British Journal of Dermatology (1976) 94,85.
Pneumatosis cystoides intestinalis in systemic sclerosis D.M.WILLIAMSON AND L.C.BELL Departments of Dermatology and General Surgery, The General Infirmary, Pontefract, Yorkshire Accepted for publication 9 April 1975
SUMMARY
Rieumatosis cvstoides intestinalis (VCV) is a rare condition characterized by the presence of multiple gas contammg thin walled cysts in the intestinal wall and mesentery. It is sometimes associated with chronic obstructive pulmonary disease, but has been described most often in patients with gastrointestinal disorders including duodenal and gastric ulceration, small bowel obstruction, regional enteritis and gastrointestinal malignancy. Its association in the patient described below with severe systemic sclerosis is of particular interest. There have been occasional previous reports of this association which should be considered in any patient with systemic sclerosis who develops abdominal symptoms suggestive of acute or sub-acute intestinal obstruction, not readily explicable by other causes. CASE REPORT
Mrs LK., aged 56 years, started with symptoms suggestive of Raynaud's phenomenon affecting tbe hands at the age of 24 years, for which she had a bilateral cervical sympathectomy at the age of 34. During tbe past 15 years she bad noted increasing flexion deformity of the fingers of both hands witb recurrent ulceration over metacarpophalangeal and interpbalangeal joints, with tbe extrusion of white calcareous material. She bad also noticed tightness of the skin of ber face and forehead, and difficulty in fitting her dentures into tbe mouth. Further symptoms included a 15 years history of upper retrosternal dyspbagia with regurgitation. She bad also developed small persistent ulcers over tbe medial malleoli and over pressure areas on tbe feet. Sbe complained of arthralgia but no muscular creaking. Bowel action bad been regular with light-coloured motions and occasional diarrhoea. Her appetite bad remained fairly good but sbe bad had a gradual loss of weight to 5 st 10 lb. There was no abnormality of micturition. No family history was obtainable as she bad been an adopted child. On 25.6.73 sbe was seen at her bome by one of us (L.C.B.) witb a history of abdominal distension and generalized intermittent colicky abdominal pain for 2 weeks. There had been occasional vomiting at tbe onset and sbe bad noticed increasing but not absolute constipation and difficulty in passing flatus. She had had no diarrhoea or passage of slime or blood per rectum. Examination showed an emaciated female witb generalized pigmentation and clinical anaemia. Tbere was no obvious dehydration. Sbe had numerous small 'mat' telangiectases of tbe face and forehead, pronounced beaking of tbe nose and tightness of tbe skin of tbe face and forehead with a small moutb opening and angular furrowing. The fingers were beld in fixed flexion and were 85
86
D.M. Williamson and L.C. Bell
thin and atrophic with marked beaking of the nails and loss of pulp tissue, together with small areas of calcinosis cuds over the interphalangeal and metacarpophalangeal joints. There was generalized distension of the abdomen with visible peristalsis. There were visible superficial veins and marked tympanites on percussion. No mass was palpable and no fluid thrill or shifting dullness could be elicited. On auscultation the bowel sounds were increased whilst rectal examination showed no abnormality other than an empty rectum. A diagnosis of systemic sclerosis with subacute intestinal obstruction was made and she was admitted to hospital for observation. A plain radiograph of the abdomen showed the presence of subphrenic gas suggesting an intestinal perforation and there was also generalized gaseous distension of both large and small bowel. A laparotomy via a right paramedian incision revealed odourless free gas in the abdomen with a little free clear fluid. There were multiple tiny aerocysts in the wall of most of the small intestine but no definite perforation was seen nor was there any apparent obstruction. The abdomen was closed in layers and the wound healed well. She was discharged home on 5.7.73. She was re-admitted on 26.7.73 following a recurrence of the same symptoms which on this occasion resolved with expectant treatment. She later had further investigations of small bowel function to exclude hypocalcaemia and hypokalaemia as a cause of intestinal atony. Investigations
Normal or negative. Serum electrolytes, phosphorus, magnesium, B 12, red cell folate, urea, aldolase. Antinuclear factor, faecal fat excretion, liver function tests. Schilling Test, creatinine clearance test. White cell count and differential. Abnormal. Hb ii-2 g/ioo ml. ESR 24 mm in ist h. Serum calcium 8-i mg/ioo ml. Serum iron 45 figjioo ml. Serum protein, total 6-8 g/ioo ml; albumin, 3-2 g/ioo ml; globulin, 3-6 g/ioo ml; electrophoresis showed increased gammaglobulin. Serum immunoglobolins IgA 380, IgG more than 2000, IgM 105 mg/ioo ml. D.A.T. positive i in 20. MSU viable count greater than 70,000/mm^. ECG, low voltage in all waves with right axis deviation. Chest X-ray showed 'some increase in basal lung markings with no active lung disease. Multiple calcified lesions in both upper zones and a small amount of free gas beneath right dome of the diaphragm.' She was treated with a total dose infusion of iron, intravenous Parenterovite, a single IM injection of vitamin D, and calcium gluconate and magnesium. She was also given Vivanex (an elemental diet) daily in view of her very poor nutritional state. Since discharge from hospital she has had no further abdominal symptoms and at her last review on 29.8.74 there was only slight distension of the abdomen with some increased resonance on percussion.
DISCUSSION
Pneumatosis cystoides intestinalis (PCI) was first described by Du Vernoi in 1730 and later in England by John Hunter in the intestines of a hog. It is an uncommon condition characterized by the presence of numerous gas containing 'cysts' in the intestinal wall. The small intestine and colon particularly in the caecal region are the areas usually involved. Gas cysts may also be present in the mesentery and retroperitoneal tissues. In an extensive review of 213 cases, Koss (1952) found the small intestine to be affected in 63% and the colon in 22%. The cysts vary in size from a pin head to several cm and contain variable mixtures of gases on analy-
Pneumatosis cystoides intestinalis in systemic sclerosis sis. Usually the oxygen tension is decreased and there is an increased concentration of carbon dioxide up to 15%. Hydrogen is sometimes found, presumably produced by intraluminal bacterial activity. Spontaneous rupture of cysts gives rise to pneumoperitoneum which may become recurrent or chronic. PCI is frequently associated with obstructive pulmonary diseases but has been described most often in patients with gastrointestinal disorders, including gastric and duodenal ulcer, small bowel obstruction, regional enteritis and malignant disease of the intestinal tract. In the past, few sporadic reports have noted an association with systemic sclerosis (Meihoff, Hirschfield & Kern, 1968; Gompels, 1969; Miercourt & Merrill, 1969). All these patients including the patient described in this report had obvious systemic sclerosis but only two of three cases reported by Meihoff et al. (1968) had obvious skin changes at the onset of the intestinal symptoms. A dietary deficient state has been postulated as a cause of the condition but so far no sound experimental evidence has supported this theory. Naeslund (1924) suggested that gas-forming organisms in the gut might be the cause of PCI, as by analogy a similar condition occurs in swine, although in man, pneumoperitoneum secondary to PCI giving rise to peritonitis is virtually unknown. Mechanical pressure may cause gas to pass from the lumen of the bowel into the lymphatics and could account for the association with peptic ulcer, especially if accompanied by pyloric stenosis. Here gas may be formed through an ulcerated area and track interstitially within the bowel wall. Keating et al. (1961) have shown that in animals air can track from the mediastinum to the root of the mesentery and suggested that rupture of pulmonary alveoli in patients with bronchial asthma and emphysema may produce PCI by this route. The radiological diagnosis of PCI may be difficult since the recognition of abdominal gas shadows within the intestinal wall from normal gas in the lumen may not be possible. Occasionally the gas is seen in the form of a linear stripe or a double walled ring-like shadow which is seen when the lumen is viewed end on. Gas streaks may also be seen in the retroperitoneal tissues and rarely in the mediastinum. Rupture of the intestinal cysts gives rise to pneumoperitoneum which is invariably sterile. The clinical picture is usually overshadowed by the presence of pyloric obstruction and the existence of cysts first revealed at operation, although a plain X-ray of the abdomen may show some of the above changes. Indecisive abdominal symptoms may be a feature, including vague generalized cohcky abdominal pain, diarrhoea with mucus and excessive flatus, some of the symptoms being suggestive of a malabsorption state and indeed malabsorption secondary to pneumatosis of the small bowel has been described by Yunich & Fradkin (1958). In systemic sclerosis there is involvement of both the respiratory system and the gastrointestinal tract. In the duodenum, jejunum, ileum and colon there is a patchy disappearance of muscle especially in the circular layer with replacement by fibrous tissue. The subserosa and submucosal tissue may also be thickened and fibrosed, the vessels in these areas showing sclerosis and intimal fibrosis. Likewise in the lungs there may be progressive diffuse alveolar fibrosis with obliteration of capillaries and alveolar spaces. Rupture of thickened alveolar walls may give rise to thin-walled gas containing cysts. It is possible therefore that PCI in systemic sclerosis may have a similar causation to PCI described with respiratory disorders, with rupture of distended pulmonary alveolar cysts, the gas tracking through the diaphragm and ultimately to the mesentery. It is, however, more likely that the gas cysts are formed locally in the intestinal wall consequent upon the local ischaemic changes, the cysts then rupturing to give rise to pneumoperitoneum. Rowell (1972) supports this theory of causation. In systemic sclerosis there is also a chronic partial intestinal obstruction associated with changes outlined above, although the mucous membrane itself often appears normal on histology. This obstruction allows massive bacterial contamination to occur in the proximal part of the small bowel producing a 'blind loop' syndrome leading at times to malabsorption.
87
88
D.M. Williamson and L.C. Bell
Extensive investigation in this patient in spite of gross wasting did not show any evidence of biochemical malabsorption except perhaps for the low serum calcium and iron. It has been suggested (Meihoff et al., 1968) that the occurrence of PCI in systemic sclerosis is of ominous prognostic significance in contrast to PCI occurring with other conditions. White, Treece & Juniper (1970) suggested that this gloomy prognosis is not invariable and described one patient who survived 6^ years. Our patient has had two episodes of PCI and has had no recurrence of symptoms up to July 1975. Recently Forgacs, Wright & Wyatt (1973) suggested that by altering the balance between diffusion of gases in and out of cysts, by lowering the total pressure of gases in the venous blood, it should be possible to 'degas' the cysts. This they achieved by allowing the patient to breath oxygen in a 75% gas mixture with nitrogen; as the administration had to be continuous, a heat tent was used continuously for 6 days and was apparently well tolerated. Cotterill (1974) has used this form of treatment with good results in a patient with pulmonary emphysema and a large solitary aerocyst of the iliac colon. From the case reported above and from other similar sporadic cases reported in the literature it is suggested that this condition should be considered in any patient with systemic sclerosis who develops symptoms of acute or subacute intestinal obstruction not readily explicable by other causes. If the diagnosis can be confirmed with a plain X-ray of the abdomen it may be possible to avoid laparotomy, the condition being treated on conservative lines. ACKNOWLEDGMENTS
We are indebted to Dr A.K.Sarma, Consultant Radiologist, Dr A.M.Fraser, Consultant Radiologist, Dr J.M.Wharton, Consultant Gastroenterologist, for their help in the management of this case, and Mrs D.Murphy, Postgraduate Secretary, for secretarial help. REFERENCES COTTERILL, J.A. (r974) Personal communication. Du VERNOI, J . G . (r730) Observations of the Anatomical Academy of Science Peropol, 5, 213. FORGACS, P., WRIGHT, P . H . & WYATT, A.P. (1973) Treatment of intestinal gas cysts by oxygen breathing. Lancet, i, 579GoMPELS, B.M. (r969) Pneumatosis cystoides intestinalis associated with progressive systemic sclerosis. British Journal of Radiology, 42,70 r. KEATING, W.S., MCCARVER, R.R., KOVARIK, J . K . & DAYWITH, A. (1961) Pneumatosis intestinalis, a new concept. Radiology,•]6,'JJ,^.
Koss, L.G. (1952) Abdominal gas cysts: analysis with report of a case and critical review of literature. Archives of Pathology, 53,523. MEIHOFF, W.E., HIRSCHFIELD, J . S . & KERN, F . (1968) Small intestinal scleroderma with malabsorption and pneumatic cystoides intestinalis. A report of three ca&e%. Journal of American Medical Association, 204,102. MEIRCORT, R . D . & MERRILL, F . G . (1969) Pneumatosis and pseudo-obstruction in scleroderma. Radiology, 92, 359. NAESLUND, J. (1924) Zur Kenntnis der Pneumatosis Cystoides Intestinalis. M.D. Thesis. Uppsalla Mareus, Sweden. ROWELL, N . R . (1972) Lupus erythematosus, scleroderma and dermatomyositis. In: Textbook of Dermatology (Ed. by A. J.Rook, D. S.Wilkinson and F.J. Ebling), Vol. 2, p. 1112. Blackwell Scientific Publications, Oxford. WHITE, W.D., TREECE, T.R. & JUNIPER, K . (1970) In letter to Editor (pneumatosis in scleroderma of small bowel). Journal of American Medical Association, 212, ro68. YUNICH, A.M. & FRADKIN, N . F . (1958) Malabsorption syndrome secondary to extensive pneumatosis cystoides intestinalis. Gastroenterology, 35,212.
Pneumatosis cystoides intestinalis in systemic sclerosis D.M.WILLIAMSON AND L.C.BELL Departments of Dermatology and General Surgery, The General Infirmary, Pontefract, Yorkshire Accepted for publication 9 April 1975
SUMMARY
Rieumatosis cvstoides intestinalis (VCV) is a rare condition characterized by the presence of multiple gas contammg thin walled cysts in the intestinal wall and mesentery. It is sometimes associated with chronic obstructive pulmonary disease, but has been described most often in patients with gastrointestinal disorders including duodenal and gastric ulceration, small bowel obstruction, regional enteritis and gastrointestinal malignancy. Its association in the patient described below with severe systemic sclerosis is of particular interest. There have been occasional previous reports of this association which should be considered in any patient with systemic sclerosis who develops abdominal symptoms suggestive of acute or sub-acute intestinal obstruction, not readily explicable by other causes. CASE REPORT
Mrs LK., aged 56 years, started with symptoms suggestive of Raynaud's phenomenon affecting tbe hands at the age of 24 years, for which she had a bilateral cervical sympathectomy at the age of 34. During tbe past 15 years she bad noted increasing flexion deformity of the fingers of both hands witb recurrent ulceration over metacarpophalangeal and interpbalangeal joints, with tbe extrusion of white calcareous material. She bad also noticed tightness of the skin of ber face and forehead, and difficulty in fitting her dentures into tbe mouth. Further symptoms included a 15 years history of upper retrosternal dyspbagia with regurgitation. She bad also developed small persistent ulcers over tbe medial malleoli and over pressure areas on tbe feet. Sbe complained of arthralgia but no muscular creaking. Bowel action bad been regular with light-coloured motions and occasional diarrhoea. Her appetite bad remained fairly good but sbe bad had a gradual loss of weight to 5 st 10 lb. There was no abnormality of micturition. No family history was obtainable as she bad been an adopted child. On 25.6.73 sbe was seen at her bome by one of us (L.C.B.) witb a history of abdominal distension and generalized intermittent colicky abdominal pain for 2 weeks. There had been occasional vomiting at tbe onset and sbe bad noticed increasing but not absolute constipation and difficulty in passing flatus. She had had no diarrhoea or passage of slime or blood per rectum. Examination showed an emaciated female witb generalized pigmentation and clinical anaemia. Tbere was no obvious dehydration. Sbe had numerous small 'mat' telangiectases of tbe face and forehead, pronounced beaking of tbe nose and tightness of tbe skin of tbe face and forehead with a small moutb opening and angular furrowing. The fingers were beld in fixed flexion and were 85
86
D.M. Williamson and L.C. Bell
thin and atrophic with marked beaking of the nails and loss of pulp tissue, together with small areas of calcinosis cuds over the interphalangeal and metacarpophalangeal joints. There was generalized distension of the abdomen with visible peristalsis. There were visible superficial veins and marked tympanites on percussion. No mass was palpable and no fluid thrill or shifting dullness could be elicited. On auscultation the bowel sounds were increased whilst rectal examination showed no abnormality other than an empty rectum. A diagnosis of systemic sclerosis with subacute intestinal obstruction was made and she was admitted to hospital for observation. A plain radiograph of the abdomen showed the presence of subphrenic gas suggesting an intestinal perforation and there was also generalized gaseous distension of both large and small bowel. A laparotomy via a right paramedian incision revealed odourless free gas in the abdomen with a little free clear fluid. There were multiple tiny aerocysts in the wall of most of the small intestine but no definite perforation was seen nor was there any apparent obstruction. The abdomen was closed in layers and the wound healed well. She was discharged home on 5.7.73. She was re-admitted on 26.7.73 following a recurrence of the same symptoms which on this occasion resolved with expectant treatment. She later had further investigations of small bowel function to exclude hypocalcaemia and hypokalaemia as a cause of intestinal atony. Investigations
Normal or negative. Serum electrolytes, phosphorus, magnesium, B 12, red cell folate, urea, aldolase. Antinuclear factor, faecal fat excretion, liver function tests. Schilling Test, creatinine clearance test. White cell count and differential. Abnormal. Hb ii-2 g/ioo ml. ESR 24 mm in ist h. Serum calcium 8-i mg/ioo ml. Serum iron 45 figjioo ml. Serum protein, total 6-8 g/ioo ml; albumin, 3-2 g/ioo ml; globulin, 3-6 g/ioo ml; electrophoresis showed increased gammaglobulin. Serum immunoglobolins IgA 380, IgG more than 2000, IgM 105 mg/ioo ml. D.A.T. positive i in 20. MSU viable count greater than 70,000/mm^. ECG, low voltage in all waves with right axis deviation. Chest X-ray showed 'some increase in basal lung markings with no active lung disease. Multiple calcified lesions in both upper zones and a small amount of free gas beneath right dome of the diaphragm.' She was treated with a total dose infusion of iron, intravenous Parenterovite, a single IM injection of vitamin D, and calcium gluconate and magnesium. She was also given Vivanex (an elemental diet) daily in view of her very poor nutritional state. Since discharge from hospital she has had no further abdominal symptoms and at her last review on 29.8.74 there was only slight distension of the abdomen with some increased resonance on percussion.
DISCUSSION
Pneumatosis cystoides intestinalis (PCI) was first described by Du Vernoi in 1730 and later in England by John Hunter in the intestines of a hog. It is an uncommon condition characterized by the presence of numerous gas containing 'cysts' in the intestinal wall. The small intestine and colon particularly in the caecal region are the areas usually involved. Gas cysts may also be present in the mesentery and retroperitoneal tissues. In an extensive review of 213 cases, Koss (1952) found the small intestine to be affected in 63% and the colon in 22%. The cysts vary in size from a pin head to several cm and contain variable mixtures of gases on analy-
Pneumatosis cystoides intestinalis in systemic sclerosis sis. Usually the oxygen tension is decreased and there is an increased concentration of carbon dioxide up to 15%. Hydrogen is sometimes found, presumably produced by intraluminal bacterial activity. Spontaneous rupture of cysts gives rise to pneumoperitoneum which may become recurrent or chronic. PCI is frequently associated with obstructive pulmonary diseases but has been described most often in patients with gastrointestinal disorders, including gastric and duodenal ulcer, small bowel obstruction, regional enteritis and malignant disease of the intestinal tract. In the past, few sporadic reports have noted an association with systemic sclerosis (Meihoff, Hirschfield & Kern, 1968; Gompels, 1969; Miercourt & Merrill, 1969). All these patients including the patient described in this report had obvious systemic sclerosis but only two of three cases reported by Meihoff et al. (1968) had obvious skin changes at the onset of the intestinal symptoms. A dietary deficient state has been postulated as a cause of the condition but so far no sound experimental evidence has supported this theory. Naeslund (1924) suggested that gas-forming organisms in the gut might be the cause of PCI, as by analogy a similar condition occurs in swine, although in man, pneumoperitoneum secondary to PCI giving rise to peritonitis is virtually unknown. Mechanical pressure may cause gas to pass from the lumen of the bowel into the lymphatics and could account for the association with peptic ulcer, especially if accompanied by pyloric stenosis. Here gas may be formed through an ulcerated area and track interstitially within the bowel wall. Keating et al. (1961) have shown that in animals air can track from the mediastinum to the root of the mesentery and suggested that rupture of pulmonary alveoli in patients with bronchial asthma and emphysema may produce PCI by this route. The radiological diagnosis of PCI may be difficult since the recognition of abdominal gas shadows within the intestinal wall from normal gas in the lumen may not be possible. Occasionally the gas is seen in the form of a linear stripe or a double walled ring-like shadow which is seen when the lumen is viewed end on. Gas streaks may also be seen in the retroperitoneal tissues and rarely in the mediastinum. Rupture of the intestinal cysts gives rise to pneumoperitoneum which is invariably sterile. The clinical picture is usually overshadowed by the presence of pyloric obstruction and the existence of cysts first revealed at operation, although a plain X-ray of the abdomen may show some of the above changes. Indecisive abdominal symptoms may be a feature, including vague generalized cohcky abdominal pain, diarrhoea with mucus and excessive flatus, some of the symptoms being suggestive of a malabsorption state and indeed malabsorption secondary to pneumatosis of the small bowel has been described by Yunich & Fradkin (1958). In systemic sclerosis there is involvement of both the respiratory system and the gastrointestinal tract. In the duodenum, jejunum, ileum and colon there is a patchy disappearance of muscle especially in the circular layer with replacement by fibrous tissue. The subserosa and submucosal tissue may also be thickened and fibrosed, the vessels in these areas showing sclerosis and intimal fibrosis. Likewise in the lungs there may be progressive diffuse alveolar fibrosis with obliteration of capillaries and alveolar spaces. Rupture of thickened alveolar walls may give rise to thin-walled gas containing cysts. It is possible therefore that PCI in systemic sclerosis may have a similar causation to PCI described with respiratory disorders, with rupture of distended pulmonary alveolar cysts, the gas tracking through the diaphragm and ultimately to the mesentery. It is, however, more likely that the gas cysts are formed locally in the intestinal wall consequent upon the local ischaemic changes, the cysts then rupturing to give rise to pneumoperitoneum. Rowell (1972) supports this theory of causation. In systemic sclerosis there is also a chronic partial intestinal obstruction associated with changes outlined above, although the mucous membrane itself often appears normal on histology. This obstruction allows massive bacterial contamination to occur in the proximal part of the small bowel producing a 'blind loop' syndrome leading at times to malabsorption.
87
88
D.M. Williamson and L.C. Bell
Extensive investigation in this patient in spite of gross wasting did not show any evidence of biochemical malabsorption except perhaps for the low serum calcium and iron. It has been suggested (Meihoff et al., 1968) that the occurrence of PCI in systemic sclerosis is of ominous prognostic significance in contrast to PCI occurring with other conditions. White, Treece & Juniper (1970) suggested that this gloomy prognosis is not invariable and described one patient who survived 6^ years. Our patient has had two episodes of PCI and has had no recurrence of symptoms up to July 1975. Recently Forgacs, Wright & Wyatt (1973) suggested that by altering the balance between diffusion of gases in and out of cysts, by lowering the total pressure of gases in the venous blood, it should be possible to 'degas' the cysts. This they achieved by allowing the patient to breath oxygen in a 75% gas mixture with nitrogen; as the administration had to be continuous, a heat tent was used continuously for 6 days and was apparently well tolerated. Cotterill (1974) has used this form of treatment with good results in a patient with pulmonary emphysema and a large solitary aerocyst of the iliac colon. From the case reported above and from other similar sporadic cases reported in the literature it is suggested that this condition should be considered in any patient with systemic sclerosis who develops symptoms of acute or subacute intestinal obstruction not readily explicable by other causes. If the diagnosis can be confirmed with a plain X-ray of the abdomen it may be possible to avoid laparotomy, the condition being treated on conservative lines. ACKNOWLEDGMENTS
We are indebted to Dr A.K.Sarma, Consultant Radiologist, Dr A.M.Fraser, Consultant Radiologist, Dr J.M.Wharton, Consultant Gastroenterologist, for their help in the management of this case, and Mrs D.Murphy, Postgraduate Secretary, for secretarial help. REFERENCES COTTERILL, J.A. (r974) Personal communication. Du VERNOI, J . G . (r730) Observations of the Anatomical Academy of Science Peropol, 5, 213. FORGACS, P., WRIGHT, P . H . & WYATT, A.P. (1973) Treatment of intestinal gas cysts by oxygen breathing. Lancet, i, 579GoMPELS, B.M. (r969) Pneumatosis cystoides intestinalis associated with progressive systemic sclerosis. British Journal of Radiology, 42,70 r. KEATING, W.S., MCCARVER, R.R., KOVARIK, J . K . & DAYWITH, A. (1961) Pneumatosis intestinalis, a new concept. Radiology,•]6,'JJ,^.
Koss, L.G. (1952) Abdominal gas cysts: analysis with report of a case and critical review of literature. Archives of Pathology, 53,523. MEIHOFF, W.E., HIRSCHFIELD, J . S . & KERN, F . (1968) Small intestinal scleroderma with malabsorption and pneumatic cystoides intestinalis. A report of three ca&e%. Journal of American Medical Association, 204,102. MEIRCORT, R . D . & MERRILL, F . G . (1969) Pneumatosis and pseudo-obstruction in scleroderma. Radiology, 92, 359. NAESLUND, J. (1924) Zur Kenntnis der Pneumatosis Cystoides Intestinalis. M.D. Thesis. Uppsalla Mareus, Sweden. ROWELL, N . R . (1972) Lupus erythematosus, scleroderma and dermatomyositis. In: Textbook of Dermatology (Ed. by A. J.Rook, D. S.Wilkinson and F.J. Ebling), Vol. 2, p. 1112. Blackwell Scientific Publications, Oxford. WHITE, W.D., TREECE, T.R. & JUNIPER, K . (1970) In letter to Editor (pneumatosis in scleroderma of small bowel). Journal of American Medical Association, 212, ro68. YUNICH, A.M. & FRADKIN, N . F . (1958) Malabsorption syndrome secondary to extensive pneumatosis cystoides intestinalis. Gastroenterology, 35,212.
