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Editor,—Pemphigus vulgaris is an autoimmune, blistering disease of the skin and mucous membranes.1 The characteristic ocular finding is conjunctivitis, and corneal involvement is rare.2 3 We present a case with pemphigus vulgaris with severe keratolysis that required a corneal transplantation.
CASE REPORT
A 41 year old man had suffered from pemphigus vulgaris for 2 years, and prednisolone 40 mg/day and cyclosporine 300 mg/day had been prescribed. He was admitted to the Hamamatsu University Hospital on 15 March 1999 with an acute exacerbation of the symptoms because of non-compliance with the corticosteroid therapy. He returned on 17 March 1999 because of increased discharge and visual loss in both eyes. His visual acuity was 20/20 right eye and 20/20 left eye, and his intraocular pressure was 24 mm Hg right eye and 20 mm Hg left eye. No remarkable findings were observed in both visual fields and optic discs. Slit lamp examination showed mild erosions of his eyelid and cornea. The treatment with prednisolone 40 mg/day and cyclosporine 300 mg/day was continued.
He returned on 9 April 1999 because of acute deterioration of vision in both eyes. His visual acuity was light perception in the right eye and counting fingers in the left. The right conjunctiva showed marked oedema and the anterior chamber was flat. Slit lamp examination showed that the lower two thirds of the right cornea had eroded leaving only Descemet's membrane and endothelium (Fig 1). The left conjunctiva showed mild oedema and slit lamp examination demonstrated anterior stromal opacities in the lower half of the cornea and bulla-like central corneal epithelial changes. Ofloxacin ointment was prescribed for both eyes.
Photograph of the anterior segment of the right eye on 13 April 1999.
The opacity of the right corneal stroma gradually increased, and scar-like tissue formed in the area of the erosion. Slit lamp examination showed that Descemet's membrane was touching the iris and lens. The corneal bullous degeneration in the left eye formed an erosion. After the corneal erosion and conjunctival oedema resolved, faint stromal opacities were observed in the region of the corneal lesion in the left eye.
Because of the overall improvement of the cornea of the right eye, an 11 mm right penetrating keratoplasty was performed on 22 September 1999. The dislocated lens was extracted and anterior vitrectomy was also performed. His visual acuity on 11 November 1999 was improved to 8/200 with +9.0 −2.5 × 65° D right eye, and 20/25 with −3.0 D −3.75 × 180° D left eye.
COMMENT
Corneal involvement is a rare complication in patients with pemphigus vulgaris. Severe corneal involvement has never been reported except in the case of a 56 year old man with severe ocular involvement including conjunctivitis, corneal ulceration, and perforation despite immunosuppressive therapy.4 Although a causative organism was not isolated, the authors suggested that the complications were due to an infectious agent.
Two mechanisms have been suggested to cause the corneal erosion—bacteria or other pathogenic organisms that infect the cornea because of the epithelial defect and tear film disorder brought on by the corticosteroid and immunosuppressive therapy. Although the culture obtained from right ocular discharge before starting ofloxacin ointment showed a negative result, we could not deny the bacterial infection. We did not perform a bacterial or viral culture or polymerase chain reaction examinations using a corneal sample.
The second mechanism is an autoimmune mechanism against one of the intercellular adhesion molecule—for example, desmoglein (Dsg). The patient was diagnosed as pemphigus vulgaris by histological examination, direct immunofluorescent staining of the skin showing intracellular deposition of immunogloblin G and high titres of circulating anti-Dsg 3 and anti-Dsg 1 antibodies (Fig 2). Because the cornea usually does not have Dsg 3 but Dsg 2,5 an autoimmune mechanism cannot be considered. However, prolonged epithelial defect by limbal damage may have resulted in the corneal erosion because of the expression of Dsg 3 by the epithelium of the corneal limbus. Although no infection was observed in both corneas, the association with an infectious mechanism may be involved in the pathogensis of corneal erosion in our case.
Histological examination by haematoxylin and eosin staining of lesional skin disclosed intraepidermal clefts which contained several acantholytic cells (left). The direct immunofluorescent staining of the skin showed intercellular deposition of immunogloblin G (right).