Possible involvement of SDF-1/CXCL12 in the pathogenesis of Degos disease - 14/12/12
Abstract |
Background |
Degos disease or malignant atrophic papulosis is a rare occlusive vasculopathic disease characterized by pathognomonic cutaneous lesions and frequently fatal systemic involvement. The etiology of malignant atrophic papulosis remains unclear, and there is currently no effective treatment for malignant atrophic papulosis. Several chemokines can potentiate and expand the platelet response to increase thrombus formation. Among these chemokines, this study examined the expression of stromal cell–derived factor (SDF)-1/CXCL12, which is secreted by bone-marrow stromal and endothelial cells, activates megakaryocyte precursors, and costimulates platelet activation.
Objective |
We sought to investigate and compare the expression of SDF-1/CXCL12 in tissue sections taken from 2 patients with Degos disease, 2 patients with other vaso-occlusive diseases, and 2 healthy control subjects.
Methods |
Immunohistochemical staining involving antibodies to SDF-1/CXCL12 was performed on 3 skin biopsy specimens taken from 2 patients with Degos disease, 1 from a patient with antiphospholipid syndrome, 1 from a patient with cryoglobulinemia, and 2 from healthy control subjects.
Results |
Strong SDF-1/CXCL12 staining was observed in the infiltrating inflammatory cells in the perivascular, intravascular, and perineural areas in tissue samples from patients with Degos disease. No staining was observed in samples from patients with antiphospholipid syndrome or cryoglobulinemia or from healthy control subjects.
Limitations |
The number of cases available for evaluation was small. The findings were based primarily on the immunohistochemical results and were not confirmed using other techniques.
Conclusions |
The intense staining of SDF-1/CXCL12 in lesions attributed to Degos disease, demonstrated for the first time to our knowledge in this study, suggests SDF-1/CXCL12 involvement in the pathogenesis of the disease.
Le texte complet de cet article est disponible en PDF.Key words : chemokine, CXCL12, Degos disease, malignant atrophic papulosis, stromal cell–derived factor-1, thrombosis
Abbreviations used : APS, EC, SDF
Plan
Supported by a grant from the Ministry of Health, Labor, and Welfare (Research for Intractable Diseases). |
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Conflicts of interest: None declared. |
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Case 2 has been described in a case report (Demitsu T, Kakurai M, Murata S, Kiyosawa T, Yaoita H. Degos’ disease associated with rheumatoid arthritis. J Dermatol 1997;24:488-90). |
Vol 68 - N° 1
P. 138-143 - janvier 2013 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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