{'fraction_non_alphanumeric': 0.027457710223093897, 'fraction_numerical': 0.008335376317724933, 'mean_word_length': 5.634146341463414, 'pattern_counts': {'":': 0, '<': 1, '<?xml version=': 0, '>': 0, 'https://': 0, 'lorem ipsum': 0, 'www.': 0, 'xml': 0}, 'pii_count': 0, 'substrings_counts': 0, 'word_list_counts': {'cursed_substrings.json': 0, 'profanity_word_list.json': 0, 'sexual_word_list.json': 1, 'zh_pornsignals.json': 0}}
Ligneous conjunctivitis: a case report with multiorgan involvement

Sir: Ligneous conjunctivitis is a rare disease of conjunctival epithelium secondary to plasminogen deficiency. In some severe instances, all epithelial surfaces are involved and the condition presents as a systemic disease. We describe a case of systemic ligneous disease in an 18-year-old Mulatto female in which all of the potential extraocular manifestations previously described in the literature were manifested within one patient. The patient was an 18-year-old well-nourished Mulatto female (75 kg, 1.7 m) with a long, protracted history of multiorgan involvement by ligneous disease first diagnosed as an infant. Manifestations of systemic disease were noted due to an early history of hydrocephalus that required placement of a ventriculoperitoneal shunt at 9 months (subsequently revised three times). Later on, her conjunctivae became erythematous with diffuse fibrin deposits (Figure 1) and her oral cavity demonstrated gingival hyperplasia near the tooth insertions. Additional systemic manifestations included otitis media, sinusitis and genital tract involvement. At age 18 years she presented with laboratory studies indicating a decreased plasminogen level and function. Significant laboratory results included an erythrocyte sedimentation rate of 22 mm ⁄ h, white blood cell count 11 300 ⁄ ll, plasminogen functional assay of 12% and plasminogen antigen < 1.2 mg ⁄ dl. Her condition continued to worsen, with extensive woody deposits in her eyes and recurrent lesions within her nasal sinuses and vaginal vault. Subsequently, she was started on topical fresh frozen plasma (FFP). Multiple surgical strippings from the woody, whitish lesions on her conjunctivae and biopsies from the vaginal vault, cervix and endometrium obtained on different occasions demonstrated abundant subepithelial deposits of eosinophilic amorphous hyaline, amyloid-like material composed mainly of fibrin admixed with inflammatory cells (Figure 2A,B). These deposits were periodic acid–Schiff (PAS) positive, diastase resistant, trichrome positive and negative with the colloidal iron stain. The inflammatory infiltrate was predominantly composed of T cells (CD3+) and macrophages (CD68+ and FXIIIa+). Most of the CD3+ cells were immunoreactive for CD25 [interleukin (IL)-2 receptor antibody]. Strong staining in the fibrinous material was noted for insulin-like growth factor (IGFR), fibronectin (Figure 2C) and fibrinogen (Figure 2D). Tumour necrosis factor (TNF)-a staining was noted predominantly in the inflammatory infiltrate. Ligneous conjunctivitis is a rare condition characterized by pseudomembranous conjunctivitis with the deposition of firm, woody, amyloid-like material. Although ophthalmic involvement is the most common manifestation of the disease, the less frequent systemic involvement of other membranes such as larynx, vocal cords, gingiva, trachea, nasopharynx, tympanic membranes, vagina and cervix and an association with congenital hydrocephalus have been reported. The development of ligneous lesions in pseudomembranous disease has been shown to be most often due to severe type I plasminogen deficiency caused by sporadic, compound-heterozygous, or homozygous mutations of the plasminogen gene. In most instances, symptoms first present in infants and children, although the disease may manifest at any age. Often, an initial triggering event is inflammation due to infection or surgical trauma, which leads to the formation of the ligneous pseudomembranes. While these patients suffer from an inability to degrade fibrin aggregates adequately, it is interesting to note that no patient with ligneous conjunctivitis has been reported as suffering from venous thrombosis. This is believed to be due to the fact that in physiological conditions, activation of coagulation within the vasculature is a slow process and leads to only small amounts of fibrin, which can be adequately degraded and removed by other proteolytic enzymes within Figure 1. Ligneous disease involving the conjunctivae.