Sweet’s syndrome associated with antiphospholipid antibody syndrome

Links checked 16/01/18.

Sweet’s syndrome (SS) can develop secondary to autoimmune conditions. This is a rare case of SS developing in association with antiphospholipid antibody syndrome (APS) (Alves et al, 2017).

Case-study.

A 55-year-old man developed skin lesions, fever, joint pain in hands, and the inflammatory eye condition, episcleritis. All of these symptoms were consistent with SS.

Blood tests revealed increased levels of inflammation in the body, and a biopsy of a lesion showed SS. The patient also had a stroke, cavernous sinus thrombosis (blood clot in the hollow spaces located under the brain, and behind each eye socket), and was positive for antiphospholipid antibodies. He was then given a diagnosis of SS-associated APS. Unfortunately, the patient did not respond well to steroids, the most common treatment for SS. The medications methotrexate and hydroxychloroquine were started but stopped, due to bone marrow suppression. Treatment with the biologic, infliximab, led to significant improvement.

Are there any other cases of SS developing in association with APS?

Yes. An earlier case of SS-associated APS was reported in a man with multiple pulmonary emboli (blockages in the artery that carries blood from the heart to the lungs) (Cohen, 2007). He was given the steroid, prednisone, which initially worked well, but experienced SS flare-ups when the dosage was reduced. In 2008, Sweet-like lesions were reported in a 37-year-old man with insufficient blood flow to the brain and APS (Tomb et al, 2008).

What is APS?

APS, or Hughes syndrome, is an autoimmune condition where the immune system produces abnormal antibodies called antiphospholipid antibodies (NHS Choices, 2015). These antibodies then target proteins attached to fat molecules (phospholipids), making the blood more likely to clot.

What are the symptoms of APS?

Symptoms of APS include high blood pressure; deep vein thrombosis; stroke; heart attack; pulmonary embolism. The syndrome can also cause other symptoms, which are sometimes mistaken for the symptoms of multiple sclerosis – balance and mobility problems; vision problems; speech and memory problems; tingling or pins and needles; fatigue; repeat headaches or migraines.

Further information.

What causes Sweet’s syndrome? Includes information on SS triggers.

References.

Alves, L., Castro, F., Sousa, T., Alverenga, C., Abreau, I., Padova, P., Costa, G. and Souza, E. (2017) Sweet’s syndrome associated with antiphospholipid antibody syndrome – case report. Brazilian Journal of Rheumatology, 57(suppl 1): 371 (Science Direct). Article in Portuguese. Use translate.

Cohen, P. (2007) Clinical description: Figure 4, in Sweet’s syndrome – a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet Journal of Rare Diseases; 2: 34 (PMC).

NHS Choices (2015) Antiphospholipid syndrome (APS) (online). Last reviewed on 9/11/15. Accessed on 16/01/18.

Tomb, R., Maalouf, E. and Attoui, S. (2008) [Cutaneous nodular lesions and antiphospholipid syndrome]. Annales de dermatologie et de vénéréologie, Jun-Jul;135(6-7):484-7 (EM|Consulte). Full-text in French.

2012-2018 Sweet’s Syndrome UK

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