We're unable to sign you in at this time. Please try again in a few minutes.
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
The Cutting Edge: Challenges in Medical and Surgical Therapies |

Retiform Purpura and Digital Gangrene Secondary to Antiphospholipid Syndrome Successfully Treated With Sildenafil

Mercedes E. Gonzalez, MD; Philip Kahn, MD; Harper N. Price, MD; Hideko Kamino, MD; Julie V. Schaffer, MD
Arch Dermatol. 2011;147(2):164-167. doi:10.1001/archdermatol.2011.4.
Text Size: A A A
Published online


Miyakis  SLockshin  MDAtsumi  T  et al.  International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 2006;4 (2) 295- 306
PubMed Link to Article[[XSLOpenURL/10.1111/jth.2006.4.issue-2]]
Abernethy  MLMcGuinn  JLCallen  JP Widespread cutaneous necrosis as the initial manifestation of the antiphospholipid antibody syndrome. J Rheumatol 1995;22 (7) 1380- 1383
Paira  SRoverano  SZunino  AOliva  MEBertolaccini  ML Extensive cutaneous necrosis associated with anticardiolipin antibodies. J Rheumatol 1999;26 (5) 1197- 1200
Asherson  RAFrancès  CIaccarino  L  et al.  The antiphospholipid antibody syndrome: diagnosis, skin manifestations and current therapy. Clin Exp Rheumatol 2006;24 (1) (suppl 40)S46- S51
Levine  JSBranch  DWRauch  J The antiphospholipid syndrome. N Engl J Med 2002;346 (10) 752- 763
PubMed Link to Article[[XSLOpenURL/10.1056/NEJMra002974]]
Jicha  DLCaty  MGLillehei  CW Primary antiphospholipid syndrome in a child with lower extremity arterial thrombosis. J Pediatr Surg 1994;29 (12) 1519- 1520
PubMed Link to Article[[XSLOpenURL/10.1016/0022-3468(94)90200-3]]
Avcin  TCimaz  RSilverman  ED  et al.  Pediatric antiphospholipid syndrome: clinical and immunologic features of 121 patients in an international registry. Pediatrics 2008;122 (5) e1100- e1107
PubMed Link to Article[[XSLOpenURL/10.1542/peds.2008-1209]]
Cervera  RPiette  JCFont  J  et al. Euro-Phospholipid Project Group, Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum 2002;46 (4) 1019- 1027
PubMed Link to Article[[XSLOpenURL/10.1002/(ISSN)1529-0131]]
Gibson  GESu  WPDPittelkow  MR Antiphospholipid syndrome and the skin. J Am Acad Dermatol 1997;36 (6, pt 1) 970- 982
PubMed Link to Article[[XSLOpenURL/10.1016/S0190-9622(97)80283-6]]
Weinstein  SPiette  W Cutaneous manifestations of antiphospholipid antibody syndrome. Hematol Oncol Clin North Am 2008;22 (1) 67- 77
PubMed Link to Article[[XSLOpenURL/10.1016/j.hoc.2007.10.011]]
Pierangeli  SSChen  PPRaschi  E  et al.  Antiphospholipid antibodies and the antiphospholipid syndrome: pathogenic mechanisms. Semin Thromb Hemost 2008;34 (3) 236- 250
PubMed Link to Article[[XSLOpenURL/10.1055/s-0028-1082267]]
Wilson  LSElbatarny  HSCrawley  SWBennett  BMMaurice  DH Compartmentation and compartment-specific regulation of PDE5 by protein kinase G allows selective cGMP-mediated regulation of platelet functions. Proc Natl Acad Sci U S A 2008;105 (36) 13650- 13655
PubMed Link to Article[[XSLOpenURL/10.1073/pnas.0804738105]]
Fries  RShariat  Kvon Wilmowsky  HBöhm  M Sildenafil in the treatment of Raynaud's phenomenon resistant to vasodilatory therapy. Circulation 2005;112 (19) 2980- 2985
Lichtenstein  JR Use of sildenafil citrate in Raynaud's phenomenon: comment on the article by Thompson et al. Arthritis Rheum 2003;48 (1) 282- 283
PubMed Link to Article[[XSLOpenURL/10.1002/art.10628]]
Gore  JSilver  R Oral sildenafil for the treatment of Raynaud's phenomenon and digital ulcers secondary to systemic sclerosis. Ann Rheum Dis 2005;64 (9) 1387
PubMed Link to Article[[XSLOpenURL/10.1136/ard.2004.034488]]
Heymann  WR Sildenafil for the treatment of Raynaud's phenomenon. J Am Acad Dermatol 2006;55 (3) 501- 502
PubMed Link to Article[[XSLOpenURL/10.1016/j.jaad.2006.04.037]]
Kumana  CRCheung  GTYLau  CS Severe digital ischaemia treated with phosphodiesterase inhibitors. Ann Rheum Dis 2004;63 (11) 1522- 1524
PubMed Link to Article[[XSLOpenURL/10.1136/ard.2003.015677]]
Gertner  E Treatment with sildenafil for the healing of refractory skin ulcerations in the antiphospholipid syndrome. Lupus 2003;12 (2) 133- 135
PubMed Link to Article[[XSLOpenURL/10.1191/0961203303lu257cr]]
Figures in this Article

Sign in

Purchase Options

• Buy this article
• Subscribe to the journal
• Rent this article ?

First Page Preview

View Large
First page PDF preview


Place holder to copy figure label and caption
Figure 1

Clinical photographs of hemorrhagic bulla and retiform purpura. A, Hemorrhagic bulla on the distal left first toe and necrotic ulcer with loss of the nail on the second toe. B, Retiform purpura on the left first and second toes, and dusky reticular erythema on the third and fourth toes.

Graphic Jump Location
Place holder to copy figure label and caption
Figure 2

Fibrin thrombi in the lumens of small and medium-sized blood vessels throughout the mid and deep reticular dermis, with partial necrosis of eccrine glands and the overlying epidermis. A, Hematoxylin-eosin, original magnification ×10; B, hematoxylin-eosin, original magnification ×40.

Graphic Jump Location
Place holder to copy figure label and caption
Figure 3

Three months after presentation, the patient had lost only the second toenail (A) and a small amount of tissue from the tips of her first and second toes (B).

Graphic Jump Location




Also Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Please click the checkbox indicating that you have read the full article in order to submit your answers.
Your answers have been saved for later.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.


Some tools below are only available to our subscribers or users with an online account.

5 Citations

Sign in

Purchase Options

• Buy this article
• Subscribe to the journal
• Rent this article ?

Related Content

Customize your page view by dragging & repositioning the boxes below.

Articles Related By Topic
Related Collections
PubMed Articles