Livedo, dementia, thrombocytopenia, and endotheliitis without antiphospholipid antibodies: Seronegative antiphospholipid-like syndrome - 24/08/11
, Luc Darnige, MD b, François Glowacki, MD c, Marie-Christine Copin, MD, PhD d, Elisabeth Martin De Lassalle, MD d, Emmanuel Delaporte, MD, PhD a, Eric Auxenfants, MD e
Reprint requests: Arnaud Duval, MD, Hôpital Saint Louis, Dermatologie, Service du Pr Dubertret, 1 Avenue Claude Vellefaux, 75475 Paris cedex 10, France.Abstract |
We report a 51-year-old woman who presented with dementia, livedo racemosa, polyarthralgia, mild renal insufficiency, proteinuria, and thrombocytopenia. Cutaneous and renal biopsy specimens both showed an identical specific occlusive arteriolopathy consistent with Sneddon syndrome and antiphospholipid syndrome. However, no antiphospholipid antibodies were detected and we, therefore, diagnosed seronegative antiphospholipid-like syndrome. We discuss the nosology of this entity and its association with non-antiphospholipid antibody–related Sneddon syndrome. The common denominator of Sneddon syndrome and antiphospholipid syndrome with or without antiphospholipid antibodies seems to be the endothelial damage and occlusive arteriolopathy. Skin biopsy is useful to confirm the diagnosis of seronegative antiphospholipid-like syndrome.
Le texte complet de cet article est disponible en PDF.Key words : antiphospholipid syndrome, biopsy, Hughes syndrome, kidney, livedo, seronegative, skin, Sneddon
Abbreviations used : APA, APS, LA, SNAPS, SS
Plan
| Funding sources: None. |
|
| Conflicts of interest: None declared. |
Vol 61 - N° 6
P. 1076-1078 - décembre 2009 Retour au numéro
Article précédent
- Improvement of recurrent urticaria in a patient with Schnitzler syndrome associated with B-cell lymphoma with combination rituximab and radiotherapy
- Hiroyuki Murota, Yukiko Shoda, Tomohiko Ishibashi, Hiroyuki Sugahara, Itaru Matsumura, Ichiro Katayama
- Freedom of expression or protectionism in the debate on sentinel node biopsy in melanoma
- J. Meirion Thomas
Bienvenue sur EM-consulte, la référence des professionnels de santé.
L’accès au texte intégral de cet article nécessite un abonnement.
Déjà abonné à cette revue ?