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Ligneous conjunctivitis in a patient of juvenile colloid milia

A rare association

Bhave, Shubhangi Sudhir; Joshi, Sanyogita Swapnil1

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Indian Journal of Ophthalmology: April 2015 - Volume 63 - Issue 4 - p 350-352
doi: 10.4103/0301-4738.158091
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Ligneous conjunctivitis is a rare form of chronic, recurrent conjunctivitis characterized by fibrinous pseudomembranes on the palpebral conjunctivae. Prevalence in the general population estimated between 0.13% and 0.42% in healthy subjects with heterozygous hypoplasminogenemia. It may be associated with systemic pseudomembranous lesions of the gingiva, ears, tracheobronchial tract, female genital tract, and kidneys. It has also been linked to congenital hydrocephalus and juvenile colloid milium.

Case Report

A 10-year-old female patient resident of Madhya Pradesh presented to us with chief complaints of redness, watering, discharge, irritation in both eyes since 8 months. There was no history of fever, instillation or intake of any drugs into the eyes. There was no history of asthma or allergy.

Her vision was 20/30 in right eye and 20/70 in left eye. Her refraction could not be done as she was unable to open her eyes.

Slit lamp examination showed matting of lashes with crusted discharge. Conjunctiva had plenty of purulent discharge and palpebral conjunctiva was studded with granulomatous, firm and woody membranous lesions [Figs. 13]. In both eyes, there was congestion. Ocular surface appeared unhealthy showing superficial punctate keratitis, epithelial defects, and peripheral vascularisation. Anterior chamber was quiet. Pupils were normal in size and reacting to light. Fundus appeared normal in both eyes.

Figure 1
Figure 1:
Upper lid showing thick, woody membrane covering upper tarsus
Figure 2
Figure 2:
Woody membrane covering upper tarsus
Figure 3
Figure 3:
Lower lid showing raw areas

On general examination, she was found to have multiple hyperpigmented papulopustular lesions over face, palms and Dermatologist's opinion was taken. The findings were suggestive of juvenile colloid milia [Fig. 4]. Pediatrician's opinion was taken regarding other associations like upper respiratory tract, middle ear which were found to be normal.

Figure 4
Figure 4:
Skin of face showing papulopustular lesions

She was started with topical moxifloxacin, lubricating eye drop and gel and was advised set of investigations. Investigations showed raised erythrocyte sedimentation rate (ESR) (28 mm/h). X-ray chest posterior-anterior view showed reticulonodular shadows. This aroused suspicion of miliary tuberculosis by radiologist. But as her total lymphocyte count, Mantoux test were normal and ESR was marginally raised, we excluded the possibility of tuberculosis. Patient was followed-up after 15 days. At that time her conjunctiva was studded with plenty of thick granulomatous lesions and formation of a woody pseudomembrane.

Excision of the lesions was planned. At the time of excision, membrane was easily peeled off and there were no underlying attachments. After the excision of membrane, hemostasis was achieved and supratarsal injection of triamcinolone acetate was given in both eyes. During same procedure, biopsy of skin lesions was taken and sent for hislopathological examination. Her vaginal mucosa was examined after obtaining informed written consent from parents during the procedure and was found to be normal.

Histopathological examination of conjunctival lesion showed mucosa lined by focally atrophic squamous epithelium with submucosal eosinophils, hyaline in nodular fashion and scattered inflammatory cells. Small blood vessels were present [Fig. 5]. Histopathological findings were suggestive of ligneous conjunctivitis.

Figure 5
Figure 5:
Focally atrophic squamous epithelium

Histopathological examination of skin lesions showed dystrophic calcification in epidermal and subepidermal zone [Fig. 6]. According to pathologists and dermatologist, calcification can occur in any form of pathology after certain time, though literature doesn't mention calcification in juvenile colloid milia.

Figure 6
Figure 6:
Submucosal eosinophlic hyaline in nodular fashion

Hematologist's opinion was taken for starting topical treatment with fresh frozen plasma. Patient was resident of remote village and poor. Treatment with fresh frozen plasma was not possible in this patient. So, we have started this patient with long-term cyclosporine drops and tear supplement.

After 1-month the patient was reviewed. The symptoms were relieved. On examination the thick woody membrane had resolved. She was continued with the same treatment and reviewed after 3 months. At this time, the woody membrane over conjunctiva had recurred because definitive treatment with plasminogen could not be started. This confirmed our diagnosis ligneous conjunctivitis.


Ligneous conjunctivitis is a rare form of chronic, recurrent conjunctivitis characterized by fibrinous pseudomembranes on the palpebral conjunctivae. It may be associated with systemic pseudomembranous lesions of the gingiva, ears, tracheobronchial tract, female genital tract, and kidneys. It has also been linked to congenital hydrocephalus and juvenile colloid milium.[12]

Colloid milium, which is characterized by the deposition of amorphous, eosinophilic granular deposits in the superficial dermis, has subtypes including the juvenile and adult variants. The juvenile variant, develops in children before puberty and has a familial incidence. Patients present with discrete papules measuring 0.2–1.5 cm in diameter, yellow-brown in color, appear translucent and when punctured characteristically express gelatinous material. The underlying tissues often feel indurated. Juvenile colloid milium predominantly affects the face, in particular the cheeks, nose, and around the mouth. It occurs due to vascular fragility due to infiltration of the blood vessel walls by colloid material. Juvenile colloid milia may present with gingival deposits and ligneous conjunctivitis as a result of infiltration of these tissues by colloid-like material.

Ligneous conjunctivitis may be inherited in an autosomal recessive pattern due to mutations in the plasminogen gene and associated with type I plasminogen deficiency (hypoplasminogenemia), leading to impaired wound healing.[34]

The conjunctivae are susceptible to frequent exposure to irritants, like dust, local infection, minor injury, and trauma, especially in young children.[1] This trauma induces the wound-healing cascade, which begins with fibrin matrix deposition. The fibrin acts as scaffolding for granulation tissue and then collagenous tissue for wound remodeling and eventual healing. Plasmin acts to degrade the fibrin clots, and it is formed from cleavage of the plasminogen. Plasminogen deficiency leads to impaired healing of injured mucosa with accumulation of a serofibrinous material that forms the fibrin-rich pseudomembranes, which harden giving the characteristic “woody” appearance.

It affects infant or child and common in females, presenting with recurrent conjunctivitis symptoms including discharge and redness of the conjunctiva.

Examination shows thick, firm pseudomembranous structures on the palpebral conjunctiva. Approximately 50% of cases are bilateral, and affected individuals have a predilection for upper eyelid involvement followed by lower eyelid and bulbar involvement.[1]

The initial signs of ligneous conjunctivitis are mucoid discharge and redness of the conjunctivae. This is followed by palpebral conjunctival pseudomembrane formation, and it progresses to the mucosal thickening with a wood-like consistency, which replaces the normal eyelid mucosa. Multiple recurrences are common and coincide with as fever, upper respiratory tract infection, ear infections, or in females, urogenital tract infection.[1] Involvement of the airway can be life-threatening.

The diagnosis of ligneous conjunctivitis can be challenging. It should be differentiated from following causes of pseudomembranous conjunctivitis:

  • Viral conjunctivitis
  • Bacterial conjunctivitis
  • Toxic conjunctivitis, e.g., secondary to medication use
  • Allergic or vernal conjunctivitis any inflammatory condition would respond to triamcinolone and cyclosporine. But in our case thick woody membrane and histopathological changes confirmed the diagnosis
  • Amyloidosis.

Management includes topical plasminogen concentrate,[3] topical fresh frozen plasma,[5] with systemic fresh frozen plasma,[67] topical heparin,[58910] with topical corticosteroids or alpha chymotrypsin,[1] topical cyclosporine A with amniotic membrane,[10] systemic low-dose lys-plasminogen,[13] surgical excision of pseudomembrane, which has high recurrence rates.

Complications are recurrence after surgical excision, corneal scarring, vascularization and perforation and steroid induced glaucoma.


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10. Tok OY, Kocaoglu FA, Tok L, Burcu A, Ornek F. Treatment of ligneous conjunctivitis with amniotic membrane transplantation and topical cyclosporine Indian J Ophthalmol. 2012;60:563–6

Source of Support: Nil.

Conflict of Interest: None declared.


Juvenile colloid milia; ligneous conjunctivitis; pseudomembranous conjunctivitis

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