Introduction Raynaud’s disease (RD) is a vascular disorder defined as an exaggerated vasoconstriction and vasospasm of the digital arteries and arterioles. It is predominately triggered by cold or emotional stress1. It was first described by Maurice Raynaud in 1862 and later studied by Sir Thomas Lewis in 19302. The reported incidence is approximately 20% of healthy women of childbearing age3. It predominately affects the fingers and less frequently, the toes but this phenomenon can also affect the nipples. RD of the nipple is a recognised condition affecting breast feeding women but on occasion this can present in the antenatal period, as seen in this case. The first report that was published highlighting a link between nipple vasospasm and the Raynaud’s phenomenon was a paper by Coates in 1992. In this report, a breastfeeding mother describes nipple symptoms similar to those associated with the RD4. The common theme is extremely painful breastfeeding, precipitation of symptoms by cold stimuli and biphasic or tri-phasic colour change of the nipple5. The colour change reflects the process occurring within the arterioles: white reflecting the ischaemic phase, then blue during deoxygenation and finally red as the blood flow returns. Multiple case reports have followed over the years since Coates’ paper now cementing this as a recognised condition. However this condition is frequently misdiagnosed by health professionals as a fungal infection or cellulitis. It is an important differential to consider, avoiding unnecessary use of antibiotics and antifungals.
Case Report: A 44 year old woman presented at 18 weeks gestation complaining of nipple soreness. She reported bilateral breast pain occurring 7-8 times a day, lasting for 5-10 minutes at a time. The nipples also changed colour- becoming white, then blue, then eventually red. The nipples felt cold to touch. Examination of the breasts was normal. In her pregnancy she had a cervical suture inserted at 13/40 due to a previous mid-trimester miscarriage and was under the care of the preterm labour clinic. She had no significant past medical history and was a non-smoker. She had similar symptoms in her previous pregnancy but was never investigated before the pregnancy miscarried. Following this initial consultation by the obstetric consultan, she was referred to the breast team and rheumatology team for review. She was reviewed by the breast team when she was 21 weeks pregnant. She underwent a breast ultrasound which showed normal retroareolar ducts with no abnormal masses, but the doppler flow did demonstrate increased vascularity of the right nipple diffusely and normal left nipple vasculature. She was diagnosed with Raynaud’s Syndrome of the nipple. Conservative measures were advised as initial management. This included avoiding exposure to cold and staying warm. She was advised to avoid stimulants e.g. caffeine, and to minimise anxiety or emotional where possible. She was also advised to wear a non-wired firm supportive bra. At 23 weeks gestation she was reviewed by rheumatology who came to the same diagnosis. Medications were discussed at this point, mainly aimed at pharmacologic therapy with a vasodilating mechanism of action. Dihydropyridine calcium channel blockers (DHP CCBs) are the first-line pharmacologic treatment for RD, including amlodipine and nifedipine. As she had noticed some improvement from conservative measures and had anxieties surrounding this pregnancy due to her history, she declined any medical treatment. Her symptoms did improve through the pregnancy and the episodes of pain became less frequent and severe. At 36+6 the cervical suture was removed and she underwent induction of labour at 37+4 due to ongoing heavy red vaginal loss. She had a spontaneous vaginal delivery followed by a 2.5 litre postpartum haemorrhage. She decided to bottle feed due to fear of exacerbation of the condition if she breast fed.
Discussion Pathophysiology: RD is common and doesn’t usually cause any severe symptoms7. There is episodic vasospasm of the arteries in the extremities, causing pallor followed by cyanosis and/or redness8. In RD, smaller arteries that supply blood to the skin narrow, limiting blood circulation to affected areas (vasospasm). This usually involves the fingers of the hand, occasionally the toes of the feet and rarely, as in this case, the nipples of the breast. The pathophysiology of Raynaud’s is not fully understood. In pregnancy the symptoms often improve as there is increased surface blood flow9. Classification: There are two main types of RD — primary and secondary. • Primary Raynaud’s – the cause isn't known. Primary Raynaud's is more common and tends to be less severe than secondary Raynaud's. • Secondary Raynaud’s – caused by an underlying disease, condition, or other factor. This type of Raynaud's is often called Raynaud's phenomenon10.
Incidence and Prevalence: It has been reported that the prevalence of RD is between 3-21%, with an incidence of 2-3% over 10 years. It is often diagnosed in the 2nd or 3rd decade of life, however symptoms and diagnosis can occur at any age. It is thought that 13% with RD have an underlying pathology (secondary Raynaud’s)11. Women are more likely than men to have RD, also known as Raynaud's phenomenon or syndrome. It appears to be more common in people who live in colder climates. Raynaud's symptoms can occur on the nipple, which are not only painful but also likely to be mistaken for other breastfeeding complications such as thrush or cracked nipples. It is rare for the phenomenon to occur antenatal and much more commonly occurs in the postnatal period in breast feeding mothers. Because the breast pain associated with RD is so severe it is often mistaken for Candida albicans infection. It is not unusual for mothers who have RD of the nipple to be treated inappropriately and often repeatedly for Candida albicans infections with topical or systemic antifungal agents. Management: In both pregnant and non-pregnant people the management of Raynaud’s Phenomenon may require the expertise of several professions. Basic management involves avoiding precipitants such as cold weather, and keeping the body warm in an attempt to improve the peripheral circulation. Exercise, stopping smoking and reducing stress levels have also been suggested as being beneficial. Medication may also be used in the treatment of RD. Nifedipine (a form of calcium channel blocker [CCB]) may be initiated by primary care physicians as prophylactic treatment of RD. However it has been reported that in the majority of people (up to 75%) unpleasant side effects can develop, for example, headache, palpitations and oedema. In the case of RD of the breast involving the nipple, a key part of the management is breast support. Underwear designed to fully support the breast and increase peripheral blood supply can often alleviate symptoms. Conclusion: Raynaud's nipple in pregnancy is not widely reported and is frequently misdiagnosed. The symptoms are often attributed to other conditions. It is an important diagnosis to consider as this avoids the unnecessary use of anti-fungals and antibiotics. Conservative methods can be used to help relieve symptoms, however if these are ineffective pharmacological agents are available. If the diagnosis is in doubt referral to secondary care and specialist teams is appropriate as it is important to rule put other conditions affecting the breast.
References: 1 Reilly A, Snyder B. Raynaud's phenomenon: whether it's primary or secondary, there is no cure, but treatment can alleviate symptoms. AJN. 2005;105:56-65. 2 Lawlor-Smith LS, Lawlor-Smith CL. Vasospasm of the nipple--a manifestation of Raynaud's phenomenon: case reports. BMJ. 1997;314: 644-64 3 Anderson JE, Held N, Wright K. Raynaud's phenomenon of the nipple: a treatable cause of painful breastfeeding. Pediatrics. 2004;113:e360-e364. 4 Coates M. Nipple pain related to vasospasm in the nipple? J Hum Lact. 1992;8:153. 5 Morino, Carolyn, and Susan M. Winn. "Raynaud's phenomenon of the nipples: an elusive diagnosis." Journal of Human Lactation, May 2007, p. 191 6 Sore Nipples and Breastfeeding. Love and Breast Milk. http://loveandbreastmilk.com/wp/sore-nipples/ (last accessed 18/03/19) 7 Raynauds. NHS. https://www.nhs.uk/conditions/raynauds/ (last accessed 18/03/19) 8 The diagnosis and treatment of Raynaud’s phenomenon: a practical approach, 2007. Pope JE. Drugs. 2007;67(4):517-25. DOI: 10.2165/00003495-200767040-00003 (https://www.ncbi.nlm.nih.gov/pubmed/17352512 [last accessed 18/03/19]) 9 Raynaud’s. National Heart, Lung and Bone Institute. https://www.nhlbi.nih.gov/health-topics/raynauds https://www.nhlbi.nih.gov/health-topics/ raynauds (last accessed 18/03/19) 10 Raynaud’s Disease. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/raynauds-disease/symptoms... (last accessed 18/03/19) 11 Raynaud’s Phenomenon. NICE. Clinical Knowledge Summaries https://cks.nice.org.uk/raynauds-phenomenon#!diagnosisSub https:// www.mayoclinic.org/diseases-conditions/raynauds-disease/symptoms-causes/... (last accessed 18/03/19)