Sarcoidal Granuloma Annulare-Like Dermatitis and Vasculitis With Granulomatous Features: An Atypical Case of Giant Cell Arteritis.

ABSTRACT: Giant cell arteritis (GCA) is a diagnosis that clinicians should not miss because of the accompanying risk of irreversible vision loss. GCA can present without the classic symptoms of headache and temporal artery tenderness, which may lead to a delay in diagnosis. Cutaneous findings, although rare, have been associated with GCA. Accordingly, it is imperative to be aware of the broad clinical and histological presentations of GCA, including the cutaneous findings, because they may prove to be harbingers of impending disease. We present a unique case of GCA where 2 distinct cutaneous morphologies, sarcoidal granuloma annulare-like dermatitis and leukocytoclastic vasculitis with granulomatous features, presented simultaneously before the classic symptoms of headache and unilateral vision loss.

A Rare Case of Infliximab-Induced Small Vessel Vasculitis With Renal Involvement.

There are few cases in the literature demonstrating vasculitis induced by tumor necrosis factor-α. There exist even fewer cases of systemic inflammation involving the skin, nerves, and kidneys. Here, we present a novel case of a 27-year-old man with Crohn disease refractory to multiple medications, most recently treated with infliximab. He presented with a 3-week history of non-blanching palpable petechial rash involving his bilateral extremities and right upper extremity as well as lesions with black eschar around his ankles. He was found to have refractory cutaneous small vessel vasculitis, nephrotic range proteinuria, and small fiber neuropathy. This case describes the evaluation and treatment of systemic small vessel vasculitis in the setting of infliximab therapy.

A case of hypocomplementemic urticarial vasculitis syndrome complicated by eosinophilic pneumonia: a case report and review of the literature.

The primary symptom of urticarial vasculitis (UV), which is a histopathological leukocytoclastic vasculitis disease, is an eruption that resembles urticaria. Other organs may also experience accompanying symptoms. Lung lesions with UV are mostly obstructive pulmonary disease with smoking. However, the coexistence of eosinophilic pneumonia (EP) and complicated UV remains unclear. We report a man in his 70s with chronic obstructive pulmonary disease who attended our department with ring-shaped erythema, marginal edema, and pigmentation. Additionally, a skin histological analysis showed nuclear dust and perivascular neutrophil infiltration, while a blood sample showed a decrease in C3 and C1q concentrations. Administration of prednisone temporarily improved the eruption. However, he developed a cough and a new UV eruption 1 year later. Computed tomography revealed infiltration in the right upper lobe of the lungs, and a blood sample showed a high eosinophil count. He was finally diagnosed with hypocomplementemic urticarial vasculitis syndrome and idiopathic chronic EP. A previous study showed that serum C1q concentrations in patients with EP were lower when this disease was active. Whether a decline in C1q concentrations can cause EP is unclear. However, our case is unique owing to the co-onset of EP with low complement concentrations and recurrence of UV.

Rat bite fever mimicking ANCA-associated vasculitis.

Rat bite fever (RBF) is a rare infectious zoonotic disease caused by two bacterial species: the Gram-negative rod Streptobacillus moniliformis and the Gram-negative coiled rod Spirillum minus. The association between RBF and skin vasculitis and arthritis has been observed. The aim of this paper was to present a case of rat-bite fever with symptoms of skin vasculitis and arthritis, associated with high titers of ANCA antibodies and anti-endothelial cell antibodies suggestive of primary vasculitis. The patient was successfully treated with antibiotics and non-steroidal anti-inflammatory drugs, leading to significant improvement. Based on the presented case, we discuss the differential diagnosis of the signs and the role of infection in the induction of ANCA antibodies. We reviewed the English language literature for cases of RBF presenting with symptoms of vasculitis and/or antibody presence. A literature review was performed in PubMed and Google using the keywords "rat bite fever" AND "vasculitis", "systemic vasculitis", "ANCA", "antiendothelial antibodies". No cases of rat-bite fever with the presence of ANCA antibodies or AECA antibodies in its course have been described thus far. Rat bite fever is a rare disease with nonspecific symptoms. In its course, general weakness, intermittent fever, leukocytoclastic vasculitis, and arthritis are reported. To our knowledge, this is the first reported case of ANCA positivity associated with RBF.

Disseminated tuberculosis presenting as cutaneous leucocytoclastic vasculitis.

Cutaneous leucocytoclastic vasculitis (CLV) is a type of small vessel vasculitis, predominantly presenting with palpable purpuras and sometimes with systemic manifestations. The following report describes the case of a woman, who presented with fever, anorexia and maculopapular lesions over both lower limbs. Skin biopsy revealed CLV. CT scan demonstrated bilateral pulmonary nodules, ileocecal wall thickening and generalised lymphadenopathy. Colonoscopy guided biopsy obtained from ileocecal valve ulcer showed epitheloid cell granuloma with Langhans-type giant cells and caseous necrosis. Rapid clinical improvement was seen with anti-tubercular therapy. Among infectious causes, although rare and an uncommon presentation, Mycobacterium tuberculosis should be considered as an important cause of CLV.

Successful treatment of urticarial vasculitis with omalizumab in children: a case series.

Urticarial vasculitis (UV) is a small vessel leucocytoclastic vasculitis, which often needs to be distinguished from urticaria and other dermatoses. Treatment of UV in children is challenging because of the unsatisfying efficacy of antihistamines and the safety concern of long-term systemic corticosteroids or immunosuppressive agents. As a classic biological agent widely used in chronic spontaneous urticaria, omalizumab might also be a potential therapeutic option in the treatment of children with UV. This report presented four children, aged 4-6 years, with glucocorticoid-unresponsive UV successfully treated by omalizumab, thus providing evidence that omalizumab can be used to treat UV with good efficacy and tolerability in the paediatric population.

Localised chronic fibrosing vasculitis versus erythema elevatum diutinum.

A woman in her 70s was referred for a painless plaque on the shin, present for 2 years and progressing in thickness. Examination revealed a large erythematous to violaceous indurated plaque with cobblestone appearance. Biopsy revealed an inflammatory infiltrate of neutrophils with scattered histiocytes, lymphocytes, eosinophils and plasma cells interspersed with areas of lamellar fibrosis and focal areas of vascular damage, suggestive of a localised chronic fibrosing vasculitis of the skin. Localised chronic fibrosing vasculitis is a rare dermatosis, typically presenting as ulcerated violet-red nodules, which can appear histologically similar to erythema elevatum diutinum (EED), which typically presents as red-brown annular plaques. EED may have a predominance of neutrophils and granulomas, while chronic fibrosing vasculitis may have a sparse infiltrate of mixed inflammatory cells without granulomas. While dapsone is a first-line treatment for EED, there are no formal guidelines on the treatment of localised chronic fibrosing vasculitis. Given the neutrophils in this sample and similarities with EED, this patient was treated with oral dapsone, resulting in plaque improvement.

Normocomplementemic urticarial vasculitis secondary to Lyme disease: A rare association with challenging treatment.

Urticarial vasculitis (UV) is a rare entity characterised by long-lasting recurrent episodes of urticarial lesions. Although frequently idiopathic, UV has been associated with multiple diseases, including infections. We present a case of Lyme disease (LD) as a trigger of normocomplementemic UV, a very rarely described association. The patient presented first with episodes of inflammatory polyarthritis and a positive serology for Borrelia burgdorferi, later followed by the appearance of long-lasting urticarial lesions, histologically suggestive of UV. Lyme arthritis resolved with doxycycline, but UV persisted. Response to cyclosporine was satisfactory but with side effects, and only methotrexate showed substantial and consistent improvement. This case reminds physicians that chronic urticaria with atypical characteristics should raise suspicion of UV. Possible triggers for this disease must be sought, even if rarely described, such as LD. Normocomplementemic UV frequently presents a therapeutic challenge, but methotrexate can be a particularly effective therapy in this setting.

Erythema elevatum diutinum in a patient with rheumatoid arthritis.

Erythema elevatum diutinum (EED) is a rare cutaneous neutrophilic vasculitis with many associated diseases reported in the literature. We report a 65-year-old woman with painful and itchy lesions on her elbows, hands, knees, and foot for a year. Histopathologic examination confirmed the diagnosis of erythema elevatum diutinum and treatment with dapsone produced significant clinical improvement within few weeks. Erythema elevatum diutinum is a rare disease that should be considered in patients with violaceous nodular plaques located over the extensor regions of the limbs. Knowledge of this unusual pathology and its association helps to avoid misdiagnosis and late treatment.

IgA Vasculitis in the Setting of Biologic Therapy for Psoriasis and Recurrent Cutaneous Methicillin-Resistant Staphylococcus aureus Colonization.

IgA vasculitis is a form of cutaneous small-vessel leukocytoclastic vasculitis (LCV) that has various triggers, including anti-tumor necrosis factor (TNF) α therapy. As the use of more targeted biologic therapies such as the IL-17 inhibitor secukinumab increases, so do reports of associated adverse events. Herein, we describe an uncommon case of IgA vasculitis in a man undergoing biologic therapy with adalimumab and secukinumab for psoriasis with recurrent cutaneous methicillin-resistant Staphylococcus aureus (MRSA) colonization. A review of the current literature also is provided.

Correct approach in urticarial vasculitis made early diagnosis of lupus nephritis possible: a case report.

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. CASE PRESENTATION: 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. CONCLUSION: 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.

Granulocyte colony-stimulating factor as a cause of acute leucocytoclastic vasculitis with anti-Ro and anti-La antibodies.

Granulocyte colony-stimulating factor (G-CSF) administration is associated with a diverse range of cutaneous sequelae. Serious dermatological side effects of G-CSF include the development of Sweet's syndrome and exacerbations of pre-existing inflammatory disorders such as psoriasis. Here, we describe a report of acute leucocytoclastic vasculitis caused by G-CSF therapy associated with anti-Ro and anti-La antibodies in a patient with multiple myeloma. This case highlights the importance of having a high index of suspicion for acute leucocytoclastic vasculitis in patients with haematological malignancies undergoing G-CSF therapy.