Chronic cicatrizing conjunctivitis in a patient with ocular cicatricial pemphigoid and fatal Wegener granulomatosis

PURPOSE: To describe a case of chronic cicatrizing conjunctivitis in a patient with ocular cicatricial pemphigoid and Wegener granulomatosis. METHODS: Observational case report. A retrospective study. RESULTS: An 80-year-old man presented with chronic cicatrizing conjunctivitis, peripheral corneal thinning, and Wegener granulomatosis, which were diagnosed by his referring physician based on clinical (recurrent epistaxis, sinus congestion) and histopathologic features of nasal mucosa (granulomatous inflammation, vasculitis). A conjunctival biopsy performed by us disclosed features of active Wegener granulomatosis and ocular cicatricial pemphigoid, which indicate lack of control of both diseases with methotrexate treatment. The patient died of pulmonary complications from Wegener granulomatosis 1 week after our evaluation. CONCLUSION: Ocular cicatricial pemphigoid and Wegener granulomatosis are both potentially fatal autoimmune diseases. Ocular involvement in Wegener granulomatosis indicates poor control of the underlying systemic condition and is a marker for active vasculitis, which indicates the need for treatment with cyclophosphamide.     

Porphyria cutanea tarda presenting as cicatricial conjunctivitis

PURPOSE: To report a case of porphyria cutanea tarda presenting as cicatricial conjunctivitis.DESIGN: Observational study.METHODS: A 31-year-old man presented with bilateral inferior symblepharon, superior tarsal conjunctival scarring and concretions, and recurrent conjunctival and episcleral injection.RESULTS: Four years after initial presentation, the patient developed hepatitis C, and 2 years later blisters on his scalp and hands. Direct immunofluorescence studies of biopsies taken from the palpebral conjunctiva of the right lower lid were negative for cicatricial pemphigoid. A twenty-four hour urine specimen analysis revealed elevated levels of uroporphyrins and polycarboxylated porphyrins, confirming the diagnosis of porphyria cutanea tarda. The patient was treated with repeated phlebotomies and oral hydroxychloroquine, which resulted in a significant decrease in the skin lesions, conjunctival injection, and concretions under the upper lids.CONCLUSIONS: Cicatricial conjunctivitis may be a manifestation of porphyria cutanea tarda.     

Chronic cicatrizing conjunctivitis

Conjunctival fibrosis may result from chronic inflammation and may lead to alterations of conjunctival architecture. This results in ocular dryness, entropion and trichiasis, and corneal complications. Causes of conjunctival cicatrization are not limited to autoimmune diseases, such as ocular cicatricial pemphigoid, a severe disease associated with poor ocular prognosis. Other well-known causes include thermal and chemical burns, postinfectious conjunctivitis, and Stevens-Johnson syndrome. Ocular rosacea and atopic keratoconjunctivitis often are underdiagnosed causes of conjunctival fibrosis. Medical history, physical exam, and laboratory tests often allow for diagnosis of the underlying disease. Medical management varies according to specific causes, and many surgical strategies are available to restore corneal transparency and normal palpebral architecture.     

Chronic allergic conjunctivitis

Chronic allergic conjunctivitis (CAC) or perennial allergic conjunctivitis (PAC) is the most frequent form of allergy of the conjunctiva that is encountered in Northern Europe, particularly in city areas. Itching, redness of the mucosa, swelling of the lids and tearing are the main symptoms. Seasonal factors and associated rhinitis are often noted. Symptoms may respond to antiallergic drugs. In contrast, there are generally no specific signs of the disease on slitlamp examination. Most of the time the mucosal aspect (papillary, follicular or atrophic type) is common to other forms of conjunctival allergy. Non-specific factors may predispose to the appearance of CAC, such as an intolerance to a contact lens, a local infection, or a trauma of the eye. Astigmatism, heterophoria and photosensitization may also be triggering agents for the appearance of symptoms of an authentic CAC in an atopic patient. The close association between allergic and non-specific factors may explain the possibility of unilateral forms of CAC and, most of all, the frequent association of CAC to other forms of chronic conjunctivitis. The diagnosis relies on good clinical evaluation of both symptoms and signs. Serum IgE levels are elevated in no more than 30% of cases while tear levels of IgE are more often pathological (over 50% of cases); skin-testing remains the best method for confirming the diagnosis. In most cases it indicates an allergy to house dust and/or mites. Elimination of the offending allergen is the first treatment in CAC. Antiallergic drugs are also effective, such as antihistamines and mast cell stabilizers, nedocromil sodium being very efficient in this respect. In cases where this kind of treatment cannot be pursued indefinitely, specific desensitization to house dust or mites is effective and induces a prompt resolution of symptoms, whereas signs will persist a long time. All non-specific factors associated with the allergy should also be carefully considered and treated, although results are better in pure rather than associated clinical forms of CAC.     

Chronic conjunctivitis related to phthiriasis palpebrarum

We describe a case of chronic conjunctivitis related to phthiriasis palpebrarum. A 36?year-old female presented with gradual pruritus and painless ocular hyperaemia over the previous 3?months. On examination, nasal pterygium, conjunctival hyperaemia, oedema, and mild hypertrophy of the palpebral margin were observed. A slit-lamp examination revealed numerous creamy oval structures approximately 1?mm in diameter localised in the middle area of the lashes, and bloody crusts and a semi-transparent deposit were present in the superior palpebral margin. Based on the observation of numerous nits at the base of the eyelashes and the ectoparasite in the palpebral margin, a diagnosis of phthiriasis palpebrarum was made. The patient was referred to an infectologist for evaluation of other sexually transmitted diseases and examination of other body areas. She was successfully treated with oral ivermectin, shampoo for ciliary hygiene and artificial tears. Other recommendations to avoid re-infestation were made, such as changing, washing and sterilising clothes, towels and sheets daily. This report emphasizes the importance of the correct diagnosis and management of this disease, considered as sexually transmitted.     

Chronic conjunctivitis due to Mycobacterium tuberculosis

To report the clinical characteristics and treatment outcome in six patients with chronic conjunctivitis due to Mycobacterium tuberculosis. In this retrospective observational case series, all patients with a diagnosis of conjunctival tuberculosis seen in our clinics between January 2000 and January 2010 were reviewed. The clinical presentation, diagnostic investigations and response to medical therapy and outcomes were analyzed. Six patients (age range 15–47 years) were diagnosed with conjunctival tuberculosis. The mean duration from onset of symptoms to diagnosis was 6.5 months (range 1–12 months). Of the six patients, two had ulceration, one had a nodulo-ulcerative lesion, one had bilateral nodular epibulbar masses, and one had a hypertrophied papillary lesion. Systemic signs of tuberculosis were noted in two patients—pleural effusion in one and preauricular and submandibular lymph node involvement in the other. All patients had resolution of symptoms after treatment with four-drug anti-tuberculosis therapy (ATT). None had ocular or systemic recurrences after completion of ATT. Tuberculosis of the conjunctiva can have varied clinical presentation. Although a rare entity, it should be suspected in non-responding chronic conjunctivitis. A high index of suspicion and clinical examination aided by appropriate microbiological and histopathological testing can help in early diagnosis and management.     

Chronic dacryoadenitis misdiagnosed as eyelid edema and allergic conjunctivitis.

PURPOSE: To report the case of a 53-year-old woman with a 2-year history of episodic upper eyelid swelling and nonspecific complaints, who was diagnosed as having allergic conjunctivitis. METHODS: A complete ocular examination, orbital computerized tomographic (CT) scans followed by complete physical and systemic examinations. RESULTS: The results of physical and systemic examinations were unremarkable for systemic lymphoma and a primary focus of cancer. The results of the ocular examination were normal. CT scans demonstrated well-defined lesions bilaterally with a homogeneous internal structure in the lacrimal gland fossa, which suggested a diagnosis of chronic dacryoadenitis. The differential diagnosis included lymphoma and orbital metastases. The patient refused a biopsy and was started on a tapering dose of 60 mg oral prednisolone daily. The follow-up CT scans 1 month after cessation of 6-week oral corticosteroid treatment showed near complete resolution of the orbital lesions. CONCLUSION: This case demonstrates that orbital inflammation can be misdiagnosed as refractory allergic conjunctivitis.     

Paraneoplastic pemphigus, cicatricial conjunctivitis, and acanthosis nigricans with pachydermatoglyphy in a patient with bronchogenic squamous cell carcinoma.

A 77-year-old man with chronic conjunctivitis, acanthosis nigricans with pachydermatoglyphy, and pemphigus-like mucocutaneous lesions was found to have a well-differentiated bronchogenic squamous cell carcinoma. Histopathologic and immunofluorescence studies confirmed the diagnosis of paraneoplastic pemphigus. Skin lesions resolved with oral prednisone and azathioprine therapy, but the conjunctivitis and mucous membrane erosions persisted. The conjunctiva later became scarred with foreshortening of the fornices and development of symblepharon. External beam irradiation arrested the growth of the tumor but did not have any effect on the diseased conjunctiva and other mucous membranes. This case demonstrates that cicatrizing conjunctivitis with bullous mucocutaneous lesions may be a clinical sign associated with an occult neoplasm.     

Ocular cicatricial pemphigoid.

Cicatricial pemphigoid is an inflammatory disease of presumed autoimmune etiology. It most commonly affects the conjunctiva and oral mucosa and less commonly the skin. The ocular manifestations of the disease include bilateral conjunctival shrinkage, xerosis, and corneal opacification. The progression of cicatricial pemphigoid is variable and can be interrupted by periods of remission or by periods of rapid exacerbation. Ocular surgery and topical pharmaceuticals may contribute to the exacerbation of this disease. Current treatment consists of systemic immunosuppressants and systemic corticosteroids.