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Sclerodactyly and Digital Osteosclerosis Postgrad Med J: first published as 10.1136/pgmj.44.513.553 on 1 July 1968. Downloaded from Case reports 553 drain with side holes may be more effective in Acknowledgments allowing access for air than the usual corrugated My thanks are due to Dr I. Howard, Medical Superin- drain. tendent of the Alfred Hospital, Mr R. S. Lawson and Mr (4) The wound should be inspected daily. K. Bradley for permission to publish these case reports. (5) An hourly pulse chart should be kept and any sustained rise immediately reported. It is emphasized that this was the first sign in both References the above patients. BRUMMELKAMP, W.H., BOEREMA, I. & HOOGENDYK, L. (1963) Treatment of clostridial infections with hyperbaric oxygen If gas infection is suspected, vigorous treat- drenching. Lancet, i, 235. ment should be instituted: GYE, R. ROUNTREE, P.M. & LOWENTHAL, J. (1961) Infection (1) The wound should be widely opened and of surgical wounds with Clostridium welchii. Med. J. Aust. a swab taken for bacteriological examination. i, 761. HAM, J.M., MACKENZIE, D.C. & LOWENTHAL, J. (1964) The (2) Blood transfusion should be commenced immediate results of lower limb amputation for atheros- as soon as possible as these patients all have clerosis obliterans. Aust. N.Z. J. Surg. 34, 97. some degree of haemolysis. KARASEWICK, E.G., HARPER, E.M., SHARP, N.C.C., SHIELDS, (3) Penicillin should be given as above. R.S., SMITH, G. & MCDOWALL, D.G. (1964) Hyperbaric (4) Hyperbaric oxygen can be life-saving oxygen in clostridial infections. Clinical Application of Boerema Hyperbaric Oxygen (Ed. by I. Boerema). Proceedings of (Brummelkamp, & Hoogendyk, 1963) the First International Congress, 1963, pp. 36-40. Elsevier, though the salvage rate in these elderly arterio- Amsterdam. sclerotics will inevitably be disappointing. Until NAGLER, F.P.O. (1945) Treatment of experimental gas the patient can be placed in the oxygen pressure- gangrene due to Clostridium welchii with penicillin and chamber, oxygen should be given by face-mask. antitoxin. Brit. J. exp. Path. 26, 57. copyright. Primary Raynaud's disease associated with sclerodactyly and digital osteosclerosis D. N. GOLDING H. BAKER M.A., M.D., M.R.C.P.I. M.B., Ch.B., M.R.C.P. http://pmj.bmj.com/ Princess Alexandra Hospital, Harlow, and St John's Hospital for Diseases of the Skin, London THE USUAL skeletal lesion associated with primary existence of both osteosclerosis and sclerodactyly, Raynaud's disease, or with Raynaud's phenom- apparently secondary to Raynaud's disease. enon secondary to another disorder such as on October 2, 2021 by guest. Protected progressive systematic sclerosis, is digital osteopor- Case report osis affecting particularly the terminal phalanges A 45-year-old housewife stated that ever since of the hands. Rarely there may be progression to childhood she had been prone to attacks of osteolysis of the terminal phalanges associated numbness and pallor followed by blueness and with wasting of the pulp and beaking of the then redness of the hands, precipitated by cold. nails, which in systemic sclerosis may be accom- Her feet were affected to a lesser degree. The panied by ulceration of the tips of the fingers, episodes were more severe in winter, when she indolent paronychia and sometimes soft tissue regularly suffered from chilblains, but they calcification. occurred throughout the year. During adoles- Increased bone density (osteosclerosis) of the cence she had observed wasting of the ulnar metacarpals and phalanges is rarely found in three fingers of the left hand, which progressed association with Raynaud's disease, and is not for a while and then became stationary. Lately mentioned in the majority of relevant textbooks. she had noticed that the left hand was habitually The following case is of interest because of the colder and bluer than its fellow. Postgrad Med J: first published as 10.1136/pgmj.44.513.553 on 1 July 1968. Downloaded from 554 Case reports There was no family history of Raynaud's showed no evidence of denervation or muscle disease, though many members of her family fibre disorder. Motor nerve conduction velocities were prone to chilblains. in the median and ulnar nerves were within On examination, the hands were cyanosed and normal limits, but there was slight prolongation cool. There was marked wasting and slight flexion of sensory conduction time in the left ulnar deformities of the left third, fourth and fifth nerve from the little finger to the wrist (latency fingers with dryness and slight thickening of the 50 msec, normal maximum 3-5 msec). All other skin, especially on the flexor aspects of the digits. investigations were normal: Hb 90% (13-3 g/ Although the finger pulps were wasted, the over- 100 ml), WBC 6000/mm3 (normal differential), lying skin was not bound down as in systemic ESR 7 mm/hr (Westergren), urine-normal, sclerosis and could be wrinkled into folds, and blood urea 24mg/100ml, serum uric acid there was no cutaneous ulceration. The feet 3-3 mg/ 100 ml, latex and Rose-Waaler tests appeared normal. Systemic examination revealed negative, LE cells not found and test for anti- no abnormality, the arm pulses being equal and nuclear factor (ANF) negative. of normal volume. The blood pressure in the arms was 190/100mmHg and equal on both Comment sides. This patient had primary Raynaud's disease as Radiographs of the hands (see Fig. 1) showed defined by attacks of pallor and numbness of the increased bone density in the shafts of the hands progressing to cyanosis and redness phalanges of both hands, especially the middle precipitated by the cold, absence of gangrene, and terminal phalanges, this being particularly onset early in life and no evidence of systemic marked in the left ring and little fingers. There connective tissue disorder or any other disease was some degree of juxta-articular osteoporosis, (Allen & Brown, 1932). More recently sclerodac- but no resorption of terminal phalanges nor any tyly (atrophic changes in the skin, subcutaneous soft tissue calcification. Radiographs of the feet tissues and muscles) had occurred insidiously, were normal, as were those of the forearm bones. particularly affecting the ulnar three fingers ofcopyright. The chest X-ray showed no abnormality. the left hand. Electromyograms on the left and right abductor Although localized para-articular osteoporosis digiti minimi and first dorsal interosseus muscles was present, the striking bone change in the digits http://pmj.bmj.com/ on October 2, 2021 by guest. Protected FIG. 1. Radiograph of hands. Postgrad Med J: first published as 10.1136/pgmj.44.513.553 on 1 July 1968. Downloaded from Case reports 555 was osteosclerosis, affecting especially cortical ance of the left ulnar three fingers was sugges- bone and more marked in the three fingers most tive of an ulnar nerve lesion, and that there was severely affected by soft-tissue atrophic changes. additionally reduction of sensory conduction Osteosclerosis is an unusual but recognized velocity in the left ulnar nerve below the wrist, change in ischaemic digits, and arteriograms although in the presence of apparently frank would have been informative in our case, but muscle wasting other signs of neuropathy were refused by the patient. (reduced motor conduction velocity and electro- Localized scleroderma (morphea) may occas- myographic evidence of denervation of the ionally be associated with certain sclerotic bone muscles supplied) would have been expected. disorders such as melorheostosis, the soft tissue Assuming that an ulnar nerve lesion was present, change corresponding to the distribution of the it is possible to speculate that this occurred as a bone abnormality (Dillehunt & Chuinard, 1936; result of endarteritis of the vasa nervorum Muller & Henderson, 1963), but scleroderma with supplying the more distal fibres of the ulnar bound-down skin was not present in our case and nerve, associated with the long-standing the bone changes were not those characteristic Raynaud's disease. of melorheostosis, in which the disorder is usually confined to the bones of one limb and there may be severe bone pain. There was no evidence of References certain systemic conditions associated with in- ALLEN, E.V. & BROWN, G.E. (1932) Raynaud's disease: a creased bone formation, such as general- critical review of minimal requisites for diagnosis. Amer. ized osteopetrosis (Albers-Schonberg disease), J. med. Sci. 183,187. osteopoikilosis, polyostotic fibrous dysplasia, lead DILLEHUNT, R.B. & CHUINARD, E.G. (1936) Melorheostosis l6ri: a case report. J. Bone Jt Surg. 18, 991. poisoning and fluorosis. MULLER, S.A. & HENDERSON, E.D. (1963) Melorheostosis It is of some interest that the clinical appear- with linear scieroderma. Arch. Derm. 88, 142. copyright. Diabetes mellitus, Addison's disease and pernicious anaemia http://pmj.bmj.com/ R. C. TURNER ARNOLD BLOOM M.A., M.R.C.P. M.D., F.R.C.P. Whittington Hospital, London, N. 19 on October 2, 2021 by guest. Protected THESE three diseases in a patient are an unusual of obesity, raised blood pressure (160/100 mm combination. That Addison's disease and pern- Hg) and lack of buccal pigmentation. Since 1963 icious anaemia may be associated with a disorder she had lost 3 stones in weight and later exper- of autoimmune mechanisms raises the possibility ienced increasing lightheadedness and fainting. of a similar association for diabetes mellitus. The In 1965 she was given a 2-month course of evidence for this suggestion is discussed. prednisone for lichen planus. She then developed thirst and polyuria and was found to have Case history glycosuria. A 50 g glucose tolerance test whilst Mrs D.G., born 1901, developed vitiligo when on Smg prednisone/day revealed diabetes (fast- aged 28, her hair went grey aged 40. In 1956 she ing blood sugar 238mg/100ml rising to a max- first presented complaining of nervousness and imum of 270 mg, falling to 256 mg after 2 hr).
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