Background Fibromuscular Dysplasia (FMD) is an non-atherosclerotic disease associated with hypertension, headache, dissection, stroke, and aneurysm. The etiology is unknown but hypothesized to involve genetic and environmental components. Previous studies suggest a possible overlap of FMD with other connective tissue diseases that present with dissections and aneurysms. The aim of this study was to investigate the prevalence of connective tissue physical features in FMD. Methods and Results 142 FMD patients were consecutively enrolled at a single referral center (97.9% female, 92.3% had multifocal FMD). Data are reported for 139 female patients. Moderately severe myopia (29.1%), high palate (33.1%), dental crowding (29.7%), and early onset arthritis (15.6%) were prevalent features. Classic connective features such as hypertelorism, cleft palate, and hypermobility were uncommon. Frequency of systemic connective tissue features was compared between FMD patients with a high vascular risk profile (having had ≥1 dissection and/or ≥2 aneurysms) and those with a standard vascular risk profile. History of spontaneous pneumothorax (5.9% high risk vs. 0% standard risk) and atrophic scarring (17.3% high risk vs. 6.8% standard risk) were significantly more prevalent in the high risk group, p<0.05. High palate was observed in 43.1% of the high risk group vs. 27.3% in the standard risk group, p=0.055. Conclusions In a cohort of women with FMD, there was a prevalence of moderately severe myopia, high palate, dental crowding, and early onset osteoarthritis. However, a characteristic phenotype was not discovered. Several connective tissue features such as high palate and pneumothorax were more prominent among FMD patients with a high vascular risk profile.
Fibromuscular dysplasia (FMD) is a noninflammatory, nonatherosclerotic arterial disease that can affect any artery but most commonly affects the renal, carotid, and vertebral arteries, predisposing to stenosis, aneurysm, dissection, and/or tortuosity. 1-3 While headache is a common symptom reported by patients with FMD, little is known about this relationship. 4,5 The etiology of FMD is not known, nor is it understood why such a high percentage of patients with FMD experience headaches. 6 In addition to FMD, patients with associated vascular abnormalities, such as intracranial aneurysm and cervical carotid and vertebral artery dissection, also commonly experience headache. 5,7,8 The aim of this study was to report the frequency and characteristics of headache in a cohort of patients enrolled in the United States Registry for FMD and to identify clinical factors associated with the presence of this symptom. From 2009 through 2016, 1433 adult patients 18 years of age and older were enrolled in the Registry. Eligibility for enrollment was determined by site investigators based on identifying the characteristic appearance of multifocal or focal FMD from vascular imaging in at least one vascular bed. 2,5 Eligible subjects provided written informed consent. Demographic and clinical data, family history, headache-related variables, presenting signs and symptoms at the time of FMD diagnosis, and medications taken at the time of enrollment were recorded. An assessment of headache at initial Registry enrollment included query for presence of headache, type of headache (migraine or other), frequency of headache, whether headache was associated with menses, and whether headache(s) required suppressive medication. The Institutional Review Board (IRB) at the University of Michigan, the Data Coordinating Center for the US Registry of FMD, approved this study (HUM00022250). In addition, all participating sites in the Registry obtained individual institutional IRB approval. Patients were categorized into two groups: those with and those without reported headaches at the time of Registry enrollment. Univariate analysis was conducted by comparing demographics, past medical history, symptoms and signs at time of FMD diagnosis, arterial bed involvement, and family history between the two groups using Student's
A 61-year-old female with pernicious anemia presented with a 20-year history of walking-induced, erythematous, macular rash on her lower extremities. The rash was usually provoked by prolonged walking, especially in hot weather, although walking for as little as 30 minutes could trigger it as well. The eruption usually started at her shin (Panel A) and could extend all the way to her lower abdomen. She was treated with cortisone cream, vitamin E, and holistic approaches without significant improvement. Previous extensive workup for autoimmune diseases was non-revealing.Golfer's vasculitis, also known as exercise-induced vasculitis (EIV), is a specific form of benign cutaneous vasculitis affecting healthy individuals. 1,2 It is underappreciated, and mostly misdiagnosed as erythematous rash, or a purpuric itchy eruption. Unlike other idiopathic cutaneous vasculitides, golfer's vasculitis constitutes a stereotyped clinical entity, occurring after prolonged exercise such as a long walk or golf session. Other activities, such as dancing, cycling, or intensive cleaning are also reported as rare triggers. 1 A hot climate is considered a prerequisite; however, milder eruptions may occur in some patients in cooler seasons. Sun exposure does not appear as a contributory factor. Skin manifestations of golfer's vasculitis can vary among patients, including isolated erythematous rash, isolated purpura, erythematous rash and purpura, pseudo-urticaria, and/or lower extremity edema.The etiology of golfer's vasculitis is unknown. It is likely multifactorial and related to excessive heat production during prolonged exercise, leading to muscular hyperthermia and tissue damage, exercise-related immune system alteration, venous stasis, and alterations in skin blood flow. [1][2][3] Histological features on skin biopsy range from urticarial vasculitis with eosinophils to typical leukocytoclastic vasculitis. 1,2 Complement components C3 and C1q, immunoglobulin IgM, fibrinoid necrosis deposition, and erythrocyte extravasation can be present as well. 1,3 In most cases, cutaneous lesions resolve within a few days, but relapses are very frequent.Treatment of golfer's vasculitis is not standardized or mandatory given its self-resolving nature. Topical corticosteroids are reported to be beneficial at the acute phase, although no clinical trials have evaluated their efficacy. Properly fitting compression stockings decrease recurrence in about one-half of cases but can sometimes increase the symptoms. 1 Antihistamines have been used in some cases but have not been shown to be effective. 1
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