Is axillary botulinum toxin efficient in controlling secondary Raynaud’s phenomenon? A case report

Raynaud’s phenomenon when secondary to underlying systemic disease such as systemic sclerosis occurs early in the disease course and progression can bring significant morbidity such as pain, digital ulceration, and necrosis. Standard medical therapies are aimed at promoting distal arterial vasodilation but are often inadequate in managing Raynaud’s phenomenon. Options for refractory cases include surgical and chemical sympathectomy with Botulinum neurotoxin type A (BoNT/A) hand injections but the latter can be associated with transient hand weakness. We describe the case of a 35-year-old woman with undifferentiated connective tissue disease, Raynaud’s phenomenon, and concomitant primary focal axillary hyperhidrosis for which she received axillary BoNT/A therapy every 6 months who noted significant improvement in her Raynaud’s phenomenon and hand arthralgias for 5 months following the axillary injections. This effect remained durable after 24 months of therapy. This improvement in Raynaud’s phenomenon after axillary BoNT/A has not been previously described.

Methods for assessment of microcirculation

To date, many methods have entered rheumatological practice in order to evaluate more accurately the microcirculation. Most of them are non-invasive or minimally invasive, easily accessible, providing different information depending on the specifics of the study. Over the years, some of them (such as chromametry and volumetry) have lost their routine application and have acquired rather historical significance, while others have become an integral part of the rheumatological armentarium. An example of this is video capillaroscopy, which, through its dynamic development over the last 20 years, has evolved in the knowledge of changes in microcirculation in systemic sclerosis, as well as in other systemic connective tissue diseases. The expansion of knowledge in this area has led to the updating of the classification criteria for systemic sclerosis, allowing the addition of capillaroscopic changes as recognized by the European League against Rheumatism (EULAR). Another important indication for performing video capillaroscopy is the differentiation of primary from secondary Raynaud's phenomenon. Laser Doppler perfusion imaging is also used to detect secondary Raynaud's phenomenon in order to distinguish whether reduced blood flow is due to systemic autoimmune disease. Thermography also has a helpful role in diagnosing Raynaud's phenomenon, but unlike the other two methods, it has a much more limited application due to the lack of discriminative ability to distinguish nutritional from thermoregulatory blood flow, which leads to erroneous conclusions in pathological conditions. Venous occlusive plethysmography is one of the "gold standards" in the assessment of vascular function in health and disease and is an accurate, reproducible and convenient method to assess the effect of new vasoactive drugs. However, its application in everyday rheumatological practice is quite limited.

Management of severe Raynaud’s Phenomenon secondary to autoimmune vasculopathy in a young woman

Raynaud’s phenomenon as a cause of acute limb ischaemia in the warmer climates of Sub-Saharan Africa region is uncommon because it is usually thought of as a disease common in cold weather. The prevalence of connective tissue diseases among Black Africans is increasing, and these conditions are associated with secondary Raynaud’s phenomenon and ischaemic digital lesions. We present the case of a 36-year old female with dermatomyositis/systemic sclerosis overlap and secondary Raynaud’s phenomenon who presented with acute limb ischemia (wet gangrene of all digits) in a Tertiary Hospital in Ghana. Young patients presenting with acute limb ischaemia should also be screened for an underlying connective tissue disease. In patients with connective tissue disease, the onset of digital vasculopathy can be rapid and progressive, hence treatment must be prompt and comprehensive to enable better clinical outcomes.

Raynaud’s phenomenon and related vasospastic disorders

Raynaud’s phenomenon, which is characterized by episodic digital pallor, cyanosis and rubor upon exposure to cold environment or to stress, is relatively common, although the prevalence depends on the climate. Still, it is under-diagnosed, under-treated, and often confused with other conditions. Primary Raynaud’s phenomenon (i.e., Raynaud disease) must be distinguished from secondary Raynaud’s phenomenon (i.e., Raynaud syndrome) as long-term morbidity and outcomes differ vastly between the two conditions. Additionally, the practitioner must differentiate between Raynaud’s phenomenon and related vascular disorders, such as acrocyanosis, pernio, and livedo reticularis. In this article, we review differences between the conditions and suggest an approach to diagnosis and treatment strategy for these disorders.