Birt-Hogg-Dubé syndrome, an extremely rare genetic disorder, is characterized by the development of fibrofolliculomas, lung cysts and subsequent recurrent pneumothorax, and kidney neoplasia. This report highlights the case of a 56-year-old female with a history of right vestibular schwannoma status post stereotactic radiotherapy and vulva bartholin\'s gland carcinoma who was initially evaluated by primary care for a 6-month history of intermittent, red, raised, widespread rash accompanied by fever, chills, and body aches. A punch biopsy of the rash was performed, which was notable for an urticarial tissue reaction with focal changes of leukocytoclasia and negative direct immunofluorescence. Laboratory tests, which included an autoimmune genetic and periodic fever panel, were unremarkable. Whole genome sequencing returned positive for a pathogenic variant in folliculin gene, consistent with a diagnosis of Birt-Hogg-Dubé syndrome.

Urticarial vasculitis (UV) is a type of small-vessel vasculitis, which is rarely associated with anti-tumor necrosis factor (TNF)-alpha medication. We describe a 72-year-old woman with multiple comorbidities on several medications, including an adalimumab biosimilar for Hurley stage II recalcitrant hidradenitis suppurativa (HS), who presented with new-onset severe angioedema and a rash with urticarial wheals that covered most of her body surface area. The diagnosis of drug-induced UV is supported by both the history of adalimumab biosimilar use and the histopathology result. The patient responded successfully to a course of doxycycline administered for three months, which was preceded by corticosteroid dosages, both orally and intravenously, to reduce inflammation. The given case highlights the correlation between a distinct dermatologic autoimmune manifestation and TNF-targeted therapy, demonstrating the importance for dermatologists to be aware of the potential side effects of adalimumab biosimilars in order to manage them effectively.

Dialysis associated reactions presenting with urticarial vasculitis is rarely reported in medical literature. We report a 61-year-old gentleman who developed sudden onset dyspnea with diffuse erythema within 20 min of haemodialysis. Patient was started on Azilsartan 3 days prior to this clinical event. Labs revealed features of hemolysis and urine was positive for hemoglobinuria. All dialysis related factors responsible for this reaction were ruled out. Due to non-resolution of skin rash, skin biopsy was attempted which revealed fibrinoid necrosis of occasional vessels with predominant lymphocytic infiltration suggestive of drug induced urticarial vasculitis. Complement levels were normal. He was managed with steroids, anti-histaminic, discontinuation of azilsartan and change of dialyzer membrane. This case highlights a rare dermatological presentation of Type A dialysis associated reaction involving azilsartan with differential diagnosis and treatment strategies.

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UNASSIGNED: Hypocomplementemic urticarial vasculitis syndrome (HUVS) is an infrequent immune complex-mediated condition characterized by nonpruritic urticarial lesions, low serum complement levels, and autoantibodies, associated with systemic manifestations like arthralgia/arthritis, angioedema, ocular inflammation with conjunctivitis, episcleritis, uveitis, renal, gastrointestinal, and pulmonary involvement. HUVS and systemic lupus erythematosus (SLE) overlap and the criteria for identifying HUVS as an entity distinct from SLE are lacking. Despite the diagnostic criteria established by Schwartz et al. [Curr Opin Rheumatol. 2014;26(5):502-9], differentiation from SLE is sometimes difficult as patients often also fulfill the classification criteria of the American College of Rheumatology (ACR). The prognosis of HUVS depends on the organ system involved. Lung disease results in significant morbidity and mortality and is made worse by smoking. Kidney involvement with glomerulonephritis may ultimately result in end-stage renal disease with the need for kidney transplant. Death may also occur due to acute laryngeal edema.
UNASSIGNED: We pre-sent a case of a 40-year-old female who had a diagnosis of SLE, presented with severe odynophagia, was found to have an erythematous macular rash, and had acute kidney injury attributed to contrast-related injury and cardiorenal syndrome. After the resolution of the AKI, she continued to have hematuria and low-grade proteinuria that led to a kidney biopsy that aided in the diagnosis of HUVS.
UNASSIGNED: Given the rarity of this disease and the difficulty in differentiating HUVS from other rheumatological diseases such as SLE, further accumulation of cases is necessary to understand the best diagnostic modality for this entity.

BACKGROUND: Urticarial vasculitis is a clinicopathologic entity defined by recurrent episodes of urticarial lesions that persist > 24 hours and demonstrate the histopathologic features of leukocytoclastic vasculitis. The most important prognostic feature is the presence of normo- or hypocomplementemia. In the latter, patients are much more likely to have systemic manifestations. Urticarial vasculitis is most often idiopathic, but it can arise in association with autoimmune connective diseases, cryoglobulinemia, infections, medications, and hematologic malignancies.
METHODS: We present the case of a 61-year-old Caucasian woman with a skin eruption that consisted of erythematous plaques on the trunk and limbs that lasted > 24 hours but were asymptomatic. The skin eruption had an acute onset and persisted for 3 months upon initial presentation in our dermatology department. A punch biopsy showed signs of a leukocytoclastic vasculitis in the superficial dermis. On laboratory examination, signs of activation of the complement system were found with low complement C3, C4, and C1q, and with a high anti-C1q antibody titer. The clinical, histological, and lab results fit the diagnosis of hypocomplementemic urticarial vasculitis. There was also a positive antinuclear factor with elevated U1 small nuclear ribonucleoprotein and high double-stranded DNA determined by Farr method. On urinalysis, marked proteinuria and massive hematuria were found. Kidney biopsy showed focal crescentic and focal mesangial type of glomerular damage with a full-blown positivity of immunoglobulin A, immunoglobulin G, and C1q, leading to lupus nephritis class III-A (according to the International Society of Nephrology/Renal Pathology Society 2003 classification of lupus nephritis). The patient was treated with hydroxychloroquine, corticosteroids, and low-dose intravenous cyclophosphamide (Euro-Lupus regimen) as remission-inducing agent, followed by azathioprine as remission-maintaining agent. This treatment regimen gave good results, with total clearance of the skin lesions and remission of the lupus nephritis.
CONCLUSIONS: Clinicopathologic recognition of urticarial vasculitis with correct screening for extracutaneous disease can lead to early diagnosis of serious organ involvement and thereby improve prognosis for the patient.

BACKGROUND: We report a male who presented with acute visual defect and was diagnosed with urticarial vasculitis with recurrent branch retinal artery occlusion (BRAO) after systemic disease survey, fluorescein angiography (FA), and MultiColor imaging (MCI).
METHODS: A 47-year-old male with a history of urticarial vasculitis presented with visual defect OD. Fundus examination showed two foci of ischemic retinal whitening beneath the inferior arcade and above the superior arcade. MCI demonstrated a greenish tinge in the corresponding area. FA revealed segmental arteriolar staining and arterial occlusive changes. BRAO with retinal arteritis was diagnosed. Toxoplasma IgG was positive. Sulfamethoxazole 400mg plus trimethoprim 80mg was given. His vision worsened after 1-week of treatment. The established lesions improved, but new lesions occurred. Interferon-gamma release assay was positive but tuberculosis DNA qualitative amplification test of sputum was negative. Sputum acid-fast stain was positive and culture revealed nontuberculous mycobacteria. Left facial itching and reactive lymphadenopathy developed. Prednisolone and cyclophosphamide were started. The initial retinal artery lesions regained perfusion.
CONCLUSIONS: Urticarial vasculitis with recurrent BRAO is an immune complex-mediated disease. Greenish-tinged occlusive lesions were noted from MCI with high resolution and contrast. MCI could be a valuable method for retinal vessel occlusive disease detection before FA and follow up.

During the COVID-19 pandemic, physicians must maintain a high index of suspicion for COVID-19 in cases of urticarial vasculitis or other forms of urticaria. This is particularly important for acute presentations in otherwise asymptomatic individuals and pregnant women, where a prompt approach to the patient can prevent undesirable complications.

Urticarial vasculitis is an entity characterized by urticarial rashes that are typically pruritic and painful, associated with angioedema and purpura. Influenza viruses are common respiratory pathogens that can cause seasonal infections and global human morbidity/mortality. A 4.5-year-old girl presented with fever and painful urticarial skin lesions leaving post-inflammatory hyperpigmentation. The nasal specimens showed a positive for influenza A/H1N1. On the basis of these findings, a diagnosis of urticarial vasculitis associated with A/H1N1 was concluded. Taking the results together, we suggest that urticarial vasculitic lesions can be considered to the list of cutaneous manifestations during the seasonal flu.

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