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Research Letter |

The Presence of IgM Antiphospholipid Antibodies in Patients With Henoch-Sch önlein Purpura and Recurrent Palpable Purpura FREE

Tamihiro Kawakami, MD, PhD; Sora Takeuchi, MD; Yoshinao Soma, MD, PhD
[+] Author Affiliations

Author Affiliations: Department of Dermatology, St Marianna University School of Medicine, Kawasaki, Japan.


Arch Dermatol. 2011;147(8):986-988. doi:10.1001/archdermatol.2011.207.
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Published online

Henoch-Sch önlein purpura (HSP) is a primary small-vessel vasculitis characterized by nonthrombocytopenic palpable purpura over the lower extremities. Our research group previously speculated that IgM antiphosphatidylserine-prothrombin complex (anti-PSPT) antibodies could be implicated in disease susceptibility for livedo racemosa.1 Others have reported the presence of antiphospholipid antibodies in patients with cutaneous vasculitis, including HSP.2,3 Whether these antibodies represent an epiphenomenon or they are thrombogenic and/or vasculitic in origin in such patients is unknown.

We reviewed the records of Japanese patients with HSP who were seen at the Department of Dermatology, St Marianna University, Kawasaki, Japan, with the recurrent cutaneous manifestation of palpable purpura on their lower extremities between 2006 and 2010. The patients were diagnosed according to the criteria defined by the American College of Rheumatology4 and the KAWAKAMI algorithm.5 Recurrent palpable purpura was defined as the occurrence of slightly elevated purpura with palpable swelling numerous times during the 3-month follow-up. None of our patients demonstrated any evidence of a coexisting malignant neoplasm, other autoimmune disease, or viral hepatitis. Lupus anticoagulant (LAC) levels and/or the presence of anti-PSPT antibodies were measured according to previously described procedures.1

We identified 12 consecutive patients with HSP and recurrent palpable purpura (4 men and 8 women), mean (SD) age, 32.7 (11.7) years) (Table). All patients demonstrated livedo racemosa over their lower extremities in association with the temporary disappearance of the palpable purpura (Figure). Seven of the 12 patients were shown to be LAC positive (58%). Eleven patients tested positive for IgM anti-PSPT antibodies (92%) but not IgG anti-PSPT antibodies. All the patients were LAC positive and/or demonstrated IgM anti-PSPT antibodies. Renal involvement was noted in 9 patients (75%), and gastrointestinal involvement was noted in 8 (67%). There were 7 patients with both renal and gastrointestinal involvement (58%) and also tested positive for IgM anti-PSPT antibodies.

Place holder to copy figure label and caption
Graphic Jump Location

Figure. Clinical images. A, Palpable purpura on the lower extremities (patient 8). B, Livedo racemosa over the lower extremities when palpable purpura disappeared temporarily (patient 9).

Table Graphic Jump LocationTable. Clinical and Serologic Findings in Patients With HSP and Recurrent Palpable Purpura

We found that all patients with HSP except 1 woman with recurrent palpable purpura tested positive for IgM anti-PSPT antibodies. Livedo racemosa was found in all patients. We suggest that the link between recurrent palpable purpura and livedo racemosa in the present patients may be related to a common immunogenetic pathogenic pathway that includes IgM anti-PSPT antibodies.

Antiphospholipid antibodies likely cause vascular thrombosis by damaging the endothelium. However, there is a controversy whether these antiphospholipid antibodies per se cause a true necrotizing vasculitis. Patterson et al6 focus on the role of endothelial cell injury as a cofactor in the evolution of small-vessel vasculitis. Based on these findings, IgM anti-PSPT antibodies may be involved in damaging the endothelial cells, which could lead to the formation of recurrent palpable purpura as well as livedo racemosa.

More than half of patients with HSP who have recurrent palpable purpura also have both renal and gastrointestinal involvement. Serum IgM anti-PSPT antibody levels could be related to the common pathogenic factors that trigger the development of severe HSP, gastrointestinal symptoms, and HSP nephritis. As such, the IgM-antigen complex, as a heavy molecule, could be preferentially trapped in the capillaries of kidney and the digestive tract.

ARTICLE INFORMATION

Correspondence: Dr Kawakami, Department of Dermatology, St Marianna University School of Medicine, 2-16-1 Sugao, Miyamae-ku, Kawasaki, Kanagawa 216-8511, Japan (tami@marianna-u.ac.jp).

Accepted for Publication: December 25, 2010.

Author Contributions: Dr Kawakami had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. Study concept and design: Kawakami, Takeuchi, and Soma. Acquisition of data: Kawakami. Analysis and interpretation of data: Kawakami. Drafting of the manuscript: Kawakami and Takeuchi. Critical revision of the manuscript for important intellectual content: Kawakami and Soma. Obtained funding: Kawakami. Administrative, technical, and material support: Kawakami and Takeuchi. Study supervision: Kawakami and Soma.

Financial Disclosure: None reported.

Kawakami T, Yamazaki M, Mizoguchi M, Soma Y. Differences in anti-phosphatidylserine-prothrombin complex antibodies and cutaneous vasculitis between regular livedo reticularis and livedo racemosa.  Rheumatology (Oxford). 2009;48(5):508-512
PubMed   |  Link to Article
Gantcheva M, Angelova I. Antiphospholipids in cutaneous vasculitis.  Clin Dermatol. 1999;17(6):619-624
PubMed   |  Link to Article
Mekkes JR, Loots MA, van der Wal AC, Bos JD. Increased incidence of hypercoagulability in patients with leg ulcers caused by leukocytoclastic vasculitis.  J Am Acad Dermatol. 2004;50(1):104-107
PubMed   |  Link to Article
Mills JA, Michel BA, Bloch DA,  et al.  The American College of Rheumatology 1990 criteria for the classification of Henoch-Sch önlein purpura.  Arthritis Rheum. 1990;33(8):1114-1121
PubMed   |  Link to Article
Kawakami T. New algorithm (KAWAKAMI algorithm) to diagnose primary cutaneous vasculitis.  J Dermatol. 2010;37(2):113-124
PubMed   |  Link to Article
Patterson CC, Ross P Jr, Pope-Harman AL, Knight DA, Magro CM. Alpha-1 anti-trypsin deficiency and Henoch-Sch önlein purpura associated with anti-neutrophil cytoplasmic and anti-endothelial cell antibodies of immunoglobulin-A isotype.  J Cutan Pathol. 2005;32(4):300-306
PubMed   |  Link to Article

Figures

Place holder to copy figure label and caption
Graphic Jump Location

Figure. Clinical images. A, Palpable purpura on the lower extremities (patient 8). B, Livedo racemosa over the lower extremities when palpable purpura disappeared temporarily (patient 9).

Tables

Table Graphic Jump LocationTable. Clinical and Serologic Findings in Patients With HSP and Recurrent Palpable Purpura

References

Kawakami T, Yamazaki M, Mizoguchi M, Soma Y. Differences in anti-phosphatidylserine-prothrombin complex antibodies and cutaneous vasculitis between regular livedo reticularis and livedo racemosa.  Rheumatology (Oxford). 2009;48(5):508-512
PubMed   |  Link to Article
Gantcheva M, Angelova I. Antiphospholipids in cutaneous vasculitis.  Clin Dermatol. 1999;17(6):619-624
PubMed   |  Link to Article
Mekkes JR, Loots MA, van der Wal AC, Bos JD. Increased incidence of hypercoagulability in patients with leg ulcers caused by leukocytoclastic vasculitis.  J Am Acad Dermatol. 2004;50(1):104-107
PubMed   |  Link to Article
Mills JA, Michel BA, Bloch DA,  et al.  The American College of Rheumatology 1990 criteria for the classification of Henoch-Sch önlein purpura.  Arthritis Rheum. 1990;33(8):1114-1121
PubMed   |  Link to Article
Kawakami T. New algorithm (KAWAKAMI algorithm) to diagnose primary cutaneous vasculitis.  J Dermatol. 2010;37(2):113-124
PubMed   |  Link to Article
Patterson CC, Ross P Jr, Pope-Harman AL, Knight DA, Magro CM. Alpha-1 anti-trypsin deficiency and Henoch-Sch önlein purpura associated with anti-neutrophil cytoplasmic and anti-endothelial cell antibodies of immunoglobulin-A isotype.  J Cutan Pathol. 2005;32(4):300-306
PubMed   |  Link to Article

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