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Cicatrising conjunctivitis due to paraneoplastic pemphigoid Article in British Journal of Ophthalmology · December 2007 DOI: 10.1136/bjo.2007.115634 · Source: PubMed
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Nisha R Acharya
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noun (owl). Another possibility in favour of the noun is that congenital glaucoma was recognised in ancient times and that large buphthalmic eyes had the appearance of large owl eyes. In addition to its controversial glossological (from the Greek ‘‘glossa’’, meaning language) derivation, glaucoma management and especially the introduction of the trabeculectomy has been another centre of debate. Although the first trabeculectomy is often attributed to Cairns in Cambridge,3 a year earlier, the Greek Koryllos was the first to publish details of this guarded penetrating filtration procedure that he called a trabeculectomy.4 Koryllos described this filtering surgical technique with the construction of a scleral flap and subsequent drainage of fluid via the gap between the scleral and conjunctival flap, little different to the trabeculectomy that is commonly performed today. His publication, however, received little attention since it was published in a Greek journal infrequently read worldwide. Perhaps glaucoma is more Greek than we realise.
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Figure 1 (A) Subepidermal serous fluid (arrowheads) and perivascular inflammation consisting of neutrophils and eosinophils in a biopsy specimen of a skin bulla. Haematoxylin and eosin, original magnification 206. (B) Direct immunofluorescence showing linear C3 staining of the basement membrane along the dermal–epidermal junction (arrow). IgG staining (not shown) was similar. Original magnification 406.
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Michael Tsatsos, David Broadway Department of Ophthalmology, Norfolk and Norwich University Hospital, UK Correspondence to: Michael Tsatsos, Norfolk and Norwich University Hospital, Ophthalmology Department, Colney Lane, Norwich, NR4 7UY UK;
[email protected]
doi: 10.1136/bjo.2007.114298 Accepted 19 January 2007 Competing interests: None.
References 1 Fronimopoulos J, Lascaratos J. The terms glaucoma and cataract in the ancient Greek and Byzantine writings. Doc Ophthalmol 1991;77:369. 2 Fronimopoulos J. Glaucoma and cataract. Hell Ann Ophthalmol 1964;3:99–108. 3 Cairns JE. Trabeculectomy: preliminary report of a new method. Am J Ophthalmol 1968;66:673–9. 4 Koryllos K. The excision of the corneoscleral meshwork (trabeculectomy) as an antiglaucomatous operation. Delt Ell Ophthalol 1967;35:147–55. 5 Koryllos K. Technique and results of goniectomy in glaucomas. Hell Ann Ophthalmol 1963;4:11–22. 6 Grewe R. Zur Geschichte des Glaukoms. Klin Mbl Augenheik 1986;188:167–9.
Cicatrising conjunctivitis due to paraneoplastic pemphigoid Ocular cicatricial pemphigoid (OCP) is a putative autoimmune, chronic, inflammatory, subepithelial blistering disease affecting mucous membranes.1 It is characterised by linear deposition of immunoglobulins and complement along epithelial basement membranes.1 In this report, we describe a cicatrising conjunctivitis that was clinically and pathologically similar to ocular cicatricial pemphigoid but occurred concurrently with systemic malignancy.
Case report A 69-year-old cachectic man with chronic obstructive pulmonary disease presented with painful mouth and skin blisters. He developed a painful red eye 1 week later. Visual acuity was 20/25 on the right and 20/30 on the left. Slit-lamp examination showed bulbar www.bjophthalmol.com
Figure 2 (A) Slit-lamp photograph showing bullae, an intense papillary reaction and subepithelial scarring of the superior palpebral conjunctiva. (B) Chest x ray showing a 5.763.5 cm spiculated opacity in the patient’s right lung and mediastinal lymph node enlargement. conjunctival injection and an intense papillary reaction of the palpebral conjunctiva on the right. At examination 10 days later, the patient had new fornix foreshortening and symblepharon formation. A skin biopsy revealed subepidermal bullous dermatosis with eosinophils and neutrophils (fig 1A). Direct immunofluorescence showed linear IgG and C3 staining along the basement membrane (fig 1B). These results were consistent with cicatricial pemphigoid. The patient began aggressive ocular lubrication and oral prednisone 80 mg daily. During the following week, his ocular symptoms improved dramatically, although he developed bullae of the superior palpebral conjunctiva on the right, and bilateral subepithelial scarring, fornix foreshortening and symblepharon (fig 2A). The patient was hospitalised that same week because of increasing difficulty with oral intake and weight loss. A chest x ray suggested lung cancer (fig 2B). Bronchoalveolar lavage and systemic evaluation confirmed non-small cell lung carcinoma, most likely squamous cell, stage IIIA. The patient underwent radiation and chemotherapy with cisplatin and etoposide. While on moderate-dose steroids, he developed new cutaneous bullae. Mycophenolate mofetil was added but had no effect. Alkylating agents were not tried due to profound neutropenia. At a 5-month follow-up, visual acuity remained 20/25 in each eye, with clear corneas despite cicatricial entropion and trichiasis of the right lower eyelid.
Comment Previously reported cases have shown a temporal association between cicatricial pemphigoid and systemic solid malignancies, including non-small cell lung cancer (adenocarcinoma, squamous cell, large cell), gastric adenocarcinoma and endometrial adenocarcinoma.2–8 In some patients, the pemphigoid responded to chemotherapy or surgical resection of the tumour, suggesting a paraneoplastic process.2 6 8 Antibodies to laminin-5, a basement membrane protein elaborated by lung and gastrointestinal epithelia, have been found in patients with cicatricial pemphigoid and lung and gastric cancers.2–4 6 8 Aberrant synthesis of laminin-5 by tumour cells, or tumour-related inflammation and invasion, may expose the antigen to the immune system.2 9 Pemphigus, an autoimmune disease characterised by intraepithelial blisters, can also present as a paraneoplastic process, although it is primarily associated with lymphoreticular malignancies.10 In a similar reported case of paraneoplastic pemphigoid, the diagnosis of squamous cell lung cancer was made 2 months after the patient developed cicatrising conjunctivitis.7 The differential diagnosis for cicatrising conjunctivitis is broad and includes OCP, Stevens Johnsons syndrome, chemical burns, radiation, toxic conjunctivitis, post-infectious conjunctivitis, graft versus host disease, atopic keratoconjunctivitis and conjunctival squamous cell carcinoma.1 Paraneoplastic pemphigoid should also be considered in the differential diagnosis, and this case
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illustrates the importance of obtaining a pertinent history and review of systems.
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Tina Rutar Department of Ophthalmology, University of California San Francisco, San Francisco, CA, USA
Matilda F Chan, Nisha R Acharya F.I. Proctor Foundation, Department of Ophthalmology, University of California San Francisco, San Francisco, CA, USA
Ayman Naseri Department of Ophthalmology, San Francisco VA Medical Center, San Francisco, CA, USA Correspondence to: Dr Tina Rutar, Department of Ophthalmology, University of California San Francisco, 10 Koret Way, K301, San Francisco, CA 94143-0730, USA;
[email protected] Informed consent was obtained for publication of the person’s details in this report.
doi: 10.1136/bjo.2007.115634 Accepted 27 January 2007 Funding: This research was supported by the That Man May See Foundation.
Figure 1 Coronal contrast-enhanced T1-weighted MR image shows MALT lymphoma (arrow) before therapy (A) and 12 months later (B). high risk of ocular morbidity, especially dry eye.1 Here, we report a case of a biopsy-proven extranodal MALT lymphoma of the lacrimal gland treated with two courses of four weekly cycles of rituximab.
Competing interests: None.
Case report
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A 64-year-old woman presented with unilateral ptosis and swelling of her right lacrimal gland. Best-corrected visual acuity was 20/25. Slit-lamp examination showed severe punctate superficial keratopathy (basal secretion 0 mm OU). MRI scan detected a tumour of the
1 Eschle-Meniconi ME, Ahmad S, Foster CS. Mucous membrane pemphigoid: an update. Curr Opin Ophthalmol 2005;16:303–17. 2 Setterfield J, Shirlaw PJ, Lazarova Z, et al. Paraneoplastic cicatricial pemphigoid, 1999. Br J Dermatol 1999;141:127–31. 3 Gibson GE, Daoud MS, Pittelkow MR. Anti-epiligrin (laminin 5) cicatricial pemphigoid and lung carcinoma: coincidence or association? Br J Dermatol 1997;137:780–2. 4 Lish KM, Washenik K, Yancey KB, et al. Antiepiligrin cicatricial pemphigoid in a patient with HIV. J Am Acad Dermatol 1997;36:486–8. 5 Lenz P, Hsu R, Yee C. [Cicatricial pemphigoid with autoantibodies to laminin 5 (epiligrin) in a patient with metastatic endometrial carcinoma]. Hautarzt 1998;49:31–5. 6 Fujimoto W, Ishida-Yamamoto A, Hsu R. Antiepiligrin cicatricial pemphigoid: a case associated with gastric carcinoma and features resembling epidermolysis bullosa acquisita. Br J Dermatol 1998;139:682–7. 7 Vroman DT, Breckenridge RR, Solomon KD, et al. Bronchogenic squamous cell carcinoma presenting as cicatrizing conjunctivitis. Cornea 2006;25:611–13. 8 Uchiyama K, Yamamoto Y, Taniuchi K, et al. Remission of antiepiligrin (laminin-5) cicatricial pemphigoid after excision of gastric carcinoma. Cornea 2000;19:564–6. 9 Skyldberg B, Salo S, Eriksson E, et al. Laminin-5 as a marker of invasiveness in cervical lesions. J Natl Cancer Inst 1999;91:1882–7. 10 Kaplan I, Hodak E, Ackerman L. Neoplasms associated with paraneoplastic pemphigus: a review with emphasis on non-hematologic malignancy and oral mucosal manifestations. Oral Oncol 2004;40:553–62.
Rituximab for the treatment of extranodal marginal zone B-cell lymphoma of the lacrimal gland Extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT lymphoma) of the lacrimal gland is a rare condition. Treatment options chiefly include radiation of the tumour, chemotherapy, surgical removal or a combination of these strategies.1 Radiation therapy is associated with a
lacrimal gland (fig 1A). Histopathological and immunohistochemical studies of a lacrimal gland biopsy specimen disclosed an extranodal MALT lymphoma (fig 2); typical extraorbital manifestations were excluded. Treatment was started with four weekly cycles of rituximab (each dose 375 mg/m2). The MRI scan at 2 months showed a subtotal involution of the tumour. Schirmer values were 2 mm OU. Exophthalmus, which according to Hertel readings was 3 mm before treatment, had resolved 3 months after initiating therapy. Another four cycles of rituximab
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Figure 2 (A) Orbital soft tissue with destruction of the lacrimal gland by an atypical lymphoid infiltrate. Remnants of the glandular structures surrounded by atypical centrocytoid or monocytoid lymphatic cells with an increase in blasts (one atypical mitotic figure is highlighted with a cross) and scattered plasma cells can be seen. A typical intra-acinar lymphoepithelial lesion is shown surrounded by a dotted line. Giemsa 6306. (B) The neoplastic lymphoid infiltrate is mainly composed of strongly CD20-positive cells. Several destructive intraglandular lymphoepithelial lesions are clearly visible, one of which is highlighted by a surrounding dotted line. The complete phenotype of the lymphoma was CD20+, CD52, CD232, cyclinD12, kappa+, lambda–; 2006. (C, D) Lambda and kappa light chain stainings show monoclonal light chain restriction of the neoplastic lymphoid cells, with a polyclonal background of plasma cells; 2006. www.bjophthalmol.com
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