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A 38-Year-Old Woman With Eyelid Discoloration—Diagnosis

A 38-Year-Old Woman With Eyelid Discoloration—Diagnosis Diagnosis: Heliotrope-like manifestation of adult-onset Still disease (AOSD). Diagnosis: Heliotrope-like manifestation of adult-onset Still disease (AOSD). Histopathologic analysis showed dilated vessels with a mild diffuse and perivascular infiltrate of mononuclear cells and neutrophils on hematoxylin-eosin staining, consistent with the patient's known AOSD. A diagnosis of a heliotrope-like manifestation of AOSD was given. The patient was treated with Vaseline (Unilever). Though her heliotrope eruption improved somewhat, it never remitted completely. She noted worsening of the heliotrope during a systemic flare (eg, arthralgias) of her AOSD. Diagnosis: Heliotrope-like manifestation of adult-onset Still disease (AOSD). Adult-onset Still disease is a rare, inflammatory, autoimmune disorder with limited discussion in the medical literature, consisting mainly of small-scale prospective studies, case reports, and retrospective studies. The diagnosis is one of exclusion, including consideration of neoplastic, infectious, and other autoimmune causes. The absence of serologic markers in this patient helped exclude other diagnoses such as dermatomyositis. Diagnostic criteria proposed by Yamaguchi et al1 allow physicians to diagnose Still disease after other diseases have been excluded if 5 criteria (more than 2 major criteria) are met. Major criteria include temperature greater than 39°C for more than 1 week; leukocytosis (white blood cell count >10 000/μL with >80% polymorphonuclear cells); typical skin eruption; and arthralgias lasting longer than 2 weeks. (To convert white blood cells to number of cells × 109/L, multiply by 0.001.) Prior to the onset of heliotrope, our patient demonstrated the more typical eruption of AOSD (an acral, erythematous, macular dermatitis that worsened with increasing arthralgia). Minor criteria include sore throat, lymph node enlargement, splenomegaly, liver dysfunction (elevated aspartate aminotransferase/alanine aminotransferase ratio), and negative findings for rheumatoid factor and antinuclear antibody. Recent dermatologic literature has reported numerous examples of atypical cutaneous presentation, including urticaria, angioedema, persistent plaques, linear pigmentation, vesiculopustules of the hands and feet, erythema chronicum migrans, and persistent generalized erythema.2-4 A search of the literature revealed only 1 case report of a heliotrope eruption in a patient with AOSD,5 which we can assume is secondary to AOSD for several reasons: (1) the eyelid erythema changed with immunosuppressant medication and disease activity (eg, arthralgias); (2) there was an absence of other causes, including dermatomyositis, as evidenced by laboratory values and physical findings; and (3) the pathology findings were the same as in our patient. Systemic treatment of AOSD includes anti-inflammatory agents, steroids, immunosuppressants, and biological agents, though no treatment has been proven more effective than another in treating skin eruptions.6 For chronic articular disease, disease-modifying antirheumatic drug (DMARD) therapy is generally more beneficial than systemic steroids. Biological agents are effective in steroid-and DMARD-resistant AOSD.6 Overall, a heliotrope eruption should be added to the broad spectrum of AOSD cutaneous findings. Early diagnosis of AOSD based on atypical presentations is essential to diagnose, treat, and prevent systemic complications of the disease. Return to Quiz Case. References 1. Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still's disease. J Rheumatol. 1992;19(3):424-4301578458PubMedGoogle Scholar 2. Affleck AG, Littlewood SM. Adult-onset Still's disease with atypical cutaneous features. J Eur Acad Dermatol Venereol. 2005;19(3):360-36315857467PubMedGoogle ScholarCrossref 3. Dauendorffer JN, Begon E, Petit A, Liote F, Dubertret L, Bachelez H. Adult-onset Still's disease revealed by facial edema. J Am Acad Dermatol. 2008;58(3):533-53518280370PubMedGoogle ScholarCrossref 4. Tomaru K, Nagai Y, Ohyama N, et al. Adult-onset Still's disease with prurigo pigmentosa-like skin eruption. J Dermatol. 2006;33(1):55-5816469087PubMedGoogle ScholarCrossref 5. Saito A, Sato Y, Miyata M, Nishimaki T, Kasukawa R. Two cases of adult Still's disease with atypical rash. Ryumachi. 1998;38(3):516-5229721560PubMedGoogle Scholar 6. Efthimiou P, Georgy S. Pathogenesis and management of adult-onset Still's disease. Semin Arthritis Rheum. 2006;36(3):144-15216949136PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

A 38-Year-Old Woman With Eyelid Discoloration—Diagnosis

Archives of Dermatology , Volume 148 (8) – Aug 1, 2012

A 38-Year-Old Woman With Eyelid Discoloration—Diagnosis

Abstract

Diagnosis: Heliotrope-like manifestation of adult-onset Still disease (AOSD). Diagnosis: Heliotrope-like manifestation of adult-onset Still disease (AOSD). Histopathologic analysis showed dilated vessels with a mild diffuse and perivascular infiltrate of mononuclear cells and neutrophils on hematoxylin-eosin staining, consistent with the patient's known AOSD. A diagnosis of a heliotrope-like manifestation of AOSD was given. The patient was treated with Vaseline (Unilever). Though her...
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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.148.8.947-f
Publisher site
See Article on Publisher Site

Abstract

Diagnosis: Heliotrope-like manifestation of adult-onset Still disease (AOSD). Diagnosis: Heliotrope-like manifestation of adult-onset Still disease (AOSD). Histopathologic analysis showed dilated vessels with a mild diffuse and perivascular infiltrate of mononuclear cells and neutrophils on hematoxylin-eosin staining, consistent with the patient's known AOSD. A diagnosis of a heliotrope-like manifestation of AOSD was given. The patient was treated with Vaseline (Unilever). Though her heliotrope eruption improved somewhat, it never remitted completely. She noted worsening of the heliotrope during a systemic flare (eg, arthralgias) of her AOSD. Diagnosis: Heliotrope-like manifestation of adult-onset Still disease (AOSD). Adult-onset Still disease is a rare, inflammatory, autoimmune disorder with limited discussion in the medical literature, consisting mainly of small-scale prospective studies, case reports, and retrospective studies. The diagnosis is one of exclusion, including consideration of neoplastic, infectious, and other autoimmune causes. The absence of serologic markers in this patient helped exclude other diagnoses such as dermatomyositis. Diagnostic criteria proposed by Yamaguchi et al1 allow physicians to diagnose Still disease after other diseases have been excluded if 5 criteria (more than 2 major criteria) are met. Major criteria include temperature greater than 39°C for more than 1 week; leukocytosis (white blood cell count >10 000/μL with >80% polymorphonuclear cells); typical skin eruption; and arthralgias lasting longer than 2 weeks. (To convert white blood cells to number of cells × 109/L, multiply by 0.001.) Prior to the onset of heliotrope, our patient demonstrated the more typical eruption of AOSD (an acral, erythematous, macular dermatitis that worsened with increasing arthralgia). Minor criteria include sore throat, lymph node enlargement, splenomegaly, liver dysfunction (elevated aspartate aminotransferase/alanine aminotransferase ratio), and negative findings for rheumatoid factor and antinuclear antibody. Recent dermatologic literature has reported numerous examples of atypical cutaneous presentation, including urticaria, angioedema, persistent plaques, linear pigmentation, vesiculopustules of the hands and feet, erythema chronicum migrans, and persistent generalized erythema.2-4 A search of the literature revealed only 1 case report of a heliotrope eruption in a patient with AOSD,5 which we can assume is secondary to AOSD for several reasons: (1) the eyelid erythema changed with immunosuppressant medication and disease activity (eg, arthralgias); (2) there was an absence of other causes, including dermatomyositis, as evidenced by laboratory values and physical findings; and (3) the pathology findings were the same as in our patient. Systemic treatment of AOSD includes anti-inflammatory agents, steroids, immunosuppressants, and biological agents, though no treatment has been proven more effective than another in treating skin eruptions.6 For chronic articular disease, disease-modifying antirheumatic drug (DMARD) therapy is generally more beneficial than systemic steroids. Biological agents are effective in steroid-and DMARD-resistant AOSD.6 Overall, a heliotrope eruption should be added to the broad spectrum of AOSD cutaneous findings. Early diagnosis of AOSD based on atypical presentations is essential to diagnose, treat, and prevent systemic complications of the disease. Return to Quiz Case. References 1. Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still's disease. J Rheumatol. 1992;19(3):424-4301578458PubMedGoogle Scholar 2. Affleck AG, Littlewood SM. Adult-onset Still's disease with atypical cutaneous features. J Eur Acad Dermatol Venereol. 2005;19(3):360-36315857467PubMedGoogle ScholarCrossref 3. Dauendorffer JN, Begon E, Petit A, Liote F, Dubertret L, Bachelez H. Adult-onset Still's disease revealed by facial edema. J Am Acad Dermatol. 2008;58(3):533-53518280370PubMedGoogle ScholarCrossref 4. Tomaru K, Nagai Y, Ohyama N, et al. Adult-onset Still's disease with prurigo pigmentosa-like skin eruption. J Dermatol. 2006;33(1):55-5816469087PubMedGoogle ScholarCrossref 5. Saito A, Sato Y, Miyata M, Nishimaki T, Kasukawa R. Two cases of adult Still's disease with atypical rash. Ryumachi. 1998;38(3):516-5229721560PubMedGoogle Scholar 6. Efthimiou P, Georgy S. Pathogenesis and management of adult-onset Still's disease. Semin Arthritis Rheum. 2006;36(3):144-15216949136PubMedGoogle ScholarCrossref

Journal

Archives of DermatologyAmerican Medical Association

Published: Aug 1, 2012

Keywords: adult onset still's disease,eyelid,abnormal color

References