999 Annals ofthe Rhewnatic Disases 1990; 49:999-1000 CASE REPORTS Sparing effect of hemiplegia on scleroderma Sanjay Sethi, Winston Sequeira Abstract The paretic limb is spared in patients who develop rheumatic diseases after a hemiplegic stroke. This has been described previously in rheumatoid arthritis, gout, and osteoarthritis. A similar presentation in a case of scleroderma is described in this report. Scleroderma skin changes are absent in the completely paretic limb and were markedly reduced in the weak left leg. Inflammationl may be modified either by neuropeptides or by an anatomical neurological lesion and this may explain the phenomenon. bodies to SS-A, SS-B, Sm, RNP, and dsDNA negative; erythrocyte sedimentation rate (Wintrobe) 40 mm/h; C3 115 mg/l (normal range 83-170); C4 38 mg/l (normal range 20-50). The chest radiograph was normal with a restrictive pattern shown on pulmonary function tests. The patient was diagnosed as having scleroderma, though at no stage in her disease was her left arm affected. Treatment was started with penicillamine. Discussion Scleroderma is an autoimmune disease characAn unusual case of scleroderma is presented terised by diffuse symmetrical involvement of here in a woman who had had an antecedent the skin with the classical changes of hidebound stroke with scleroderma sparing the paretic left skin extending proximal to the metacarpophalangeal joints.' We believe that this is an side. unusual case of scleroderma, which spared the side affected by the stroke. The degree to which skin is affected is inversely proportional to the Case report A 56 year old black woman was first examined degree of weakness. The paretic left arm is the in the rheumatology clinic in 1986 for changes least affected, skin changes are mild in the weak in skin colour and a 'petechial rash'. She had left leg with diffuse involvement of the normal also complained of cold induced, triple colour limbs. This sparing of the paretic limb has not changes classical for Raynaud's phenomenon in been described in scleroderma, though it has her right hand without similar changes occurring been noted in rheumatoid arthritis.2 3 Inflamin her left hand. The patient noticed dyspnoea mation, erosions, and nodules are uncommon on moderate exertion and mild dysphagia with on the affected side, and this sparing of the paretic limb occurs only if the stroke precedes solid food. She had had a stroke in May 1967 as a the arthritis.2 3 Acute attacks of gout4 and consequence of a ruptured right posterior com- Heberden's nodes5 are also less prevalent in municating artery aneurysm, which was subse- paretic limbs. Why this happens is not clear but quently clipped. She continued to have residual certainly poses a number of questions. Why are paralysis of her left hand and weakness in her some diseases bilateral and symmetrical? Does left leg and had received little or no physical the nervous system have a role in causing or treatment. modifying these diseases? Might injury to the On physical exmination the patient had nervous system prevent the development of areas of hypopigmentation and hyperpigmenta- bilateral disease? Might this be a clue to the tion with prominent telangiectasia on her face pathogenesis of scleroderma and other rheumatic and anterior chest wall. She had generalised diseases? Decreased use of the paretic limb or a sclerodermatous skin changes that spared the left spastic and paretic hand (figure), and the modification in its blood flow might be responweak left leg was minimally affected. Passive sible for the sparing effect described above. motion of the arm and hand was markedly There is no evidence that resting a neurologically Department of Medicine, limited because of severe contractures. She had intact limb prevents progression of rheumatoid Cook County Hospital, 3/5 motor power in her left leg and foot; her arthritis, however. Decreased skin blood flow, Chicago, Illinois, USA S Sethi deep tendon reflexes were exaggerated in the as measured by phenolsulphonphthalein clearleft hand and foot. There was no sensory deficit ance time, has been noted in paretic limbs,6 but Division of Rheumatology, and the other results of her systemic examina- its pathophysiological significance is uncertain. Cook County Hospital, tion were normal. Modification of inflammation by neurological Chicago, lilinois, USA Her laboratory tests including a complete lesions has been shown experimentally. Section W Sequeira blood count, serum electrolytes, liver profile, of the sciatic nerve before induction of adjuvant Sequeira, and urine analysis were normal. Immunological arthritis in rats causes sparing of the paretic Polkc Chicago, Illinois 60612, USA studies were as follows: antinuclear antibody limb.' The flare component ofthe triple response titre 1/1280 with a nucleolar pattern; rheumatoid of Lewis is diminished significantly after neuroAccepted for publication 26 January 1990 factor <60 units; anti-Sc1-70 positive; anti- logical lesions (both central and peripheral).8 Correspondence to: Dr Winston 1900 West Street, Sethi, Squeira 4 . i._. .i ... X; I f. .ir (A) Right hand showing sckerodermatous skin changes; (B) paretic left hand with normal appeanng skin. More recently, the release of substance P and other neuropeptides from the terminal ends of sensory nerves has been shown to have an important role in mediating inflammatory responses.9 These observations suggest that interruption of neural pathways may modify the inflammatory and vascular responses seen in the affected limb: both considered to be key factors in the pathogenesis of scleroderma. 10 This may account for the unusual distribution of sclerodermatous changes seen in our patient. Recent reports have considered the possibility of the autonomic nervous system playing a part in the pathogenesis ofscleroderma. " Autonomic dysfunction induced by the stroke may have had an ameliorating effect in our patient.'2 1 Subcommittee for scderoderma criteria of the American Rheumatism Association diagnostic and therapeutic criteria committee. Preliminary criteria for the classification of systemic sclerosis (scieroderma). Arthriis Rheum 1980; 23: 581-90. 2 Thomason M, Bywater E G C. Unilateral rheumatoid arthritis following hemiplegia. Ann Rheum Dis 1%2; 21: 370-7. 3 Glick E N. Asymmetrical rheumatoid arthritis following polio. Br Med 1967; ii: 26-9. 4 Glyn j J, Clayton M L. Sparing effect of hemiplegia on tophaceous gout. Ann Rheum Dis 1976; 35: 534-5. 5 Winter S. Unilateral Heberden nodes in a case of hemiplegia. NY State Med 1952; 52: 349-50. 6 Mizushima Y, Yamaura M. Arthropathy and inflammatory reaction in hemiplegic patients. Acta Rheumatologica Scandinavica 1969; 15: 297-304. 7 Courtright L J, Kuzell W C. Sparing effect of neurological deficit and trauma on the course of adjuvant arthritis in rats. Ann Rheun Dis 1965; 24: 360-6. 8 Cooper I S. A neurological evaluation of the cutaneous histamine reaction. J Clin Invest 1950; 29: 465-9. 9 Levine J D, Collier D H, Bassaum A I, Maskowitz A, Helms L A. Hypothesis: the nervous system may contribute to the pathophysiology in rheumatoid arthritis. J Rheumatol 1985; 12: 406-11. 10 Stemnberg E M. Pathogenesis of scleroderma: the interrlationship of the immune and vascular hypotheses. Survy of Immunological Research 1985; 4: 69-80. 11 Klimiuk P 5, Taylor L, Baker R D, Jayson M I V. Autonomic neuropathy in systemic sclerosis. Am Rhewn Dis 1988; 47: 5425. 12 Eto F, Yoshikawa M, Ueda S, Hirai S. Posthemiplegic shoulder-hand syndrome, with special reference to related cerebral localisation. J Am Geriatr Soc 1980; 28: 13-17.