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Postoperative endophthalmitis in a patient with Darier—White disease
Postoperative endophthalmitis in a patient with Darier–White disease Anthony M. Carlsson, MD, FRCSC; Graham D. Barrett, FRACS, FRACO ABSTRACT • RÉSUMÉ
Case report: We report a case of endophthalmitis after cataract surgery in a patient with Darier–White disease. Comments: A keratotic lid lesion in contact with the ocular surface near the temporal corneal wound is believed to be the source of infection. Such lesions are the predominant manifestation of Darier–White disease and are known to harbour gram-positive skin flora. This has implications for planning intraocular surgery in these patients. Observation : Nous faisons état d’un cas d’entophtalmie consécutive à une chirurgie de la cataracte chez un patient atteint de la maladie de Darier–White. Commentaires : L’on pense qu’une lésion kératosique de la paupière en contact avec la surface oculaire près de la blessure temporale de la cornée serait à l’origine de l’infection. Les lésions de ce genre, qui constituent la principale manifestation de la maladie de Darier–White, sont reconnues pour héberger une flore cutanée Gram positive. Cela a des implications pour les projets de chirurgie intraoculaire chez ces patients.
D
arier–White disease, or keratosis follicularis, was described in 1889 simultaneously by Dr. James White and Dr. Ferdinand-Jean Darier.1 It is a rare autosomal dominant inherited disease of the skin with high penetrance and variable expressivity affecting 1:30 000 to 1:100 000 individuals in the 1st to 3rd decades of life.2 The defective gene ATP2A2 codes for a calcium channel pump in the endoplasmic reticulum of skin cells, resulting in abnormal keratinization.3 Signs include skin, nail, and mucous membrane changes resulting in eruptions of hyperkeratotic papules, typically in a seborrheic distribution. The lesions may coalesce into large, often malodorous, papillomatous masses that harbour gram-positive bacteria.1 The pathognomonic histology includes features of premature keratinization, hyperkeratosis, and parakeratosis.2 Eye findings associated with Darier–White disease include keratotic eyelid plaques, peripheral corneal opacities, and central epithelial irregularities.4 Only one case of postoperative endophthalmitis has been reported in association with this disease.5 We report a similar case and review the literature of this rare disease. CASE
ral clear corneal technique. Best corrected visual acuity (BCVA) on postoperative day 1 was 6/9, and intraocular pressure (IOP) measured 16 mm Hg. Prednisolone acetate 1% and ofloxacin drops were given 4 times a day. Five days later he returned complaining of pain, with BCVA of 6/18 and IOP of 12 mm Hg. Clinical findings included diffusely injected conjunctiva, 4+ cellular reaction in the anterior chamber with 1 mm hypopyon, and
Fig. 1—Hyperkeratotic plaque along the superotemporal grey line, in contact with the bulbar conjunctiva.
REPORT
A 70-year-old man with Darier–White disease underwent uncomplicated left cataract surgery using a tempoFrom the Lion’s Eye Institute, University of Western Australia, Perth, Australia Originally received Jan. 22, 2006 Accepted for publication Oct. 10, 2006 Correspondence to: Graham D. Barrett, FRACS, 2 Verdun St., Nedlands, Perth, 6009 Australia This article has been peer-reviewed. Cet article a été évalué par les pairs. Can J Ophthalmol 2007;42:134–5 doi:10.3129/can.j.ophthalmol.06-094
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Fig. 2—Hyperkeratotic plaque along the superotemporal grey line, in contact with the bulbar conjunctiva.
Endophthalmitis with Darier–White disease—Carlsson & Barrett
Endophthalmitis with Darier–White disease—Carlsson & Barrett
significant posterior vitritis. A waxy, minimally elevated hyperkeratotic plaque was found along the left superotemporal grey line, in contact with the bulbar conjunctiva (Figs. 1 and 2). Similar lesions were present in the periorbital skin of the contralateral eye (Fig. 3) and on the forehead. Endophthalmitis was diagnosed, and an anterior chamber tap and intravitreal injection of
vancomycin (1 mg/0.1 mL) and ceftazidime (2.25 mg/0.1 mL) were performed. Topical ofloxacin (0.3%), 1% prednisolone acetate, and 1% atropine were administered. One month later his visual acuity had improved to 6/6 unaided, and all inflammation had resolved. Aqueous humour cultures failed to grow any organisms. COMMENTS
Fig. 3—Multiple periocular hyperkeratotic nodules.
Our patient demonstrated many of the clinical stigmata associated with Darier–White disease, including widespread hyperkeratotic papules (Figs. 4 and 5) and characteristic nail defects (Fig. 6). Many lesions were present in the periocular region and on the eyelids. We believe the infectious source of the endophthalmitis to be a lid lesion in contact with the ocular surface near the temporal corneal wound. Such skin lesions are known to harbour high densities of gram-positive skin flora. A recent study has shown that Staphylococcus aureus can persist intracellularly within these lesions, causing antibiotic treatment failure.6 Although we failed to grow any organisms in culture, the clinical course after intravitreal antibiotics supports a bacterial etiology. Special consideration should be given to patients with Darier–White disease before intraocular surgery. Thorough treatment of any blepharitis is essential. Other treatment measures may be considered for skin lesions, including oral and topical retinoids, dermabrasion, laser vaporization, and photodynamic therapy.7 Consultation with a dermatologist before intraocular surgery is recommended.
Fig. 4—Hyperkeratotic papular rash over trunk.
REFERENCES
Fig. 5—Hyperkeratotic nodules and plaques on backs of hands.
Fig. 6—Characteristic linear nail defects.
1. Burge S. Darier’s disease: the clinical features and pathogenesis. Clin Exp Dermatol 1994;19:193–205. 2. Sehgal V, Srivastava G. Darier’s (Darier-White) disease/keratosis follicularis. Int J Dermatol 2005;44:184–92. 3. Dhitavat J, Dode L, Leslie N, Sakuntabbhai A, Lorette G, Hovnanian A. Mutations in the sarcoplasmic/endoplasmic reticulum Ca2+ ATPase isoform causes Darier’s disease. J Invest Dermatol 2003;121:486–9. 4. Blackman H, Rodrigues M, Peck G. Corneal epithelial lesions in keratosis follicularis (Darier’s disease). Ophthalmology 1980;87:931–43. 5. Macsai M, Agarwal S. Staphylococcal endophthalmitis following cataract surgery in a patient with Darier’s disease. Cornea 1998;17:335–7. 6. Von Eiff C, Becker K, Metze D, et al. Intracellular persistence of Staphylococcus aureus small-colony variants within keratinocytes – a cause for antibiotic treatment failure in a patient with Darier’s disease. Clin Infect Dis 2001;32:1643–7. 7. Exadaktylou D, Kurwa HA, Calonje E, Barlow RJ. Treatment of Darier’s disease with photodynamic therapy. Br J Dermatol 2003;149:606–10. Key words: Darier–White disease, endophthalmitis
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Case report: We report a case of endophthalmitis after cataract surgery in a patient with Darier–White disease. Comments: A keratotic lid lesion in contact with the ocular surface near the temporal corneal wound is believed to be the source of infection. Such lesions are the predominant manifestation of Darier–White disease and are known to harbour gram-positive skin flora. This has implications for planning intraocular surgery in these patients. Observation : Nous faisons état d’un cas d’entophtalmie consécutive à une chirurgie de la cataracte chez un patient atteint de la maladie de Darier–White. Commentaires : L’on pense qu’une lésion kératosique de la paupière en contact avec la surface oculaire près de la blessure temporale de la cornée serait à l’origine de l’infection. Les lésions de ce genre, qui constituent la principale manifestation de la maladie de Darier–White, sont reconnues pour héberger une flore cutanée Gram positive. Cela a des implications pour les projets de chirurgie intraoculaire chez ces patients.
D
arier–White disease, or keratosis follicularis, was described in 1889 simultaneously by Dr. James White and Dr. Ferdinand-Jean Darier.1 It is a rare autosomal dominant inherited disease of the skin with high penetrance and variable expressivity affecting 1:30 000 to 1:100 000 individuals in the 1st to 3rd decades of life.2 The defective gene ATP2A2 codes for a calcium channel pump in the endoplasmic reticulum of skin cells, resulting in abnormal keratinization.3 Signs include skin, nail, and mucous membrane changes resulting in eruptions of hyperkeratotic papules, typically in a seborrheic distribution. The lesions may coalesce into large, often malodorous, papillomatous masses that harbour gram-positive bacteria.1 The pathognomonic histology includes features of premature keratinization, hyperkeratosis, and parakeratosis.2 Eye findings associated with Darier–White disease include keratotic eyelid plaques, peripheral corneal opacities, and central epithelial irregularities.4 Only one case of postoperative endophthalmitis has been reported in association with this disease.5 We report a similar case and review the literature of this rare disease. CASE
ral clear corneal technique. Best corrected visual acuity (BCVA) on postoperative day 1 was 6/9, and intraocular pressure (IOP) measured 16 mm Hg. Prednisolone acetate 1% and ofloxacin drops were given 4 times a day. Five days later he returned complaining of pain, with BCVA of 6/18 and IOP of 12 mm Hg. Clinical findings included diffusely injected conjunctiva, 4+ cellular reaction in the anterior chamber with 1 mm hypopyon, and
Fig. 1—Hyperkeratotic plaque along the superotemporal grey line, in contact with the bulbar conjunctiva.
REPORT
A 70-year-old man with Darier–White disease underwent uncomplicated left cataract surgery using a tempoFrom the Lion’s Eye Institute, University of Western Australia, Perth, Australia Originally received Jan. 22, 2006 Accepted for publication Oct. 10, 2006 Correspondence to: Graham D. Barrett, FRACS, 2 Verdun St., Nedlands, Perth, 6009 Australia This article has been peer-reviewed. Cet article a été évalué par les pairs. Can J Ophthalmol 2007;42:134–5 doi:10.3129/can.j.ophthalmol.06-094
134
Fig. 2—Hyperkeratotic plaque along the superotemporal grey line, in contact with the bulbar conjunctiva.
Endophthalmitis with Darier–White disease—Carlsson & Barrett
Endophthalmitis with Darier–White disease—Carlsson & Barrett
significant posterior vitritis. A waxy, minimally elevated hyperkeratotic plaque was found along the left superotemporal grey line, in contact with the bulbar conjunctiva (Figs. 1 and 2). Similar lesions were present in the periorbital skin of the contralateral eye (Fig. 3) and on the forehead. Endophthalmitis was diagnosed, and an anterior chamber tap and intravitreal injection of
vancomycin (1 mg/0.1 mL) and ceftazidime (2.25 mg/0.1 mL) were performed. Topical ofloxacin (0.3%), 1% prednisolone acetate, and 1% atropine were administered. One month later his visual acuity had improved to 6/6 unaided, and all inflammation had resolved. Aqueous humour cultures failed to grow any organisms. COMMENTS
Fig. 3—Multiple periocular hyperkeratotic nodules.
Our patient demonstrated many of the clinical stigmata associated with Darier–White disease, including widespread hyperkeratotic papules (Figs. 4 and 5) and characteristic nail defects (Fig. 6). Many lesions were present in the periocular region and on the eyelids. We believe the infectious source of the endophthalmitis to be a lid lesion in contact with the ocular surface near the temporal corneal wound. Such skin lesions are known to harbour high densities of gram-positive skin flora. A recent study has shown that Staphylococcus aureus can persist intracellularly within these lesions, causing antibiotic treatment failure.6 Although we failed to grow any organisms in culture, the clinical course after intravitreal antibiotics supports a bacterial etiology. Special consideration should be given to patients with Darier–White disease before intraocular surgery. Thorough treatment of any blepharitis is essential. Other treatment measures may be considered for skin lesions, including oral and topical retinoids, dermabrasion, laser vaporization, and photodynamic therapy.7 Consultation with a dermatologist before intraocular surgery is recommended.
Fig. 4—Hyperkeratotic papular rash over trunk.
REFERENCES
Fig. 5—Hyperkeratotic nodules and plaques on backs of hands.
Fig. 6—Characteristic linear nail defects.
1. Burge S. Darier’s disease: the clinical features and pathogenesis. Clin Exp Dermatol 1994;19:193–205. 2. Sehgal V, Srivastava G. Darier’s (Darier-White) disease/keratosis follicularis. Int J Dermatol 2005;44:184–92. 3. Dhitavat J, Dode L, Leslie N, Sakuntabbhai A, Lorette G, Hovnanian A. Mutations in the sarcoplasmic/endoplasmic reticulum Ca2+ ATPase isoform causes Darier’s disease. J Invest Dermatol 2003;121:486–9. 4. Blackman H, Rodrigues M, Peck G. Corneal epithelial lesions in keratosis follicularis (Darier’s disease). Ophthalmology 1980;87:931–43. 5. Macsai M, Agarwal S. Staphylococcal endophthalmitis following cataract surgery in a patient with Darier’s disease. Cornea 1998;17:335–7. 6. Von Eiff C, Becker K, Metze D, et al. Intracellular persistence of Staphylococcus aureus small-colony variants within keratinocytes – a cause for antibiotic treatment failure in a patient with Darier’s disease. Clin Infect Dis 2001;32:1643–7. 7. Exadaktylou D, Kurwa HA, Calonje E, Barlow RJ. Treatment of Darier’s disease with photodynamic therapy. Br J Dermatol 2003;149:606–10. Key words: Darier–White disease, endophthalmitis
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