Journal Information
Vol. 13. Issue 4.
Pages 233-234 (July - August 2017)
Vol. 13. Issue 4.
Pages 233-234 (July - August 2017)
Images in Clinical Rheumatology
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Cutaneous Manifestations in Antiphospholipid Syndrome
Manifestaciones cutáneas del síndrome antifosfolípido
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Lucía Campos-Muñoza,
Corresponding author
luciacampos78@hotmail.com

Corresponding author.
, Alejandro Fueyo-Casadoa, Jose Antonio Cortés-Torob, Eduardo López-Brana
a Servicio de Dermatología, Hospital Clínico San Carlos, Madrid, Spain
b Servicio de Anatomía Patológica, Hospital Clínico San Carlos, Madrid, Spain
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The patient was a 54-year-old woman who had no significant previous medical history. She presented with pruriginous lesions that had started on her legs 1 year earlier and had spread to her thighs, buttocks and upper limbs. They were accompanied by slight fever and arthralgia. Physical examination revealed an irregular and diffuse violaceous reticulated discoloration that in certain regions had become purpuric. They were very suggestive of livedo reticularis or livedo racemosa (Fig. 1a). She also had distal purpura on the second toe of her right foot (Fig. 1b). The differential diagnosis included cryoglobulinemia, polyarteritis nodosa, livedoid vasculopathy, cholesterol crystal embolism concomitant with arteriosclerosis, polycythemia vera, essential thrombocytopenia, atrial myxoma, etc. Skin biopsy revealed hyaline thrombi in the papillary dermal vessels, with no inflammatory infiltrate in the vascular wall (Fig. 1c, d). An antibody positive for lupus anticoagulant was detected, and was confirmed 12 weeks later. She was treated with acetylsalicylic acid, chloroquine and prednisone, which led to the disappearance of the systemic clinical manifestations, complete resolution of the distal purpura and attenuation of the livedo racemosa, which became less evident.

Fig. 1.

(a) Livedo reticularis in its variant, livedo racemose, involving both lower limbs in their entirety. (b) Purpuric discoloration on the second toe. (c) Skin displayed epidermal necrosis and the presence of hyaline thrombi in the papillary dermal vessels (hematoxylin–eosin, 100×). (d) Greater detail of the thrombi (hematoxylin–eosin, 20×).

(1.16MB).

Antiphospholipid syndrome (APS) is a prothrombotic state characterized by the presence of antiphospholipid antibodies (anticardiolipin antibodies, lupus anticoagulant or anti-β2-glycoprotein I). The diagnosis also requires evidence of arterial, venous or microcirculation thrombosis or a history of recurrent fetal loss.1–3 It is also frequently accompanied by cutaneous manifestations (which occur in up to 40%–50% of the patients). Livedo reticularis is the disorder most widely reported and, on occasion, they may be the first sign of the disease.4,5 Other possible lesions are skin ulcers, purpuric lesions, skin necrosis, superficial thrombophlebitis and atrophie blanche-type lesions.6–8 Our familiarity with these manifestations would enable us to make a diagnosis as early as possible and prevent potential complications of the disease.

Ethical DisclosuresProtection of human and animal subjects

The authors declare that no experiments were performed on humans or animals for this study.

Confidentiality of data

The authors declare that they have followed the protocols of their work center on the publication of patient data.

Right to privacy and informed consent

The authors have obtained the written informed consent of the patients or subjects mentioned in the article. The corresponding author is in possession of this document.

Conflicts of Interest

The authors declare they have no conflicts of interest.

References
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M. Abreu, A. Darowski, D. Wahh, M.C. Amigo.
The relevance of non-criteria clinical manifestations of antiphospholipid syndrome: 14th International Congress on Antiphospholipid Antibodies Technical Task Force Report on Antiphospholipid Syndrome Clinical Features.
Autoimmun Rev, 14 (2015), pp. 401-414
[2]
C. Garcia-Garcia.
Anticuerpos antifosfolípido y síndrome antifosfolípido: actitudes diagnósticas y terapéuticas.
Actas Dermasifiliogr, 98 (2007), pp. 16-23
[3]
R.A. Asherson, C. Frances, L. Laccarinol.
The antiphopholipid antibody syndrome: diagnosis, skin manifestations and current therapy.
Clin Exp Rheumatol, 24 (2006), pp. S46-S51
[4]
S.R. Sangle, D.P. D’Cruz.
Livedo reticularis: an enigma.
Isr Med Assoc J, 17 (2015), pp. 104-107
[5]
T. Pinto-Almeida, M. Caetano, M. Sanches.
Cutaneous manifestations of antiphospholipid syndrome.
Acta Reumatol Port, 38 (2013), pp. 10-18
[6]
C. Frances, S. Barete, A. Soria.
Dermatologic manifestations of the antiphospholipid syndrome.
Rev Med Intern, 33 (2012), pp. 200-205
[7]
S. Caporuscio, M.L. Sorgi, S. Nistico.
Cutaneous manifestations in antiphospholipid syndrome.
Int J Immunopathol Pharmacol, 28 (2015), pp. 270-273
[8]
M.R. Perello-Alzamora, J.C. Santos-Duran, A. Santos-Briz.
Widespread cutaneous necrosis as the first clinical manifestation of secondary antiphospholipid syndrome.
Actas Dermosifiliogr, 33 (2012), pp. 200-205

Please cite this article as: Campos-Muñoz L, Fueyo-Casado A, Cortés-Toro JA, López-Bran E. Manifestaciones cutáneas del síndrome antifosfolípido. Reumatol Clin. 2017;13:233–234.

Copyright © 2016. Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología
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