremities are the best places to find rheumatoid nodules, which can be found in about 28% of patients with rheumatoid arthritis. About 90% of such patients have nodules on the elbows, 14% on the fingers, 13% on the knees, 7% on the ankles, and 5% on the occiput. Rheumatoid nodules are not diagnostic for rheumatoid arthritis, but in patients with rheumatoid arthritis, the presence of nodules predicts a 90% chance of having rheumatoid factor (Kaye et al., 1984).

Nodules also occur in about 9% of patients with rheumatic fever. About 80% of such patients have nodules on the elbow, 19% on the fingers, 48% on the knees, 20% on the ankles, and 20% on the occiput (Benedek, 1984).

Table 24.1 shows diseases associated with subcutaneous nodules similar to those found in rheumatoid arthritis. Those in boldfaced letters have the best track record for fooling the clinician. Fortunately, most of these diseases have either decreased somewhat in prevalence (rheumatic fever, tophaceous gout, gummatous syphilis) or the diagnoses can be made on other grounds.


A Self-study

In your practice, you see 95 patients with rheumatoid arthritis for every five patients with rheumatic fever. What, then, is the likelihood (in your practice) that a patient with an occipital nodule has one or the other of the two diseases? The answer is in Appendix 24.1.


The Hand in Various Arthritides and Connective Tissue Diseases


Rheumatoid Arthritis

Rheumatoid arthritis can be diagnosed in some cases simply by inspecting the hand, even when no nodules are present. First, there may be interosseus atrophy, best noted by inspecting the dorsum of the hand in oblique lighting, but with practice, discernible in direct light. Weakness of the interossei results in inability to forcefully extend the interphalangeal joints, which is necessary for finger-flicking movements. Second, there is ulnar deviation of the hand and the fingers. The best fingers to check for ulnar deviation are the third and index fingers, as many normal older persons will appear to have mild ulnar deviation of the other fingers. In severe carpal rheumatoid arthritis, however, all the fingers show a clear ulnar deviation.

It is worth remembering that rheumatoid arthritis of the wrist tends to spare the radial side of the joint. In fact, a severe arthritis of the wrist that spares the ulnar side and vigorously attacks the radial side is likely to be osteoarthritis (degenerative joint disease). Also remember that classic rheumatoid arthritis never attacks the distal interphalangeal (DIP) joints.

Other manifestations of rheumatoid arthritis include (a) loss of grip strength; (b) limited flexion in one or more fingers due to nodular tenosynovitis (felt as a small movable nodule in the flexor tendon as you passively flex and extend the joint); (c) vasculitic lesions, which may herald serious systemic vasculitis (Weiss, 1984); and (d) Haygarth nodes (actually not nodes but fusiform synovial swellings of the proximal interphalangeal [PIP] joints).

Dr Gerry Rodnan of Pennsylvania made the diagnosis of rheumatoid arthritis by squeezing the metacarpal heads during a handshake (Rodnan et al., 1973). This can be quite painful for the patient. If you suspect this diagnosis, it is better not to shake hands but simply to ask what would happen if you were to squeeze the patient’s hand.

The American College of Rheumatology criteria for the diagnosis of rheumatoid arthritis are given in Table 24.2. Although the criteria focus on joint manifestations, remember that rheumatoid arthritis is a systemic disease (see Chapter 10 for eye findings).
Generalized symptoms such as fatigue, weight loss, malaise, and depression are also common (Lee and Weinblatt, 2001).








TABLE 24.1 Diseases associated with subcutaneous nodules similar to those found in rheumatoid arthritisa













































































































Rheumatic and immunologic diseases



Rheumatic fever



Systemic lupus erythematosus



Tophaceous gout



Discoid lupus erythematosus



Anarthritic rheumatoid syndrome



Degenerative joint disease (Heberden nodes)



Sarcoidosis



Weber-Christian disease



Agammaglobulinemia with polyarthritis



Dermatologic disease



Granuloma annulare



Erythema elevatum diutinum



Acrodermatitis chronic atrophicans



Discoid lupus erythematosus



Basal cell carcinoma



Sebaceous cysts



Metabolic diseases



Tuberous xanthomatosis (see Chapter 7)



Neoplastic diseases



Multicentric reticulohistiocytosis



Basal cell carcinoma



Infectious diseases



Syphilis



Synovial tuberculosis



Candidiasis



Bejel



Yaws



Pinta



Leprosy (Hansen disease)



Miscellaneous conditions



Ganglions of the hand or wrist



Foreign body reactions



Chemical irritations



Delayed local reaction to tuberculin


aConditions in bold face have fooled the clinician most consistently. From Kaye BR, Kaye RL, Bobrove A. Rheumatoid nodules: Review of the spectrum of associated conditions and proposal of a new classification, with a report of four seronegative cases. Am J Med. 1984;76:279-292, with permission.


image Although the patient’s and the doctor’s attention may be focused on the peripheral joints, the cervical spine is affected early in the course of rheumatoid arthritis, even within the first two years (see Chapter 25). This is for some reason strongly correlated with involvement of the carpal and metacarpophalangeal (MCP) joints (K. Smith, personal communication, 2009) and with erosions in the bones of the hands and feet (Winfield et al., 1983). Wasting of the forearm and hand muscles may result from compression of the anterior spinal artery at upper and mid cervical levels (Mathews, 1998).








TABLE 24.2 Diagnostic criteria for rheumatoid arthritis










































Criterion


Definition


1.


Morning stiffness


In/around joints, lasting at least 1 hour before maximal improvement


2.


Arthritis of three or more joint areas


At least three areas simultaneously have had soft tissue swelling or fluid; the 14 possible areas are R/L PIP, MCP, wrist, elbow, knee, ankle, and MTP joints


3.


Arthritis of the hand joints


At least one area swollen in the wrist, MCP, or PIP joint


4.


Symmetric arthritis


Bilateral PIP, MCP, MTP without absolute symmetry acceptable


5.


Rheumatoid nodules


Subcutaneous nodules over bony prominences, extensor surfaces, or in juxta-articular regions


6.


Serum rheumatoid factor


By any method in which result has been positive in <5% of normal control subjects


7.


Radiographic changes


PA hand/wrists X-ray with erosions or bony decalcification in or adjacent to involved joints


Four or more criteria needed to make diagnosis; must have 1-4 for at least 6 weeks.


PIP, proximal interphalangeal; MCP, metacarpophalangeal.


Source: From Mayeaux EJ Jr. 2000 Revised ARA Criteria for Classification of Rheumatoid Arthritis. Intern-in-the-middle-of-the-night series. Shreveport, LA: Louisiana State University Health Sciences Center. Available at: lib-sh.lsumc.edu/fammed/intern/ra.html. Accessed: June 18, 2004, with permission.



Psoriatic Arthritis

This seronegative rheumatoid variant attacks the DIP joints. The diagnosis is suggested by the associated skin and nail changes (see Chapter 7). Psoriatic arthritis may be severely deforming, whereas osteoarthritis is not.


Osteoarthritis

This “degenerative,” seronegative arthritis produces characteristic nodules of the DIP joints called Heberden nodes. These feel like two little dried split peas placed subcutaneously on the lateral and medial dorsal surface of the finger at the DIP. When they are present on the PIP joint, they are called Bouchard nodes. Osteoarthritis tends to spare the MCP joints.


Scleroderma

The American College of Rheumatology criteria for the diagnosis of progressive systemic sclerosis require the presence of
proximal scleroderma (skin tightness) or the presence of two of three other criteria. These criteria are



  • Sclerodactyly (vide infra);


  • Digital pitting scars;


  • Bibasilar pulmonary fibrosis.

Proximal is defined as above the wrists by the American College of Rheumatology, and above the elbows by various others. The criteria were designed for research and thus exclude individuals in whom the disease is not fully expressed. Scleroderma skin changes above the MCP or metatarsophalangeal (MTP) joints are 91% sensitive and 99.8% specific for definite scleroderma (Wigley, 2001).

Nail-fold capillary changes are described in Chapter 7.

Patients with sclerodactyly alone have some or all of the features of the CREST syndrome, and it has been argued that this syndrome should be called “limited scleroderma.” The CREST syndrome includes calcinosis, the Raynaud phenomenon (see Chapter 18), esophageal dysmotility, sclerodactyly, and telangiectasia.








TABLE 24.3 Diagnostic criteria for systemic lupus erythematosus






















































Criterion


Definition


1.


Malar rash (see Fig. 9-9)


Fixed malar erythema, flat or raised, tending to spare the nasolabial folds


2.


Discoid rash (see Fig. 7-10)


Erythematous raised patches with adherent keratotic scaling and follicular plugging


3.


Photosensitivity


Skin rash as a result of unusual sensitivity to sunlight


4.


Oral ulcers


Oral or nasopharyngeal ulceration, usually painless


5.


Arthritis


Nonerosive arthritis involving peripheral joints, with tenderness, swelling, or effusion


6.


Serositis


Pleuritis—convincing history of pleuritic chest pain or rub or evidence of pleural effusion OR pericarditis—documented by ECG, rub, or evidence of pericardial effusion


7.


Renal disorder


Persistent proteinuria, >0.5 g/day or >3+ if quantification is not performed OR cellular casts—may be red cell, hemoglobin, granular, tubular, or mixed


8.


Neurologic disorder


Seizures OR psychosis in the absence of offending drugs or known metabolic derangements (uremia, ketoacidosis, or electrolyte imbalance)


9.


Hematologic disorder


Hemolytic anemia—with reticulocytosis OR leukopenia: <4,000 total at least twice OR lymphopenia: <1,500 at least twice times OR thrombocytopenia: <100,000 in absence of offending drugs


10.


Immunologic disorders


Positive antiphospholipid antibody OR anti-DNA OR anti-SM OR false-positive serologic test for syphilis


11.


Antinuclear antibody


Positive antinuclear antibody (ANA), at any point in time, and in the absence of known drugs to be associated with “drug-induced lupus” syndrome (see Table 24.4)


Diagnosis requires four or more manifestations present serially or simultaneously. Remember that these are simply “criteria.” Always use your clinical judgment in evaluating a patient for potential lupus.


From Gill JM, Quisel AM, Rocca PV, et al. Diagnosis of systemic lupus erythematosus. Am Fam Physician. 2003;68: 2179-2186 and Callegari PE, Williams WV. Laboratory tests for rheumatic diseases: When are they useful? Postgrad Med. 1995;97(4):65-74, with permission.


New criteria have been proposed for early diagnosis and classification on the basis of advances such as the identification of scleroderma (anticentromere) autoantibodies and other advances (LeRoy and Medsger, 2001). Disease manifestations are variable and complex. The use of a term such as “undifferentiated connective disease with features of scleroderma” has been suggested as a hedge against overdiagnosis and overly aggressive treatment (Wigley, 2001).


Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is a multisystem disease diagnosed by a combination of clinical and laboratory criteria. The American College of Rheumatology diagnostic criteria are given in Table 24.3. Always remember that diseases do not necessarily fit precisely into authoritatively defined pigeonholes. In particular, there are many more neurologic manifestations, both central and peripheral, that have been attributed to lupus. These include demyelinating disorders, chorea, Guillain-Barré syndrome, myasthenia gravis, and autonomic disorders (Ruiz-Irastorza et al., 2001).









TABLE 24.4 Drugs associated with systemic lupus erythematosus






















































































































































Definite association



Chlorpromazine



Hydralazine



Isoniazid



Methyldopa



Procainamide



Quinidine


Possible association



β blockers



Atenolol, labetalol, timolol



Captopril



Carbamazepine



Clonidine HCl



Danazol



Disopyramide



Ethosuximide



Gold compounds



Griseofulvin



Interferon alpha



Leuprolide acetate



Levodopa



Lithium



Lovastatin



Mephenytoin



Methyldopa



Methysergide



Minoxidil



Nalidixic acid



Nitrofurantoin



Nonsteroidal anti-inflammatories



Diclofenac, ibuprofen, sulindac



Oral contraceptives



Penicillamine



Penicillin



Phenelzine sulfate



Phenytoin



Prazosin



Primidone



Promethazine



Propylthiouracil



Psoralen



Spironolactone



Streptomycin



Sulfasalazine



Tetracycline



Thioridazine



Tolazamide



Tolmetin



Trimethadione


From Greenberg B, Michalska M. Systemic lupus erythematosus. Postgrad Med. 1999;106:213-223, with permission.


Almost all patients experience arthralgias and myalgias, and most develop intermittent arthritis. Pain is often out of proportion to physical findings. Characteristically, there is symmetric fusiform swelling of joints, most frequently the PIP and MCP joints and the wrists and knees. Diffuse puffiness of hands and feet, and tenosynovitis, may be seen. In contrast to rheumatoid arthritis, joint deformities are unusual, with only 10% of patients developing swan-neck deformities of the fingers and ulnar drift at MCP joints. Erosions are rare. Subcutaneous nodules occur.

A careful drug history is mandatory. Drugs that have been associated with SLE are listed in Table 24.4. The syndrome is rare except with procainamide (the most common offender) and hydralazine, and is probably related to genetically determined drug acetylation rates.


Gout

Tophi occur more frequently on the ears and feet, and when present on the hand (Fig. 24-1), though highly suggestive of gout, could be initially confused with a rheumatoid nodule. Definitive diagnosis is made by joint aspiration and demonstration of urate crystals (see Chapter 28). Presence of a tophus is 33% sensitive and 93% specific for gout.


Hemochromatosis

Patients with hemochromatosis are prone to a degenerative type of arthritis that can affect the hands and fingers as well as the joints of the lower extremities. The PIP joints of the middle and ring fingers may be affected, mimicking the Haygarth fusiform swelling of rheumatoid arthritis confined to those joints.


Self-test

See Figure 24-2 Legend.






FIGURE 24-1 This phlegmatic patient accepted the diagnosis of “arthritis” for many years. No one seemed to realize that the development of these tophi was not an inevitable consequence of “arthritis.” Aspiration of the joint by Dr Renee Ridzon of Massachusetts revealed the milk of urate shown in the syringe. Examination of the fluid by means of the improvised polarizing microscope and first-order compensator (see Chapter 28) revealed the pathognomonic urate crystals. (Courtesy of Dr Renee Ridzon, Massachusetts.)







FIGURE 24-2 A, B: Self-test: One hand belongs to a patient with rheumatoid arthritis and one to a patient with porphyria cutanea tarda. Identify the diagnosis and then the skin lesions. Skin lesions must always be considered in the context in which they occur. Neither of these diagnoses could easily be made from the skin lesions alone. Therefore, look for interosseus muscle atrophy and slight ulnar deviation, the signs of rheumatoid arthritis but not porphyria (see Appendix 24.2 for the answer).


Bony Anomalies


Short Fourth Metacarpal

One can diagnose a short fourth metacarpal without taking a radiograph by having the patient make a list and noticing that the fourth knuckle is dimpled inward. Shortening of this bone is seen in pseudohypoparathyroidism, pseudopseudohypoparathyroidism, gonadal dysgenesis (Turner syndrome), and in 10% of healthy subjects (Levin and Kupperman, 1964; Slater, 1970).

The hypothesis that pseudohypoparathyroidism resulted from end-organ resistance to a circulating hormone was first proposed by the Boston endocrinologist Fuller Albright. This possibility, previously unknown as a mechanism for human disease but subsequently confirmed, was suggested to him by analogy with the Sebright bantam rooster, which fails to develop secondary sex characteristics owing to end-organ resistance to circulating male sex hormones. A careless typesetter changed the Sebright bantam syndrome to the “Seabright-Bantam” syndrome, allowing Drs Seabright and Bantam to join Dr Jod (see Chapter 14) in the pantheon of eponymously immortalized, albeit nonexistent, endocrinologists.


Metacarpal Index

By palpation, one can estimate the metacarpal length-to-width ratio (metacarpal index) as a method of quantitating the elongation of the metacarpal bones in Marfan syndrome (also see section on “Arachnodactyly” below). The independent covariable is posteroanterior hand films taken with the hands flat against the plate, which are used for measurement of the second through fifth metacarpals. The ratio of the length of the bone divided by the width (at the midpoint of the length) is used to generate an average, whose upper limit of normal is 8.8 for men and 9.4 for women (Eldridge, 1964). This ratio distinguished the patients with Marfan syndrome from those without it.


Acromegaly

Acral enlargement, the literal translation of “acromegaly,” involving the hand, foot, and head, occurs in 98% of cases of this disease. Actually, the hand develops a characteristic appearance that is easy to recognize once seen. This is called the “spade hand” because the hand vaguely resembles the garden implement (Fig. 24-3). The diagnosis of acral enlargement may also be based on the history of an increase in glove, ring, shoe, and/or hat size (Braunstein, 1986).

The feet also enlarge in acromegaly, and the heel pad thickens, the latter originally being demonstrated radiographically.


The Palms


Skin

Lesions of the palmar skin may be divided into dermatologic conditions and those that are part of a systemic medical illness.


Dermatologic Conditions

Dermatologic conditions include ichthyosis vulgaris, X-linked ichthyosis, recalcitrant pustules, hand-foot-and-mouth disease, lichen planus, pompholyx, some forms of psoriasis, the dermatophytoses (including tinea), and a variety of contact dermatitides.


Conditions Related to Systemic Illness

Conditions related to systemic illness include those that are part of a generalized eruption—secondary syphilis (see Fig. 21-4),
Reiter syndrome, rare cases of disseminated gonococcemia, erythema multiforme (from any of its causes), and roseola—and arsenical and other hyperkeratoses, which are confined to the palms and soles. Palmar keratoses, especially when multiple, may be associated with arsenic toxicity. Skin, bladder, and lung cancer may also be associated with palmar keratoses (Cuzick et al., 1984), but here the keratoses may be few or single. (For a discussion of other keratoses, see Chapter 7.) Palmar and finger crease xanthomas are also discussed in Chapter 7.






FIGURE 24-3 Spade hand in acromegaly. (From Osborne OT. Acromegaly. In: Buck AH, ed. A Reference Handbook of the Medical Sciences. Vol. I. New York: William Wood and Company; 1900;86-97, with permission.)

Hyperpigmented macules and patches of the palms were present in 35% of black adults and in more than 60% of those over the age of 65 (McDonald and Kelly, 1987).

Another diagnosis that can be made by shaking hands with the patient is hyperthyroidism. Dr Eugene Robin of California correctly diagnosed apathetic thyrotoxicosis in an elderly man who had orthopnea and warm moist palms.


Palmar Dermatoglyphs

In patients with Down syndrome, one may see a bilateral simian crease (Fig. 24-4), in which the normal (two) transverse palmar lines are replaced by a single line that completely crosses the palm. However, the diagnosis of Down syndrome should never be based on this finding in isolation (see Chapters 9 and 10 for more on Down syndrome).

If both transverse lines are maintained, but the proximal one completely crosses the palm, it is referred to as a Sydney crease (Fig. 24-5). It indicates an increased propensity to develop granulocytic leukemia. It is also seen in Down syndrome and congenital rubella (Wertelecki, 1979). It was first described by Purvis-Smith of Australia and shown to me by Dr W. Wertelecki of Alabama.






FIGURE 24-4 A simian crease.

In ichthyosis vulgaris, there are extra vertical palm creases between the thenar and hypothenar eminences (Fig. 24-6).


The Dupuytren1 Contracture

This condition may affect one or more fingers, most commonly the ring finger, almost always sparing the index finger and thumb (Fig. 24-7). It is caused by a stenosing tenosynovitis or fibrosis of the palmar tendons. During maximum attempted extension of the
fingers, the afflicted fingers may be held in flexion. Sometimes the tendons can be felt better than seen.






FIGURE 24-5 A Sydney crease.

The Dupuytren contracture especially occurs in older, white male patients who smoke cigarettes and have a family history of Dupuytren contracture (see Tables 24.5 and 24.6 for associations).

Aug 10, 2020 | Posted by in GENERAL & FAMILY MEDICINE | Comments Off on The Extremities
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