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Blistering Mucosal Eruption in a Young Woman With Systemic Lupus Erythematosus —Diagnosis

Blistering Mucosal Eruption in a Young Woman With Systemic Lupus Erythematosus —Diagnosis Diagnosis: Mucosal lupus erythematosus. Microscopic findings and clinical course Histopathologic examination of a biopsy specimen showed interface mucositis with lymphocytes attacking the mucosal-submucosal junction associated with vacuolar change of the basal layer and scattered apoptotic keratinocytes (Figure 2). Cleft formation was observed between the mucosa and submucosa with hemorrhage present within the vesicle (Figure 3). Direct immunofluorescence microscopy demonstrated moderate to strong staining for IgG and C3 and discontinuous, weak staining for IgA and IgM along the mucosal-submucosal junction. View LargeDownload Figure 2. View LargeDownload Figure 3. The patient began treatment with mycophenolate mofetil, 500 mg/d increased to twice daily after 1 week; her hydroxychloroquine dose was increased to 200 mg twice daily, and she began taking a 20-mg prednisone taper. She had no evidence of increased systemic disease activity. After 3 weeks, she reported diarrhea, and her mycophenolate mofetil dose was decreased back to 500 mg/d. Within 2 months, her lips were clear. Discussion The oral mucosa is a common target of disease activity in SLE, and oral ulceration is an American College of Rheumatology criterion for SLE diagnosis.1 In addition to ulceration, up to 45% of patients with SLE display a wide variety of mucosal lesions including discoid lesions, cheilitis, chronic plaques, lichen planus –like lesions, erythematous patches, and honeycomb plaques.2-6 Eruptions at the vermillion border have been reported in 25% of patients with SLE with oral mucosal lesions. These lesions are often poorly demarcated erosions that may advance over the vermillion border.7 Vermillion border lesions are usually painless and occur with oral cavity mucosal erosions, as was seen in our patient.7 Patients with SLE with oral lesions also may have involvement of other mucosal surfaces, including the conjunctiva, nasal, genital, and perianal mucosa, and often have additional active cutaneous lesions.3,8 Oral mucosal lupus erythematosus is associated with increased physician-reported systemic disease activity.6,8 However, oral lesions are not associated with nephritis,6 vasculitis,5 or with a significant change in serum levels of anti –double-stranded DNA antibodies, antinuclear antibodies, or complement.6 There is no known survival difference for patients with SLE with and without oral mucosal involvement.6 The characteristic histopathologic findings of mucosal lupus erythematosus include interface mucositis with a lymphocytic infiltrate, hydropic degeneration of the basal layer, and necrotic keratinocytes.9 The observed interface mucositis is the mucosal counterpart of the interface dermatitis observed on biopsy specimens of cutaneous lupus erythematosus lesions.9 Direct immunofluorescence microscopy findings are frequently positive and most commonly demonstrate linear or granular deposition of IgM, IgG, and C3 in the basement membrane zone.7 The microscopic findings usually readily distinguish mucosal lupus erythematosus lesions from conditions that may present similarly, including herpes simplex and other vesiculobullous disorders, but are nonspecific and can be confused with lichenoid mucositis in the setting of lichen planus, lichenoid drug reactions, or other hypersensitivity reactions.9 Treatment of mucosal lupus erythematosus can include topical and intralesional corticosteroids, antimalarial agents, and systemic immunosuppressive agents for severe or refractory cases.2,8 Conscientious photoprotection is recommended, although the role of sun exposure in mucosal lupus erythematosus development remains unclear.8 Return to Quiz Case. References 1. Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982;25(11):1271-12777138600PubMedGoogle ScholarCrossref 2. Orteu CH, Buchanan JA, Hutchison I, Leigh IM, Bull RH. Systemic lupus erythematosus presenting with oral mucosal lesions: easily missed? Br J Dermatol. 2001;144(6):1219-122311422045PubMedGoogle ScholarCrossref 3. Burge SM, Frith PA, Juniper RP, Wojnarowska F. Mucosal involvement in systemic and chronic cutaneous lupus erythematosus. Br J Dermatol. 1989;121(6):727-7412611124PubMedGoogle ScholarCrossref 4. Yell JA, Mbuagbaw J, Burge SM. Cutaneous manifestations of systemic lupus erythematosus. Br J Dermatol. 1996;135(3):355-3628949425PubMedGoogle ScholarCrossref 5. Jorizzo JL, Salisbury PL, Rogers RS III, et al. Oral lesions in systemic lupus erythematosus. Do ulcerative lesions represent a necrotizing vasculitis? J Am Acad Dermatol. 1992;27(3):389-3941401272PubMedGoogle ScholarCrossref 6. Urman JD, Lowenstein MB, Abeles M, Weinstein A. Oral mucosal ulceration in systemic lupus erythematosus. Arthritis Rheum. 1978;21(1):58-61623694PubMedGoogle ScholarCrossref 7. Jonsson R, Heyden G, Westberg NG, Nyberg G. Oral mucosal lesions in systemic lupus erythematosus: a clinical, histopathological and immunopathological study. J Rheumatol. 1984;11(1):38-426366226PubMedGoogle Scholar 8. Callen JP. Oral manifestations of collagen vascular disease. Semin Cutan Med Surg. 1997;16(4):323-3279421226PubMedGoogle ScholarCrossref 9. Nico MM, Vilela MA, Rivitti EA, Louren ço SV. Oral lesions in lupus erythematosus: correlation with cutaneous lesions. Eur J Dermatol. 2008;18(4):376-38118573706PubMedGoogle Scholar http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Blistering Mucosal Eruption in a Young Woman With Systemic Lupus Erythematosus —Diagnosis

Archives of Dermatology , Volume 148 (1) – Jan 1, 2012

Blistering Mucosal Eruption in a Young Woman With Systemic Lupus Erythematosus —Diagnosis

Abstract

Diagnosis: Mucosal lupus erythematosus. Microscopic findings and clinical course Histopathologic examination of a biopsy specimen showed interface mucositis with lymphocytes attacking the mucosal-submucosal junction associated with vacuolar change of the basal layer and scattered apoptotic keratinocytes (Figure 2). Cleft formation was observed between the mucosa and submucosa with hemorrhage present within the vesicle (Figure 3). Direct immunofluorescence microscopy demonstrated moderate to...
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Publisher
American Medical Association
Copyright
Copyright © 2012 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.148.1.114-a
Publisher site
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Abstract

Diagnosis: Mucosal lupus erythematosus. Microscopic findings and clinical course Histopathologic examination of a biopsy specimen showed interface mucositis with lymphocytes attacking the mucosal-submucosal junction associated with vacuolar change of the basal layer and scattered apoptotic keratinocytes (Figure 2). Cleft formation was observed between the mucosa and submucosa with hemorrhage present within the vesicle (Figure 3). Direct immunofluorescence microscopy demonstrated moderate to strong staining for IgG and C3 and discontinuous, weak staining for IgA and IgM along the mucosal-submucosal junction. View LargeDownload Figure 2. View LargeDownload Figure 3. The patient began treatment with mycophenolate mofetil, 500 mg/d increased to twice daily after 1 week; her hydroxychloroquine dose was increased to 200 mg twice daily, and she began taking a 20-mg prednisone taper. She had no evidence of increased systemic disease activity. After 3 weeks, she reported diarrhea, and her mycophenolate mofetil dose was decreased back to 500 mg/d. Within 2 months, her lips were clear. Discussion The oral mucosa is a common target of disease activity in SLE, and oral ulceration is an American College of Rheumatology criterion for SLE diagnosis.1 In addition to ulceration, up to 45% of patients with SLE display a wide variety of mucosal lesions including discoid lesions, cheilitis, chronic plaques, lichen planus –like lesions, erythematous patches, and honeycomb plaques.2-6 Eruptions at the vermillion border have been reported in 25% of patients with SLE with oral mucosal lesions. These lesions are often poorly demarcated erosions that may advance over the vermillion border.7 Vermillion border lesions are usually painless and occur with oral cavity mucosal erosions, as was seen in our patient.7 Patients with SLE with oral lesions also may have involvement of other mucosal surfaces, including the conjunctiva, nasal, genital, and perianal mucosa, and often have additional active cutaneous lesions.3,8 Oral mucosal lupus erythematosus is associated with increased physician-reported systemic disease activity.6,8 However, oral lesions are not associated with nephritis,6 vasculitis,5 or with a significant change in serum levels of anti –double-stranded DNA antibodies, antinuclear antibodies, or complement.6 There is no known survival difference for patients with SLE with and without oral mucosal involvement.6 The characteristic histopathologic findings of mucosal lupus erythematosus include interface mucositis with a lymphocytic infiltrate, hydropic degeneration of the basal layer, and necrotic keratinocytes.9 The observed interface mucositis is the mucosal counterpart of the interface dermatitis observed on biopsy specimens of cutaneous lupus erythematosus lesions.9 Direct immunofluorescence microscopy findings are frequently positive and most commonly demonstrate linear or granular deposition of IgM, IgG, and C3 in the basement membrane zone.7 The microscopic findings usually readily distinguish mucosal lupus erythematosus lesions from conditions that may present similarly, including herpes simplex and other vesiculobullous disorders, but are nonspecific and can be confused with lichenoid mucositis in the setting of lichen planus, lichenoid drug reactions, or other hypersensitivity reactions.9 Treatment of mucosal lupus erythematosus can include topical and intralesional corticosteroids, antimalarial agents, and systemic immunosuppressive agents for severe or refractory cases.2,8 Conscientious photoprotection is recommended, although the role of sun exposure in mucosal lupus erythematosus development remains unclear.8 Return to Quiz Case. References 1. Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982;25(11):1271-12777138600PubMedGoogle ScholarCrossref 2. Orteu CH, Buchanan JA, Hutchison I, Leigh IM, Bull RH. Systemic lupus erythematosus presenting with oral mucosal lesions: easily missed? Br J Dermatol. 2001;144(6):1219-122311422045PubMedGoogle ScholarCrossref 3. Burge SM, Frith PA, Juniper RP, Wojnarowska F. Mucosal involvement in systemic and chronic cutaneous lupus erythematosus. Br J Dermatol. 1989;121(6):727-7412611124PubMedGoogle ScholarCrossref 4. Yell JA, Mbuagbaw J, Burge SM. Cutaneous manifestations of systemic lupus erythematosus. Br J Dermatol. 1996;135(3):355-3628949425PubMedGoogle ScholarCrossref 5. Jorizzo JL, Salisbury PL, Rogers RS III, et al. Oral lesions in systemic lupus erythematosus. Do ulcerative lesions represent a necrotizing vasculitis? J Am Acad Dermatol. 1992;27(3):389-3941401272PubMedGoogle ScholarCrossref 6. Urman JD, Lowenstein MB, Abeles M, Weinstein A. Oral mucosal ulceration in systemic lupus erythematosus. Arthritis Rheum. 1978;21(1):58-61623694PubMedGoogle ScholarCrossref 7. Jonsson R, Heyden G, Westberg NG, Nyberg G. Oral mucosal lesions in systemic lupus erythematosus: a clinical, histopathological and immunopathological study. J Rheumatol. 1984;11(1):38-426366226PubMedGoogle Scholar 8. Callen JP. Oral manifestations of collagen vascular disease. Semin Cutan Med Surg. 1997;16(4):323-3279421226PubMedGoogle ScholarCrossref 9. Nico MM, Vilela MA, Rivitti EA, Louren ço SV. Oral lesions in lupus erythematosus: correlation with cutaneous lesions. Eur J Dermatol. 2008;18(4):376-38118573706PubMedGoogle Scholar

Journal

Archives of DermatologyAmerican Medical Association

Published: Jan 1, 2012

Keywords: systemic lupus erythematosus,exanthema,blister,blistering eruption,mucous membrane,lupus erythematosus

References