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LIGNEOUS CONJUNCTIVITIS
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consumption it is found to be normal.8 Such results suggest that there is no increased oxygen delivery to tissues and consequently no need to suggest that increased peripheral vascular resistance is necessary to correct for it. Although some young subjects may have a hyperkinetic circulation with increased cardiac output, this is by no means a prerequisite step in the development of hypertension and is probably a feature of centrally mediated autonomic nervous activity. As such, it could be a predictor of future hypertension. Thus, the work of Andersson and co-workers seems to indicate that the hyperkinesis is not an abnormal alarm response but fails to take us further.7 That no vascular structural changes were seen in subjects with an early increase in cardiac index could well be regarded as more evidence that the autoregulation hypothesis is found
wanting. 1. Freis ED. Haemodynamics of 2. Widimsky J, Fejfarova MH,
hypertension. Physiol Rev 1960, 40: 27-54. Fejfar Z. Changes in cardiac output in hypertensive disease. Cardiologica 1957; 31: 381-89. 3. Korner PI, Fletcher PJ. Role of the heart in causing and maintaining hypertension. Cardiovasc Med 1977; 2: 139-55. 4. Johnson PC. Autoregulation of blood flow. Circ Res 1964; 14/15 (suppl 1): 1-291.
Ledingham JM. Autoregulation in hypertension: a review. J Hypertens 1989; 7 (suppl 4): 97-104. 6. Korner PI. Causal and homeostatic factors in hypertension. Clin Sci 5.
1982; 63: S5-26. 7. Andersson OK, Beckman-Suurküla M, Sannerstedt R, Magnusson M, Sivertsson R. Does hyperkinetic circulation constitute a prehypertensive stage? A 5-year follow-up of haemodynamics in young men with mild blood pressure elevation. J Intern Med 1989; 226: 401-08. 8. Lund-Johansen P. Haemodynamics in essential hypertension. Clin Sci 1980; 59: S343-54. 9. Lund-Johansen P. Hemodynamic alterations in early essential hypertension: recent advances. In: Gross F, Strasser T, eds. Mild hypertension: recent advances. New York: Raven, 1983: 237-49.
LIGNEOUS CONJUNCTIVITIS The solution
diagnostic puzzle sometimes comes from unexpected quarters. A bleeding cervical mass, which recurred repeatedly following excision and CO2 laser ablation, was diagnosed as a component of ligneous conjunctivitisl on ophthalmological review. Disorders involving the same type of tissue in different locations not only imply a common pathological process but also give rise to
to a
difficulties in management.
Ligneous conjunctivitis is a membranous or pseudomembranous conjunctivitis that causes pronounced thickening of the upper tarsal conjunctiva; the lower tarsal and bulbar conjunctiva are less frequently involved. It is uncommon (some seventy cases have been reported) and begins in early childhood, but may persist into middle age. Autosomal recessive inheritance has been suggestedand other reports record affected siblings.34 The eye changes are often accompanied by recurrent granulomas in other mucous membranes, notably the cervixl, gingiva,s respiratory tract6 and vocal cords.’7 Hydrocephalus accompanied ligneous conjunctivitis in three reported cases.4’8 The condition takes its name from the hard hyaline submucous infiltrate which leads to conjunctival thickening by up to 3 mm. The infiltrate may abrade the cornea, resulting in ulceration, and occasionally perforation. The plaque becomes partly separated from the underlying tarsus, and is sometimes covered by a fibrinous exudate. Histologically, there is an amorphous eosinophilic mass
resembling, but staining negatively for, amyloid, with focal deposits of fibrin. There is a variable cellular component, including neutrophils, lymphocytes, eosinophils, plasma cells, macrophages, and mast cells, and foreign body inclusions are common. Immunohistochemistry shows evidence of fibrin, albumin, and immunoglobulin fragments;3 immunoglobulin staining has not been observed.6,9 The epithelium is focally degenerate, but in places becomes hypertrophic and invades the submucosa. Submucosal blood vessels show focal degeneration, with gaps between endothelial cells. Treatment has been singularly unsuccessful. Regrowth of the membrane follows surgical removal, sometimes after as little as two days 4 Thermal, electric, and silver nitrate cautery, ablation by CO2 laser, and radiotherapy, appear to be similarly fruitless. Microbiological examination has yielded a catalogue of bacteria, but these are probably secondary colonists, and neither topical nor systemic antibiotic (nor antiviral) treatment has any consistent effect.
Topical cromoglycate, steroids, idoxuridine, cytarabine, and acetylcysteine have all been proposed, tried, and failed to produce repeatable benefit. Attempts to remove the plaque enzymatically with hyaluronidase and alpha chymotrypsin were initially reported as successsful,10,11 but the results have not been reproducible.12 Irrigation with fibrinolysin, combined with cryotherapy, was effective in one reported case.13 Some success has been claimed for treatment with systemic steroids and azathioprine. 3,7 The histochemical nature of the hyaline plaque, and the vessel wall abnormalities, suggest that it is derived from plasma. This leakage may have an immunological basis, although the cause of focal damage to the endothelium of the mucosal blood vessels is obscure. The initiating insult may be local (exposure of ocular and respiratory or genital mucous membrane to a common factor), or the condition may represent a multifocal response to a systemic process. Prevention of recurrence by systemic azathioprine, which requires further evaluation, suggests a cell-mediated immune process.
Macdonald
MR. Ligneous conjunctivitis involving the Rubin A, Buck D, cervix. Br J Obstet Gynaecol 1989; 96: 1228-30. 2. Bateman JB, Pettit TH, Isenberg SJ, Simons KB. Ligneous conjunctivitis: an autosomal recessive disorder. J Ped Ophthalmol Strab 1986; 23: 137-40. 3. Hidayat A, Riddle P. Ligneous conjunctivitis. A clinicopathologic study of 17 cases. Ophthalmology 1987; 94: 949-59. 4. Chambers JD, Blodi FC, Golden B, MeKee AP. Ligneous conjunctivitis. Trans Am Acad Ophthalmol Otol 1969; 73: 996-1003. 5. Frimodt-Møller J. Conjunctivitis ligneosa combined with a dental affection. Acta Ophthalmol 1973; 51: 34-38. 6. Cooper TJ, Kazdan JJ, Cutz E. Ligneous conjunctivitis with tracheal obstruction. A case report with light and electron microscopy findings. Can J Ophthalmol 1979; 14: 57-62. 7. Tervaert D, Cruysberg J, Deutman A, Manschot W. Ligneous conjunctivitis. Doc Ophthalmol 1986; 64: 5-11. 8. Berlin AJ, Carim M, Langston R, Price R. Scleral grafting in the management of ligneous conjunctivitis. Ophthalmic Surg 1982; 13: 1.
288-91. 9. McGrand
JC, Rees DM, Harry J. Ligneous conjunctivitis. Br J Ophthalmol 1969; 53: 373-81. 10. Francois J, Victoria-Troncoso V. Treatment of ligneous conjunctivitis. Am J Ophthalmol 1968; 65: 674-78. 11. Firat T. Ligneous conjunctivitis. Am J Ophthalmol 1974; 78: 679-88. 12. Kanai A, Polack FM. Histologic and electron microscopic studies of ligneous conjunctivitis Am J Ophehalmol 1971; 72: 909-16. 13. Melikian H. Treatment of ligneous conjunctivitis. Am Ophthalmol 1985, 17: 763-65.
consumption it is found to be normal.8 Such results suggest that there is no increased oxygen delivery to tissues and consequently no need to suggest that increased peripheral vascular resistance is necessary to correct for it. Although some young subjects may have a hyperkinetic circulation with increased cardiac output, this is by no means a prerequisite step in the development of hypertension and is probably a feature of centrally mediated autonomic nervous activity. As such, it could be a predictor of future hypertension. Thus, the work of Andersson and co-workers seems to indicate that the hyperkinesis is not an abnormal alarm response but fails to take us further.7 That no vascular structural changes were seen in subjects with an early increase in cardiac index could well be regarded as more evidence that the autoregulation hypothesis is found
wanting. 1. Freis ED. Haemodynamics of 2. Widimsky J, Fejfarova MH,
hypertension. Physiol Rev 1960, 40: 27-54. Fejfar Z. Changes in cardiac output in hypertensive disease. Cardiologica 1957; 31: 381-89. 3. Korner PI, Fletcher PJ. Role of the heart in causing and maintaining hypertension. Cardiovasc Med 1977; 2: 139-55. 4. Johnson PC. Autoregulation of blood flow. Circ Res 1964; 14/15 (suppl 1): 1-291.
Ledingham JM. Autoregulation in hypertension: a review. J Hypertens 1989; 7 (suppl 4): 97-104. 6. Korner PI. Causal and homeostatic factors in hypertension. Clin Sci 5.
1982; 63: S5-26. 7. Andersson OK, Beckman-Suurküla M, Sannerstedt R, Magnusson M, Sivertsson R. Does hyperkinetic circulation constitute a prehypertensive stage? A 5-year follow-up of haemodynamics in young men with mild blood pressure elevation. J Intern Med 1989; 226: 401-08. 8. Lund-Johansen P. Haemodynamics in essential hypertension. Clin Sci 1980; 59: S343-54. 9. Lund-Johansen P. Hemodynamic alterations in early essential hypertension: recent advances. In: Gross F, Strasser T, eds. Mild hypertension: recent advances. New York: Raven, 1983: 237-49.
LIGNEOUS CONJUNCTIVITIS The solution
diagnostic puzzle sometimes comes from unexpected quarters. A bleeding cervical mass, which recurred repeatedly following excision and CO2 laser ablation, was diagnosed as a component of ligneous conjunctivitisl on ophthalmological review. Disorders involving the same type of tissue in different locations not only imply a common pathological process but also give rise to
to a
difficulties in management.
Ligneous conjunctivitis is a membranous or pseudomembranous conjunctivitis that causes pronounced thickening of the upper tarsal conjunctiva; the lower tarsal and bulbar conjunctiva are less frequently involved. It is uncommon (some seventy cases have been reported) and begins in early childhood, but may persist into middle age. Autosomal recessive inheritance has been suggestedand other reports record affected siblings.34 The eye changes are often accompanied by recurrent granulomas in other mucous membranes, notably the cervixl, gingiva,s respiratory tract6 and vocal cords.’7 Hydrocephalus accompanied ligneous conjunctivitis in three reported cases.4’8 The condition takes its name from the hard hyaline submucous infiltrate which leads to conjunctival thickening by up to 3 mm. The infiltrate may abrade the cornea, resulting in ulceration, and occasionally perforation. The plaque becomes partly separated from the underlying tarsus, and is sometimes covered by a fibrinous exudate. Histologically, there is an amorphous eosinophilic mass
resembling, but staining negatively for, amyloid, with focal deposits of fibrin. There is a variable cellular component, including neutrophils, lymphocytes, eosinophils, plasma cells, macrophages, and mast cells, and foreign body inclusions are common. Immunohistochemistry shows evidence of fibrin, albumin, and immunoglobulin fragments;3 immunoglobulin staining has not been observed.6,9 The epithelium is focally degenerate, but in places becomes hypertrophic and invades the submucosa. Submucosal blood vessels show focal degeneration, with gaps between endothelial cells. Treatment has been singularly unsuccessful. Regrowth of the membrane follows surgical removal, sometimes after as little as two days 4 Thermal, electric, and silver nitrate cautery, ablation by CO2 laser, and radiotherapy, appear to be similarly fruitless. Microbiological examination has yielded a catalogue of bacteria, but these are probably secondary colonists, and neither topical nor systemic antibiotic (nor antiviral) treatment has any consistent effect.
Topical cromoglycate, steroids, idoxuridine, cytarabine, and acetylcysteine have all been proposed, tried, and failed to produce repeatable benefit. Attempts to remove the plaque enzymatically with hyaluronidase and alpha chymotrypsin were initially reported as successsful,10,11 but the results have not been reproducible.12 Irrigation with fibrinolysin, combined with cryotherapy, was effective in one reported case.13 Some success has been claimed for treatment with systemic steroids and azathioprine. 3,7 The histochemical nature of the hyaline plaque, and the vessel wall abnormalities, suggest that it is derived from plasma. This leakage may have an immunological basis, although the cause of focal damage to the endothelium of the mucosal blood vessels is obscure. The initiating insult may be local (exposure of ocular and respiratory or genital mucous membrane to a common factor), or the condition may represent a multifocal response to a systemic process. Prevention of recurrence by systemic azathioprine, which requires further evaluation, suggests a cell-mediated immune process.
Macdonald
MR. Ligneous conjunctivitis involving the Rubin A, Buck D, cervix. Br J Obstet Gynaecol 1989; 96: 1228-30. 2. Bateman JB, Pettit TH, Isenberg SJ, Simons KB. Ligneous conjunctivitis: an autosomal recessive disorder. J Ped Ophthalmol Strab 1986; 23: 137-40. 3. Hidayat A, Riddle P. Ligneous conjunctivitis. A clinicopathologic study of 17 cases. Ophthalmology 1987; 94: 949-59. 4. Chambers JD, Blodi FC, Golden B, MeKee AP. Ligneous conjunctivitis. Trans Am Acad Ophthalmol Otol 1969; 73: 996-1003. 5. Frimodt-Møller J. Conjunctivitis ligneosa combined with a dental affection. Acta Ophthalmol 1973; 51: 34-38. 6. Cooper TJ, Kazdan JJ, Cutz E. Ligneous conjunctivitis with tracheal obstruction. A case report with light and electron microscopy findings. Can J Ophthalmol 1979; 14: 57-62. 7. Tervaert D, Cruysberg J, Deutman A, Manschot W. Ligneous conjunctivitis. Doc Ophthalmol 1986; 64: 5-11. 8. Berlin AJ, Carim M, Langston R, Price R. Scleral grafting in the management of ligneous conjunctivitis. Ophthalmic Surg 1982; 13: 1.
288-91. 9. McGrand
JC, Rees DM, Harry J. Ligneous conjunctivitis. Br J Ophthalmol 1969; 53: 373-81. 10. Francois J, Victoria-Troncoso V. Treatment of ligneous conjunctivitis. Am J Ophthalmol 1968; 65: 674-78. 11. Firat T. Ligneous conjunctivitis. Am J Ophthalmol 1974; 78: 679-88. 12. Kanai A, Polack FM. Histologic and electron microscopic studies of ligneous conjunctivitis Am J Ophehalmol 1971; 72: 909-16. 13. Melikian H. Treatment of ligneous conjunctivitis. Am Ophthalmol 1985, 17: 763-65.