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Discoid lupus erythematosus presenting as madarosis
autosomal dominant Stargardt-like macular dystrophy with homology to the SUR4 protein family. Invest Ophthalmol 2001;42:2652–2663. 4. Bernstein PS, Tammur J, Singh N, et al. Diverse macular dystrophy phenotype caused by a novel complex mutation in the ELOVL4 gene. Invest Ophthalmol 2001;42:3331– 3336. 5. Donoso LA, Frost AT, Stone EM, et al. Autosomal dominant Stargardt-like macular dystrophy. Arch Ophthalmol 2001; 119:564 –570.
Discoid Lupus Erythematosus Presenting as Madarosis Dinesh Selva, FRANZCO, Celia S. Chen, MBBS, Craig L. James, FRCPA, and Shyamala C. Huilgol, FACD Discoid lupus erythematosus (DLE) is an autoimmune disorder that usually affects the sun– exposed skin. Periocular involvement occurs uncommonly and may progress from eyelid erythema to scarring and madarosis. METHOD: Observational case report. RESULT: A case of DLE that presented with madarosis alone in the absence of preceding skin erythema and scarring. CONCLUSION: Our case demonstrates that DLE may present with madarosis alone in the absence of a history of preceding erythema and scarring. Discoid lupus erythematosus should therefore be considered as a differential diagnosis in chronic blepharitis that persists despite usual medical management and eyelid hygiene. Biopsy should be considered in the presence of clinical features such as erythematous scale on the face and alopecia and sent for direct immunofluorescence staining. (Am J Ophthalmol 2003;136:545–546. © 2003 by Elsevier Inc. All rights reserved.) PURPOSE:
A
45-YEAR-OLD WOMAN PRESENTED WITH A 2-MONTH
history of focal loss of eyelashes (madarosis) on the upper eyelids. Examination revealed mild bilateral blepharitis and patchy madarosis of the upper eyelids (Figure 1A). There was no evidence of erythema or scaling of the
Accepted for publication Feb 17, 2003. From the Oculoplastic & Orbital Clinic, Department of Ophthalmology, Royal Adelaide Hospital, University of Adelaide (D.S., S.C.), Adelaide Pathology Partners, West Terrace (C.J.), and the Department of Dermatology, Royal Adelaide Hospital and University of Adelaide, Adelaide, Australia (S.H.). Inquiries to Dinesh Selva, FRANZCO, Oculoplastic & Orbital Clinic, Department of Ophthalmology, Royal Adelaide Hospital, North Terrace, Adelaide 5000, South Australia.
VOL. 136, NO. 3
pretarsal skin. The remainder of her skin showed no abnormalities, and examination of the scalp revealed no alopecia areata. A diagnosis of madarosis secondary to blepharitis was made, and she was treated with eyelid scrubs and ocular lubricants. Nine months later she presented with irritation of the left eye and scaly erythema associated with eyelash loss on the pretarsal skin of the lateral half of the left lower eyelid (Figure 1B). Scalp examination then demonstrated an area of scarring alopecia (Figure 1C). Biopsies of the left pretarsal skin and the scalp lesion were performed. Immunofluorescent staining demonstrated deposition of immunoglobulins at the dermoepidermal junction at both sites, and a diagnosis of discoid lupus erythematosus was made (Figure 2). Blood tests showed a positive antinuclear antibody (titer 1:60) with a nucleolar pattern but a negative anti-dsDNA. The eyelid lesion failed to respond to topical hydrocortisone 1%, and the patient required treatment with intralesional triamcinolone before the lesion resolved. At last follow-up 12 months later, the periocular skin was normal and the eyelid and scalp alopecia was unchanged. Discoid lupus erythematosus (DLE) is characterized by erythematous, scaly patches most commonly found on the face. Lower eyelid involvement is seen in 6% and may be associated with conjunctival injection and scarring.1 Palpebral lesions may rarely be the presenting or sole manifestation of the disease.2 The eyelid lesions may itch and are often exacerbated by trauma or sunlight. Involved areas have a tendency to heal with scarring, pigmentary changes, and atrophy. In the case of the eyelid margin, this may lead to loss of eyelashes. Superficial keratopathy and stromal keratitis have also been reported.3 Involvement of the eyelid only with DLE is uncommon and the diagnosis may be difficult. In fact, Donzis and coworkers4 reported an average delay of 2 years before the correct diagnosis. The typical presentation of DLE with ocular involvement is symmetrical erythema and edema of the outer third of the lower eyelids, which progresses to scarring with permanent loss of eyelashes.1 However, our case demonstrates that DLE may present with madarosis alone in the absence of a history of preceding erythema and scarring. When madarosis is the only presenting symptom, the appearance may resemble blepharitis. Discoid lupus erythematosus should therefore be considered as a differential diagnosis in chronic blepharitis that persists despite usual medical management and eyelid hygiene.1 Other differential diagnoses of madarosis include eczema, sebaceous carcinoma, and trichotillomania. It is important to note that chronic untreated DLE may lead to permanent scarring and deformities in the lid margin.5 In addition, approximately 5% to 10% of DLE patients develop systemic lupus erythematosus.5 Patients with DLE should be encouraged to avoid
BRIEF REPORTS
545
FIGURE 1. (A) Clinical photograph demonstrating patches of eyelash loss (arrows) on the right upper eyelid. (B) Clinical photograph showing erythema of the lateral aspect of the left lower eyelid associated with eyelash loss. (C) Clinical photograph of an area of scarring alopecia on the scalp.
excessive sunlight, use sunscreens, and wear sunglasses. Therapy with topical corticosteroids is successful in a proportion of patients, but resistant cases may require
intralesional corticosteroids or oral hydroxychloroquine.5 Our case demonstrates that DLE may present with madarosis alone in the absence of a history of preceding erythema and scarring. Biopsy should be considered in the presence of clinical features like erythematous scale on the face and alopecia and sent for direct immunofluorescence staining. REFERENCES
1. Burge SM, Frith PA, Juniper RP, Wojnarowski F. Mucosal involvement in systemic and chronic cutaneous lupus erythematosus. Br J Dermatol 1989;121:727–741. 2. Tosti A, Tosti G, Gidvannini A. Discoid lupus erythematosus solely involving the eyelids: report of three cases. J Am Acad Dermatol 1987;16:1259 –1260. 3. Raizman MB, Baum J. Discoid lupus keratitis. Arch Ophthalmol 1989;107:545–547. 4. Donzis PB, Insler MS, Buntin DM, Wolfley D. Discoid lupus erythematosus involving the eyelids. Am J Ophthalmol 1984; 98:32–36. 5. Gloor P, Kim M, McNiff JM, Wolfley D. Discoid lupus erythematosus presenting as asymmetric posterior blepharitis. Am J Ophthalmol 1997;124:707–709.
FIGURE 2. Left lower eyelid section immunofluorescent staining demonstrated coarse granular thick band of immunoglobulin M along the dermoepidermal junction (lupus band) (ⴛ400, direct immunofluorescence).
546
AMERICAN JOURNAL
OF
OPHTHALMOLOGY
SEPTEMBER 2003
Discoid Lupus Erythematosus Presenting as Madarosis Dinesh Selva, FRANZCO, Celia S. Chen, MBBS, Craig L. James, FRCPA, and Shyamala C. Huilgol, FACD Discoid lupus erythematosus (DLE) is an autoimmune disorder that usually affects the sun– exposed skin. Periocular involvement occurs uncommonly and may progress from eyelid erythema to scarring and madarosis. METHOD: Observational case report. RESULT: A case of DLE that presented with madarosis alone in the absence of preceding skin erythema and scarring. CONCLUSION: Our case demonstrates that DLE may present with madarosis alone in the absence of a history of preceding erythema and scarring. Discoid lupus erythematosus should therefore be considered as a differential diagnosis in chronic blepharitis that persists despite usual medical management and eyelid hygiene. Biopsy should be considered in the presence of clinical features such as erythematous scale on the face and alopecia and sent for direct immunofluorescence staining. (Am J Ophthalmol 2003;136:545–546. © 2003 by Elsevier Inc. All rights reserved.) PURPOSE:
A
45-YEAR-OLD WOMAN PRESENTED WITH A 2-MONTH
history of focal loss of eyelashes (madarosis) on the upper eyelids. Examination revealed mild bilateral blepharitis and patchy madarosis of the upper eyelids (Figure 1A). There was no evidence of erythema or scaling of the
Accepted for publication Feb 17, 2003. From the Oculoplastic & Orbital Clinic, Department of Ophthalmology, Royal Adelaide Hospital, University of Adelaide (D.S., S.C.), Adelaide Pathology Partners, West Terrace (C.J.), and the Department of Dermatology, Royal Adelaide Hospital and University of Adelaide, Adelaide, Australia (S.H.). Inquiries to Dinesh Selva, FRANZCO, Oculoplastic & Orbital Clinic, Department of Ophthalmology, Royal Adelaide Hospital, North Terrace, Adelaide 5000, South Australia.
VOL. 136, NO. 3
pretarsal skin. The remainder of her skin showed no abnormalities, and examination of the scalp revealed no alopecia areata. A diagnosis of madarosis secondary to blepharitis was made, and she was treated with eyelid scrubs and ocular lubricants. Nine months later she presented with irritation of the left eye and scaly erythema associated with eyelash loss on the pretarsal skin of the lateral half of the left lower eyelid (Figure 1B). Scalp examination then demonstrated an area of scarring alopecia (Figure 1C). Biopsies of the left pretarsal skin and the scalp lesion were performed. Immunofluorescent staining demonstrated deposition of immunoglobulins at the dermoepidermal junction at both sites, and a diagnosis of discoid lupus erythematosus was made (Figure 2). Blood tests showed a positive antinuclear antibody (titer 1:60) with a nucleolar pattern but a negative anti-dsDNA. The eyelid lesion failed to respond to topical hydrocortisone 1%, and the patient required treatment with intralesional triamcinolone before the lesion resolved. At last follow-up 12 months later, the periocular skin was normal and the eyelid and scalp alopecia was unchanged. Discoid lupus erythematosus (DLE) is characterized by erythematous, scaly patches most commonly found on the face. Lower eyelid involvement is seen in 6% and may be associated with conjunctival injection and scarring.1 Palpebral lesions may rarely be the presenting or sole manifestation of the disease.2 The eyelid lesions may itch and are often exacerbated by trauma or sunlight. Involved areas have a tendency to heal with scarring, pigmentary changes, and atrophy. In the case of the eyelid margin, this may lead to loss of eyelashes. Superficial keratopathy and stromal keratitis have also been reported.3 Involvement of the eyelid only with DLE is uncommon and the diagnosis may be difficult. In fact, Donzis and coworkers4 reported an average delay of 2 years before the correct diagnosis. The typical presentation of DLE with ocular involvement is symmetrical erythema and edema of the outer third of the lower eyelids, which progresses to scarring with permanent loss of eyelashes.1 However, our case demonstrates that DLE may present with madarosis alone in the absence of a history of preceding erythema and scarring. When madarosis is the only presenting symptom, the appearance may resemble blepharitis. Discoid lupus erythematosus should therefore be considered as a differential diagnosis in chronic blepharitis that persists despite usual medical management and eyelid hygiene.1 Other differential diagnoses of madarosis include eczema, sebaceous carcinoma, and trichotillomania. It is important to note that chronic untreated DLE may lead to permanent scarring and deformities in the lid margin.5 In addition, approximately 5% to 10% of DLE patients develop systemic lupus erythematosus.5 Patients with DLE should be encouraged to avoid
BRIEF REPORTS
545
FIGURE 1. (A) Clinical photograph demonstrating patches of eyelash loss (arrows) on the right upper eyelid. (B) Clinical photograph showing erythema of the lateral aspect of the left lower eyelid associated with eyelash loss. (C) Clinical photograph of an area of scarring alopecia on the scalp.
excessive sunlight, use sunscreens, and wear sunglasses. Therapy with topical corticosteroids is successful in a proportion of patients, but resistant cases may require
intralesional corticosteroids or oral hydroxychloroquine.5 Our case demonstrates that DLE may present with madarosis alone in the absence of a history of preceding erythema and scarring. Biopsy should be considered in the presence of clinical features like erythematous scale on the face and alopecia and sent for direct immunofluorescence staining. REFERENCES
1. Burge SM, Frith PA, Juniper RP, Wojnarowski F. Mucosal involvement in systemic and chronic cutaneous lupus erythematosus. Br J Dermatol 1989;121:727–741. 2. Tosti A, Tosti G, Gidvannini A. Discoid lupus erythematosus solely involving the eyelids: report of three cases. J Am Acad Dermatol 1987;16:1259 –1260. 3. Raizman MB, Baum J. Discoid lupus keratitis. Arch Ophthalmol 1989;107:545–547. 4. Donzis PB, Insler MS, Buntin DM, Wolfley D. Discoid lupus erythematosus involving the eyelids. Am J Ophthalmol 1984; 98:32–36. 5. Gloor P, Kim M, McNiff JM, Wolfley D. Discoid lupus erythematosus presenting as asymmetric posterior blepharitis. Am J Ophthalmol 1997;124:707–709.
FIGURE 2. Left lower eyelid section immunofluorescent staining demonstrated coarse granular thick band of immunoglobulin M along the dermoepidermal junction (lupus band) (ⴛ400, direct immunofluorescence).
546
AMERICAN JOURNAL
OF
OPHTHALMOLOGY
SEPTEMBER 2003