Conjunctivitis, Ligneous

Conjunctivitis, Ligneous

National Organization for Rare Disorders, Inc.

Important

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Disorder Subdivisions

  • None

General Discussion

Ligneous Conjunctivitis is a rare disorder that is characterized by recurrent lesions of the mucous membranes, especially in the eye. This disorder usually presents itself during childhood and may also be found in the mucous membranes of the larynx, vocal chords, nose, trachea, bronchi, vagina, cervix, and gingiva. The lesions in the mucous membranes have a wood-like (ligneous) consistency to them and are thick, firm, knotty and tough. The cause of this disorder is not known although there have been multiple cases of siblings with this condition suggesting an autosomal recessive inheritance.

Symptoms

The symptoms of Ligneous Conjunctivitis are redness of the membranes in the eye (conjunctivae), tearing of the eye that develops slowly and persists for a long periods of time, a false thin layer of tissue that covers the membranes of the eye (pseudomembranes) on and near the eyelids, and lesions of the mucous membranes that develop into tough, thick, firm, knotty masses replacing the normal mucous membranes. The changes in the mucous membranes have a wood-like (ligneous) consistency. These symptoms may be found in one or both eyes.



Some patients with this disorder may also have wood-like lesions and inflammation of the mucous membranes in the larynx, vocal chords, nose, trachea, bronchi, vagina, cervix and/or gingiva.



Ligneous Conjunctivitis has resolved itself spontaneously in some patients. An unusually large head caused by the abnormal dilation of the ventricles in the brain and the obstruction of the spinal fluid passages of the central nervous system (hydrocephalus), swelling of the trachea and bronchi (tracheobronchitis), respiratory problems (especially pneumonia), and airway obstruction due to recurrent growth of ligneous membranes have been found in association with this disorder in some patients.

Causes

The exact cause of Ligneous Conjunctivitis is not known. It may be an autoimmune disease which occurs when the body's natural defenses (e.g., antibodies and lymphocytes) attack normal tissue for unknown reasons. Multiple cases of this disorder have occurred within the same family suggesting an autosomal recessive inheritance.



Human traits, including the classic genetic diseases, are the product of the interaction of two genes, one received from the father and one from the mother. In recessive disorders, the condition does not appear unless a person inherits the same defective gene for the same trait from each parent. If an individual receives one normal gene and one gene for the disease, the person will be a carrier for the disease, but usually will not show symptoms. The risk of transmitting the disease to the children of a couple, both of whom are carriers for a recessive disorder, is 25 percent. Fifty percent of their children risk being carriers of the disease, but generally will not show symptoms of the disorder. Twenty-five percent of their children may receive both normal genes, one from each parent, and will be genetically normal. The risk is the same for each pregnancy.

Affected Populations

Ligneous Conjunctivitis appears to affect males and females in equal numbers. This disorder usually appears during childhood, although it has presented itself in patients of all ages. There have been less than 100 reported cases in the medical literature with approximately 50 of them being well documented. Many cases of Ligneous Conjunctivitis have been observed in the country of Turkey.

Standard Therapies

Treatment of Ligneous Conjunctivitis is not always completely successful. The topical use of hyaluronidase and chymotrypsin before removing the affected membranes, as well as without removal of the affected membranes, has been successful in some patients.



Repeated surgical stripping of the affected membranes often results in reoccurence unless the area is also treated with a topical ointment.



Frequent treatment with topical cyclosporine has completely resolved the membrous problems in some, and slowed the recurrences in others. A ophthalmologic form of cyclosporine is being developed by Sandoz Pharmaceuticals.



It has been found that surgical stripping of the affected membranes, when located in the trachea, is best accomplished when the anesthesia is given with a mask. This procedure eliminates the problem of the membranes becoming dislodged and obstructing the airways.



Genetic counseling may be of benefit for patients and their families in cases where there is a history of this disorder among family members. Other treatment is symptomatic and supportive.

References

JOURNAL ARTICLES

Ligneous Tracheobronchitis: An Unusual Case of Airway Obstruction. M. F. Babcock et al.; Anesthesiology (Nov 1987; 67(5)). Pp. 819-21.



Ligneous Conjunctivitis: An Autosomal Recessive Disorder. J. B. Bateman M.D., et al.; Journal of Pediatric Ophthalmology & Strabismus (May/June 1986; 23(3)). Pp. 137-40.



Immunohistologic Findings and Results of Treatment with Cyclosporine in Ligneous Conjunctivitis. E. J. Holland M.D., et al.; American Journal of Ophthalmology (Feb 1989; 107)). Pp. 160-66.



Ligenous Conjunctivitis with Tracheal Obstruction: A Case Report, With Light and Electron Microscopy Findings. T. J. Cooper M.D., et al.; Canad J Ophthal (1979; 14(57)). Pp. 57-62.



Ligneous Conjunctivitis. A Clinicopathologic Study of 17 Cases. A. A. Hidayat et al.; Ophthalmology (Aug 1987; 94(8)). Pp. 949-59.



Therapy with a Purified Plasmonogen Concentrate in an Infant with Ligneous Conjunctivitis and Homozygous Plasminogen Deficiency. D. Schott et al.; New Eng J Med (Dec 3 1998; 339(23)). Pp. 1679-86.



Induction of Ligneous Conjunctivitis by Conjunctival Surgery. G. S. Schwartz et al.; Am J Ophthalmol (Aug 1995; 120(2)). Pp. 253-54.



Ligneous Conjunctivitis: An Ophthalmic Disease with Potentially Fatal Tracheobronchial Obstruction. Laryngeal and Tracheobronchial Features. S. R. Cohen; Ann Otol Rhinol Laryngol (Jul 1990; 99(7pt1)). Pp. 509-12.



Homozygous Mutations in the Plasminogen Gene of Two Unrelated Girls with Ligneous Conjunctivitis. V. Schuster et al.; Blood (Aug 1 1997; 90(3)). Pp. 958-66.



Ligneous Conjunctivitis. Ten Years Follow-Up. Z. Nussgens et al.; Ophthalmic Pediatr Genet (Sept 1993; 14(3)). Pp. 137-40.



Tropical Heparin in the Treatment of Ligneous Conjunctivitis. R. DeCock et al.; Ophthalmology (Nov 1995; 102(11)). Pp. 1654-59.



FROM THE INTERNET

Online Mendelian Inheritance in Man (OMIM). Victor A. McKusick, Editor; Johns Hopkins University, Last Edit Date 12/8/98, Entry Number 217090.

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