Cicatrizing conjunctivitis is generally found in autoimmune disorders affecting the skin and mucous membranes. It can be caused by topical medications, infectious conjunctivitis, and chemical or physical trauma. We describe a case of unilateral cicatrizing conjunctivitis with a large conjunctival cyst after preseptal cellulitis in a 6-year-old boy.
A 6-year-old asymptomatic boy was seen at a pediatric ophthalmology clinic because his mother was concerned about a “bump” in his right eye.
At 21 months of age, he presented at the Denver Health Medical Center and was diagnosed as having preseptal cellulitis in his right eye. He was treated with intravenous ampicillin/sulbactam and discharged with amoxicillin/clavulanic acid. The patient was evaluated by the ophthalmology department during this admission and the diagnosis of preseptal cellulitis was confirmed. No topical treatment was given to the patient, he responded appropriately to antibiotics, and his treatment course was unremarkable. Two months later, the patient’s mother noted a “cyst-like” lesion in the right inferior fornix, which enlarged during the next few months. Although the “cyst-like” lesion was noted when the patient was 2 years old, the parents asked to see an ophthalmologist 4 years later.
On examination, visual acuity was 20/30 in both eyes. Retracting the right lower eyelid, a large cyst was present on the medial part of the conjunctival fornix. Scar tissue was present, causing forniceal foreshortening (Figure 1). Mild subepithelial fibrosis was also present on the tarsal conjunctiva of the upper eyelid. Fluorescein staining showed a decrease in tear film meniscus. The cornea was clear and the rest of the ocular examination was normal. The left eye did not show conjunctival cysts, fibrosis, or scarring. The culture was negative for chlamydia and serum immunoglobulin G titers to chlamydia species were also negative.
Figure 1. A cyst on the inferior conjunctival fornix of the right eye. The scar tissue caused forniceal foreshortening.
An excisional biopsy was performed (Figure 2), which showed a cyst with one to two layers of non-keratinizing epithelium with occasional goblet cells. The surrounding stroma was involved by foci of lymphoplasmacytic inflammation with scattered neutrophils that extended into the cyst lining. Additionally, multiple conjunctival biopsies sent for immunoflurescence were negative. No basophilic intracytoplasmic epithelial inclusion bodies on Giemsa staining were found.
Figure 2. The hematoxylin–eosin stained sections from the conjunctiva showed a cyst with one to two layers of non-keratinizing epithelium with occasional goblet cells. The surrounding stroma was involved by foci of lymphoplasmacytic inflammation with scattered neutrophils that extended into the cyst lining (hematoxylin–eosin; original magnification ×30).
Six months after the excisional biopsy, the patient showed no recurrence of cyst or scarring.
There are several causes of cicatrizing conjunctivitis in children. Stevens-Johnson syndrome, toxic epidermal necrolysis, and erythema multiform are part of a spectrum of diseases that affect skin and mucous membranes. They are usually a result of an idiosyncratic drug reaction.1 Mucous membrane pemphigoid, a subepithelial blistering disease, is chronic in nature and affects mucous membranes and skin with progressive scar formation. Females are more commonly affected and the incidence is highest between ages 40 and 80 years2; children are rarely affected. Linear immunoglobulin A disease is a chronic, blistering sub-epidermal disease that also affects skin and mucous membranes. It is similar to mucous membrane pemphigoid. A conjunctival biopsy with immunoflurescence immunoglobulin A staining can help differentiate linear immunoglobulin A disease from mucous membrane pemphigoid where immunoglobulin G, immunoglobulin M, and complement can be present.3
Drug-induced cicatrizing conjunctivitis, also known as pseudopemphigoid,4,5 is extremely rare in children. It is a chronic and often progressive form of cicatrizing conjunctivitis that resembles mucous membrane pemphigoid. Early diagnosis of drug-induced cicatrizing conjunctivitis is important because withdrawal of the medication sometimes halts the progression of the disease, although in some cases the process can continue after discontinuing the drug. The most common eye drops associated with drug-induced cicatrizing conjunctivitis are those that treat glaucoma, although several other topical eye medications have been implicated.
Preseptal cellulitis is known to have other orbital consequences, typically orbital extension, eyelid necrosis, ectropion, abscess formation, and rarely Brown’s syndrome.6,7
This case of unilateral cicatrizing conjunctivitis with a large forniceal conjunctival cyst and without mucosal or skin lesions is, to our knowledge, the first reported case in the literature. The lack of mucosal and skin findings, the negative immunofluorescent studies, the unilateral presentation, and the lack of progression during 4 years eliminated the typical causes of cicatrizing conjunctivitis in a child from the differential diagnosis. This case of cicatrizing conjunctivitis and large conjunctival cyst was associated with the previous preseptal cellulitis.
- Letko E, Papaliodis DN, Papaliodis GN, Daoud YJ, Ahmed AR, Foster CS. Stevens-Johnson syndrome and toxic epidermal necrolysis: a review of the literature. Ann Allergy Asthma Immunol. 2005;94:419–436. doi:10.1016/S1081-1206(10)61112-X [CrossRef]
- Daniel E, Thomas JE. Recent advances in mucous membrane pemphigoid. Curr Opin Ophthalmol. 2008;19:292–297. doi:10.1097/ICU.0b013e328302cc61 [CrossRef]
- Letko E, Bhol K, Foster CS, Ahmed AR. Linear IgA bullous disease limited to the eye: a diagnostic dilemma: response to intravenous immunoglobulin therapy. Ophthalmology. 2000;107:1524–1528. doi:10.1016/S0161-6420(00)00245-1 [CrossRef]
- Pouliquen Y, Patey A, Foster CS, Goichot L, Salvoldelli M. Drug–induced cicatricial pemphigoid affecting the conjunctiva: light and electron microscopic features. Ophthalmology. 1986;93:775–783.
- Thorn JE, Anhalt GL, Jabs DA. Mucous membrane pemphigoid and pseudopemphigoid. Ophthalmology. 2004;111:45–52. doi:10.1016/j.ophtha.2003.03.001 [CrossRef]
- Chaudhry IA, Shamsi FA, Elzaridi E, Al-Rashed W, Al-Amri A, Arat YO. Inpatient preseptal cellulitis: experience from a tertiary eye care centre. Br J Ophthalmol. 2008;92:1337–1341. doi:10.1136/bjo.2007.128975 [CrossRef]
- Schielke KC, Hilton AJ, Brown R. Acquired Brown’s syndrome associated with preseptal cellulitis. Strabismus. 2008;16:95–96. doi:10.1080/09273970802240767 [CrossRef]