808 Proc. roy. Soc. Med. Volume 69 November 1976 Ptogressive Systemic Sclerosis and Polymyositis A M Silas MB DPhysMed (for A G White MRCP DPhysMed) (Department of Rheumatology and Rehabilitation, Whittington Hospital, London N19 3UA) Mrs L H, aged 51. Waitress History: In early 1974 she developed Raynaud's phenomenon with parnsthesix of hands and feet occurring even in warm weather. In January 1975 she found difficulty in swallowing solid food; care was needed even with mincemeat to avoid choking. She lost 9.5 kg in weight but she did not vomit. Simultaneously, the skin of the face swelled and became tight. General weakness disabled her progressively until she could no longer rise from her chair and she had to give up work. The neck and arm joints were -painful and stiff on both sides, but indomethacin (25 mg three times daily) relieved the symptoms. Four months ago she also developed paroxysms of palpitation without chest pain, and shortness of breath. Bowel actions remained normal. Past history: 1957: Right-sided pulmonary tuberculosis was successfully treated with streptomycin and Pasinah. 1971: Thyrotoxicosis was excluded. 1973: Extensive psoriasis responded well to Betnovate ointment.
46
with tight, atrophic and shiny facial skin with telangiectases and puckering round the mouth and a gap of only just 3 cm between upper and lower incisor teeth. Cardiovascular and respiratory systems normal. Blood pressure 130/80. Proximal muscles of upper and lower limbs on both sides weak (power 3/4) and tender with no tendon reflexes. Movements of shoulders, elbows, wrists, hands, ankles and feet on both sides restricted by pain. Breasts normal. No psoriatic lesions found.
Investigations: Barium swallow revealed prolonged stasis but no obstruLction of cesophagus. Chest X-ray suggested bilateral basal -fibrosis. Lung function: FEV1 1.4 1, FVC. 2.0 1, FEVJ/FVC 70%, DCo2 (at rest) 17.4 ml/mmHg. X-ray of haInds showed terminal tuft atrophy with calcification in soft tissues of several finfgers. Serum enzyme levels: CPK 546, aldolase 6.5, HBD 430, AST 26 iu/l. Urinary excretion of creatine 376 mg in 24 h. ANA positive 1:10
(nucleolar pattern) and anti-DNA 43% (normal 20-32%). EMG showed low voltage, short duration, highly polyphasic action- potentials in biceps, triceps and quadriceps muscles with pseudomyotonic potentials; there was much nsertional activity.
Social history: She smoked more than 30 cigarettes daily until 1973. Her youngest daughter of three children, now aged 21, was born spastic and is mentally handicapped and epileptic.
Biopsies: (1) Triceps muscle: extensive fibre necrosis with regeneration and widespread infiltration with small round inflammatory cells. (2) Adjoining skin: epidermal atrophy with loss of rete pegs of dermis.
On examination (5.2.76): Weight 66.8 kg. Classical scleroderma of skin of hands with tapering fingers and curved nails, due to pulp atrophy and collapse (Fig 1). This is associated with palmar erythema and pink discoloration of the dorsal skin of. the metacarpophalangeal and interphalangeal joints. Microstomia is present
Normal results were obtained in the following investigations: Hxemoglobin, WBC, ESR, rheumatoid antibody (SCAT), ECG, plasma urea and electrolytes, creatinine clearance, MSU, total and C3 complement, IVP, serum bilirubin, proteins and electrophoretic strip, alkaline phosphatase, gamma GT, thyroid uptake and scan.
Fig I Dorsal discoloration of interphalangealjoints
Clinical Section
47 Progress: Prednisolone therapy was begun in a dose of 10 mg three times daily on 10 March 1976 with Propaderm and emulsifying ointment for the skin. Within two weeks her muscle power and skin texture had improved till she needed no help with her personal care (Fig 2). She was discharged on 26 March 1976 on prednisolone (5 mg three times daily). At follow up on 4 May 1976 her weight had risen 5 kg and her CPK level had fallen from 272 to 233 iu/l. Comment Myopathy is known to occur in progressive systemic sclerosis (Hollander 1974) but is usually benign and clearly differentiated from true untreated polymyositis by EMG and muscle biopsy. In progressive systemic sclerosis all such investigative findings are much less severe than those in polymyositis (Table 1; Furst 1975). Our
809
...
i;:~~~~~~~~~~. ...r ..:...
-...- ... .&
..:
...f
Table 1 Contrasting features of progressive systemic sclerosis myopathy and polymyositis (Furst 1975)
Fig 2 Improved skin texture after steroids
EMG
Myopathy in progressive -systemic sclerosis Polyphasic waves of normal duration and size (85 Y.)
Biopsy
Denervation potentials (5 %) Denervation potentials (85 %), i.e. fibrillation, Normal potentials (10%) positive sharp waves, insertional irritability Mild interstitial Widespread inflammafibrosis (20%) tion (100%), i.e. degeneration, necrosis and regeneration + + 4 +
True polymyositis
Distinctive polyphasic potentials; small, short, motor units
(100%)
Weakness Enzymes
patient had sclerodermal changes and severe disabling polymyositis; both conditions responded well to steroid therapy. This and the presence of 'collodion knuckles' may suggest dermatomyositis. This may be looked upon as a spectrum of disease having end-stage sclerodermal lesions at one end and polymyositis alone at the other.
Correction The second and third sentences of the paragraph headed Investigations of Dr G M Scott's paper 'Cryptogenic Brain Abscess' (August 1976 Proceedings, p 577 should read as follows: EEG showed slow wave activity on the left side with phase reversal over the midtemporal region. Technetium brain scan showed an area of abnormal uptake in the area of the insula on the left.
The prognosis of dermatomyositis is more serious in the adult than in the young as the incidence of carcinoma increases from a level of 20% to more than 40% after the age of 50 (Huskisson & Hart 1975). So far, careful search has not revealed any such malignancy but the possibility will be kept in mind. REFERENCES Purst D E (i975) Rhewmatology News International 3, No.?7,1 Hollander'J L (i974) Arthritis and Aiiied Conditions. 8th edn. Lea & Febiger, Philadelphia, p970 Huskisson E C & Hart F D (1975) Joint Diseases: All the Arthropathies. 2nd edn. Wright, Bristol; p90
The following case was also shown:
Testosterone-induced Liver Tumour Mr G B Coombes (for Mr Ian Burn) (Ipswich Hospital, Ipswich, IP1 3PY)
46
with tight, atrophic and shiny facial skin with telangiectases and puckering round the mouth and a gap of only just 3 cm between upper and lower incisor teeth. Cardiovascular and respiratory systems normal. Blood pressure 130/80. Proximal muscles of upper and lower limbs on both sides weak (power 3/4) and tender with no tendon reflexes. Movements of shoulders, elbows, wrists, hands, ankles and feet on both sides restricted by pain. Breasts normal. No psoriatic lesions found.
Investigations: Barium swallow revealed prolonged stasis but no obstruLction of cesophagus. Chest X-ray suggested bilateral basal -fibrosis. Lung function: FEV1 1.4 1, FVC. 2.0 1, FEVJ/FVC 70%, DCo2 (at rest) 17.4 ml/mmHg. X-ray of haInds showed terminal tuft atrophy with calcification in soft tissues of several finfgers. Serum enzyme levels: CPK 546, aldolase 6.5, HBD 430, AST 26 iu/l. Urinary excretion of creatine 376 mg in 24 h. ANA positive 1:10
(nucleolar pattern) and anti-DNA 43% (normal 20-32%). EMG showed low voltage, short duration, highly polyphasic action- potentials in biceps, triceps and quadriceps muscles with pseudomyotonic potentials; there was much nsertional activity.
Social history: She smoked more than 30 cigarettes daily until 1973. Her youngest daughter of three children, now aged 21, was born spastic and is mentally handicapped and epileptic.
Biopsies: (1) Triceps muscle: extensive fibre necrosis with regeneration and widespread infiltration with small round inflammatory cells. (2) Adjoining skin: epidermal atrophy with loss of rete pegs of dermis.
On examination (5.2.76): Weight 66.8 kg. Classical scleroderma of skin of hands with tapering fingers and curved nails, due to pulp atrophy and collapse (Fig 1). This is associated with palmar erythema and pink discoloration of the dorsal skin of. the metacarpophalangeal and interphalangeal joints. Microstomia is present
Normal results were obtained in the following investigations: Hxemoglobin, WBC, ESR, rheumatoid antibody (SCAT), ECG, plasma urea and electrolytes, creatinine clearance, MSU, total and C3 complement, IVP, serum bilirubin, proteins and electrophoretic strip, alkaline phosphatase, gamma GT, thyroid uptake and scan.
Fig I Dorsal discoloration of interphalangealjoints
Clinical Section
47 Progress: Prednisolone therapy was begun in a dose of 10 mg three times daily on 10 March 1976 with Propaderm and emulsifying ointment for the skin. Within two weeks her muscle power and skin texture had improved till she needed no help with her personal care (Fig 2). She was discharged on 26 March 1976 on prednisolone (5 mg three times daily). At follow up on 4 May 1976 her weight had risen 5 kg and her CPK level had fallen from 272 to 233 iu/l. Comment Myopathy is known to occur in progressive systemic sclerosis (Hollander 1974) but is usually benign and clearly differentiated from true untreated polymyositis by EMG and muscle biopsy. In progressive systemic sclerosis all such investigative findings are much less severe than those in polymyositis (Table 1; Furst 1975). Our
809
...
i;:~~~~~~~~~~. ...r ..:...
-...- ... .&
..:
...f
Table 1 Contrasting features of progressive systemic sclerosis myopathy and polymyositis (Furst 1975)
Fig 2 Improved skin texture after steroids
EMG
Myopathy in progressive -systemic sclerosis Polyphasic waves of normal duration and size (85 Y.)
Biopsy
Denervation potentials (5 %) Denervation potentials (85 %), i.e. fibrillation, Normal potentials (10%) positive sharp waves, insertional irritability Mild interstitial Widespread inflammafibrosis (20%) tion (100%), i.e. degeneration, necrosis and regeneration + + 4 +
True polymyositis
Distinctive polyphasic potentials; small, short, motor units
(100%)
Weakness Enzymes
patient had sclerodermal changes and severe disabling polymyositis; both conditions responded well to steroid therapy. This and the presence of 'collodion knuckles' may suggest dermatomyositis. This may be looked upon as a spectrum of disease having end-stage sclerodermal lesions at one end and polymyositis alone at the other.
Correction The second and third sentences of the paragraph headed Investigations of Dr G M Scott's paper 'Cryptogenic Brain Abscess' (August 1976 Proceedings, p 577 should read as follows: EEG showed slow wave activity on the left side with phase reversal over the midtemporal region. Technetium brain scan showed an area of abnormal uptake in the area of the insula on the left.
The prognosis of dermatomyositis is more serious in the adult than in the young as the incidence of carcinoma increases from a level of 20% to more than 40% after the age of 50 (Huskisson & Hart 1975). So far, careful search has not revealed any such malignancy but the possibility will be kept in mind. REFERENCES Purst D E (i975) Rhewmatology News International 3, No.?7,1 Hollander'J L (i974) Arthritis and Aiiied Conditions. 8th edn. Lea & Febiger, Philadelphia, p970 Huskisson E C & Hart F D (1975) Joint Diseases: All the Arthropathies. 2nd edn. Wright, Bristol; p90
The following case was also shown:
Testosterone-induced Liver Tumour Mr G B Coombes (for Mr Ian Burn) (Ipswich Hospital, Ipswich, IP1 3PY)
