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Erythema elevatum diutinum is a rare, chronic cutaneous leukocytoclastic vasculitis, with prominent fibrosis at its later stage. In this article, we report a case of erythema elevatum diutinum in a 23-year-old woman with well-controlled Crohn disease. To our knowledge, erythema elevatum diutinum has been reported in only three other cases of Crohn disease, in which eruptions of erythema elevatum diutinum were associated with features of active Crohn. Our patient was in clinical remission at the time of erythema elevatum diutinum onset, making this report significant not only for its uncommon presentation, but more importantly, to aid readers. diagnosis and clinical management of similar cases.

Erythema elevatum diutinum (EED) is a rare form of chronic cutaneous leukocytoclastic vasculitis that is characterized by the appearance of red or purplish papules, plaques, or nodules on the skin. These lesions are typically found on the extensor surfaces of the limbs, such as the elbows, knees, and ankles. EED is frequently associated with other systemic diseases, such as autoimmune diseases, infections, and malignant diseases, particularly hematologic abnormalities. We report a case of EED in a 41-year-old Caucasian male with a previously unknown diagnosis of HIV infection. The treatment was performed with oral dapsone, resulting in complete regression of the lesions after 1 year of treatment. [ABSTRACT FROM AUTHOR]

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Introduction: Erythema elevatum diutinum (EED) is a rare form of cutaneous fibrosing type of leukocytoclastic vasculitis leading sometimes to nodule formation, imitating neoplastic process. Case report: We present the case of a 65-year-old woman with a 6-year history of cutaneous presentation of erythema elevatum diutinum, coexisting with myasthenia gravis and monoclonal IgA gammopathy. Dermoscopy of the skin lesions showed prominent thick arborizing vessels in a concentric alignment. Previous treatment with the use of methotrexate, dapsone, mycophenolate mofetil and cyclosporine did not stop the disease progression. Laboratory tests showed the presence of monoclonal protein IgAk in the serum and X-ray examination showed numerous fine-spot thinning of bone structure, especially within the skull bones. Hematological treatment with thalidomide and cyclophosphamide was introduced. A rapid resolution of skin lesions was observed. Conclusions: This case shows that management of the underlying hematological abnormality may be essential for successful therapy of the otherwise treatment resistant erythema elevatum diutinum. [ABSTRACT FROM AUTHOR]

Introduction: Small artery disease caused by neutrophils and immune-mediated is known as leucocytoclastic vasculitis (LCV). Clinically, it manifests as palpable, asymptomatic purpuric papules on the limbs. Ocular manifestation is rare. Here, we describe a case of peripheral ulcerative keratitis (PUK) associated with LCV. Case presentation: A 59-year-old man was referred to the hospital with blurred vision due to corneal perforation in his left eye. He complained of itchy nodules on his hands and lower legs for 15 years and the skin biopsy of the back of his hand revealed LCV 6 years ago, which suggested erythema elevatum diutinum. The patient was under treatment with anti-inflammatory and immunosuppressive drugs and physical features of LCV seen in him included erythema on his hands and legs. After receiving conjunctival flap covering surgery, the corneal perforation was resolved. Conjunctival flaps covered cornea that limited his vision to hand motion. Six months later, he was referred to our clinic again because of pain, redness, photophobia, and tearing in the right eye, presenting with PUK. Necrotic tissue was removed during surgery, which also included a conjunctival flap covering procedure. Following surgery, the symptoms were reduced, and the postoperative eye condition remained stable. Conclusion: To our knowledge, it is the first case of PUK secondary to LCV which was diagnosed 6 years ago. This case demonstrates that PUK associated with LCV can be successfully treated by surgical interventions. [ABSTRACT FROM AUTHOR]

Nodular lesions can be seen in a wide range of conditions such as infections, granulomatous conditions, neutrophilic disorders, vasculitis, benign tumours and malignant conditions. These numerous aetiologies behind the occurrence of nodular lesions signify the importance of thorough work-up of the patient to diagnose the underlying reason behind the clinical condition. Erythema elevatum diutinum (EED) is a rare vasculitis with variable clinical presentation, the diagnosis of which can be challenging. Extracutaneous signs such as arthralgias, oral and penile ulcers, involvement of the eye and neuropathy have all been linked to it. Various systemic illnesses, infectious diseases, autoimmune diseases, haematological abnormalities and plasma cell dyscrasias are also associated with EED. Such extracutaneous signs in EED patients indicate that the condition may involve several organ systems. Patients with EED should be assessed for systemic manifestations to ensure focused care, since extracutaneous forms of EED may comprise deposition of circulating immune complexes.

The skin manifestations of monoclonal(M)-proteinemia are rare and present in patients with monoclonal gammopathy of undetermined significance (MGUS), smoldering plasma cell myeloma (SMM) and multiple myeloma (MM). In this study, we reported 4 cases with M-proteinemia-related rare skin lesions, including pyoderma gangrenosum (PG), erythema elevatum diutinum (EED), cutis laxa (CL) and lichen myxedematosus(LM). These skin lesions are specific, where the potential mechanism was immune-mediated paraneoplastic syndrome rather than direct plasma cell infiltration. Anti-plasma cell treatment was effective in treating skin lesions. The clinical outcome of MM-related skin changes was correlated to tumor control, whereas the prognosis of MGUS or SMM related skin lesions was favorable. Skin involvement in M-proteinemia is extremely rare and less well-known, which greatly impairs quality of life. The diagnosis and treatment of these 4 cases support the need for futher study.

As EED and PG are both neutrophilic dermatoses of the same spectrum,[1] cases of EEDs complicated by PG may not be surprising. EED and PG both belong to the family of neutrophilic dermatoses; however, their concomitant occurrence has rarely been reported.[1] Herein, we present a case of EED associated with PG. Keywords: erythema elevatum diutinum; granulocyte colony-stimulating factor; IgA-type monoclonal gammopathy; neutrophilic dermatoses; pyoderma gangrenosum EN erythema elevatum diutinum granulocyte colony-stimulating factor IgA-type monoclonal gammopathy neutrophilic dermatoses pyoderma gangrenosum 288 291 4 05/12/23 20230501 NES 230501 INTRODUCTION Erythema elevatum diutinum (EED) is a rare cutaneous leukocytoclastic vasculitis that occurs almost symmetrically on the dorsum of the hands, elbows, knees, forearms, extensor surfaces of the lower legs, and the buttocks. [Extracted from the article]

A 24-year-old man with untreated human immunodeficiency virus (HIV) infection consulted our outreach clinic owing to the development of numerous asymptomatic nodules on his palms and fingers. Histopathologic evaluation revealed leukocytoclastic vasculitis and prominent fibrosis with a neutrophilic infiltrate consistent with erythema elevatum diutinum (EED). We referred the patient for initiation of antiretroviral therapy and started him on dapsone. The pathogenesis of EED is not completely understood, but it has been associated with numerous systemic conditions that may be infectious, inflammatory, or neoplastic. Only recently has EED been recognized as a defined reactive dermatosis of HIV. We present an exemplary case of HIV-associated EED and review the differential diagnosis, highlighting clinical features of EED that appear to be more frequently encountered in the HIV-infected population.

Nodular lesions can be seen in a wide range of conditions such as infections, granulomatous conditions, neutrophilic disorders, vasculitis, benign tumours and malignant conditions. These numerous aetiologies behind the occurrence of nodular lesions signify the importance of thorough work-up of the patient to diagnose the underlying reason behind the clinical condition. Erythema elevatum diutinum (EED) is a rare vasculitis with variable clinical presentation, the diagnosis of which can be challenging. Extracutaneous signs such as arthralgias, oral and penile ulcers, involvement of the eye and neuropathy have all been linked to it. Various systemic illnesses, infectious diseases, autoimmune diseases, haematological abnormalities and plasma cell dyscrasias are also associated with EED. Such extracutaneous signs in EED patients indicate that the condition may involve several organ systems. Patients with EED should be assessed for systemic manifestations to ensure focused care, since extracutaneous forms of EED may comprise deposition of circulating immune complexes. [ABSTRACT FROM AUTHOR]

Introduction: Granuloma annulare is a chronic, inflammatory skin disease occurring mainly in young people, predominantly in women. Skin lesions may be solitary or diffuse. They are usually located on the dorsal aspect of hands or feet and in the area of the ankles. The etiology of the disease remains unknown. A relationship between granuloma annulare and type 2 diabetes, hyperlipidemia and autoimmune diseases is postulated. Case report: We present the case of a 55-year-old woman with symetric skin lesions on extensor parts of 15 years duration. Clinically, the lesions corresponded to erythema elevatum diutinum. On the basis of histological examination, granuloma annulare was diagnosed. Conclusions: We describe a diagnostically challenging, clinically atypical granuloma anulare. In this case a histological examination was necessary to establish the diagnosis. [ABSTRACT FROM AUTHOR]

Erythema elevatum diutinum (EED) is a rare, chronic small-vessel vasculitis that presents as firm, red, violaceous, or brown papules and nodules on the extensor surfaces of the limbs. Oral dapsone is considered first-line therapy for EED; in the current case report, a patient presenting with EED began dapsone treatment and symptoms subsided within two weeks. Seven months later, the patient became pregnant and stopped dapsone owing to her concerns with dapsone use during pregnancy, resulting in recurrence of EED symptoms. We present a novel treatment approach with oral sulfasalazine, which was given to the patient in lieu of dapsone and resulted in complete resolution of EED symptoms.

BACKGROUND: Erythema elevatum diutinum is a leukocytoclastic vasculitis of chronic evolution, it has been associated with infectious processes, rheumatological diseases and neoplasms. CLINICAL CASE: A 57-year-old female patient with cystic ovarian teratoma that triggered erythema elevatum diutinum. CONCLUSIONS: It is extremely important to carry out an adequate anamnesis in all patients with this dermatosis in search of the etiological agent. [ABSTRACT FROM AUTHOR]

This article is a continuation of the literature review on skin vasculitis. In the first part, information was presented on the current nomenclature and prevalence of vasculitis, pathomorphological changes in the skin, as well as diagnostic algorithms for examining patients with this pathology are reflected. The second part of the article contains a detailed description of the elements of the skin rash with systemic and organ-specific vasculitis, presents the clinical manifestations observed with damage to other organs and systems, the development of possible complications. The data on modern treatment tactics are summarized, modern approaches using pathogenetic and symptomatic therapy of patients with various vasculitis are reflected.

Ghadah I ALHetheli,1 Ohoud Z Aljarbou,2 Ahmed ALHumidi,3 Mohammed I AlJasser4 1Division of Dermatology, College of Medicine, Qassim University, Buraidah, Saudi Arabia; 2Department of Pathology and Laboratory Medicine, King Abdulaziz Medical City, Riyadh, Saudi Arabia; 3Department of Pathology, College of Medicine, King Saud University, Riyadh, Saudi Arabia; 4Division of Dermatology, King Saud Bin Abdulaziz University for Health Sciences, Riyadh, Saudi ArabiaCorrespondence: Ghadah I ALHetheliDivision of Dermatology, College of Medicine, Qassim University, Buraidah 56218-6665, Saudi ArabiaEmail dr.gadah@gmail.comAbstract: Erythema elevated diutinum (EED) is a rare distinctive form of cutaneous leukocytoclastic vasculitis. EED typically presents with asymptomatic symmetrical erythematous-brown papules, nodules or plaques which favor the extensor aspect of extremities while distinctly sparing the palms. We report two cases of EED with a rare presentation limited to the palms.Keywords: palmar vasculitis, erythema elevatum diutinum, streptococcus

Introduction: Most severe-appearing keloids tend to occur around joints because of the increased extensional stimulation of the scar in those areas. However, erythema elevatum diutinum (EED) appears more commonly on friction sites including extensor surfaces of the extremities and dorsal surfaces of joints. EEDs also presents as red-brown and elevated lesions. Case Presentation: In this report, we describe a 42-year-old female who presented with firm, sporadic, brown-colored raised nodules on her bilateral lower extremities. As the appearance of these nodules resembled keloids, resection of the affected area with subsequent radiation therapy was initiated. However, histopathologic examination performed after treatment revealed tuberous lesions in the dermis, increased wired collagen fibers, neutrophilic infiltrate with nuclear dust, and edematous endothelial cells in the small vessels. Consequently, the patient was later diagnosed with EED. Post-surgery, no recurrence or abnormal scars appeared. Discussion: Whereas clinical findings of EED are similar to that of keloids, the mechanisms of the two conditions differ considerably, leading to varying management strategies. EEDs can be misdiagnosed as keloids on several grounds; they can both appear morphologically similar, exhibit as stiff lesions, demonstrate chronic inflammation of the reticular dermis, and appear anywhere on the body. The only definitive method of differentiating between the two is through histopathologic examination. Conclusion: EED should be considered as one of the differential diagnoses for any patients presenting with keloid-like lesions on friction sites and biopsy should be performed prior to resection and radiotherapy. [ABSTRACT FROM AUTHOR]

BACKGROUND: Erythema elevatum diutinum is a rare leukocytoclastic vasculitis. Its pathophysiology is not fully understood, but it is associated with immune complex deposition, complement activation, and inflammatory cytokines. The initial manifestations are macules, papules and purpuric plaques and finally fibrotic nodules with symmetrical distribution, mainly on the extensor and articular surfaces. CLINICAL CASES: We report two cases of erythema elevatum diutinum in association with two different entities. Case 1 : a 69-year-old man with papillary renal cell carcinoma of the left kidney, in whom a new association of erythema elevatum diutinum with sunitinib and everolimus was documented. Case 2: a 36-year-old man was diagnosed with erythema elevatum diutinum associated with stage 3 HIV. CONCLUSIONS: Due to the wide spectrum of cutaneous manifestations and associations, erythema elevatum diutinum is a diagnostic challenge that requires an extensive paraclinical study and must be confirmed with skin biopsy. Clinical suspicion and early diagnosis can achieve a more effective treatment. [ABSTRACT FROM AUTHOR]

Erythema elevatum diutinum (EED) is a rare chronic form of leukocytoclastic vasculitis that belongs to the group of neutrophilic dermatoses. The exact pathogenesis of the disease is still unclear. It has been associated with a number of systemic diseases including infections, autoimmune diseases, and both benign and malignant hematologic disorders. We report the case of a 50-year-old woman with complaints of symmetrical lesions on the extensor aspects of bilateral upper and lower limbs and pressure areas for 6 months. This case was difficult to manage because the patient was also hypersensitive to dapsone which is the drug of choice in EED.

We report the case of a 46‐year‐old Japanese woman with leukocytoclastic vasculitis (LCV) neuropathy who presented with skin symptoms (skin ulcers and livedo reticularis) and multiple mononeuropathies of the axonal degeneration type. Intravenous methylprednisolone pulse therapy and subsequent oral prednisolone and warfarin gradually ameliorated all her clinical symptoms. Although rare, neuropathy is an important clinical manifestation of LCV. [ABSTRACT FROM AUTHOR]

Erythema elevatum diutinum (EED) is a rare form of vasculitis characterized clinically by red-violet brown papules, plaques, and nodules mainly involving the extensor surfaces; histologically by leukocytoclastic vasculitis in early lesions, and fibrosis and cholesterolosis in late lesions. EED has been associated with many systemic disorders including infections, autoimmune disorders, and both benign and malignant hematological disorders. As it is a rare form of vasculitis and only 250 cases reported till date, we report a case of EED in association with IgA monoclonal gammopathy with partial response to dapsone treatment.

The neutrophilic dermatoses (ND) comprise a group of heterogeneous disorders characterised by inflammatory skin lesions that histologically show an intense inflammatory infiltrate composed primarily by neutrophils, with no evidence of infection. Although there are distinct clinical differences in the classical lesions of these disorders, many patients have overlapping features. In this mini-review we describe the clinical aspects of the main NDs, including: Sweet’s syndrome, neutrophilic dermatosis of the dorsal hands, pyoderma gangrenosum, erythema elevatum diutinum, subcorneal pustular dermatosis, neutrophilic eccrine hidradenitis, rheumatoid neutrophilic dermatitis, neutrophilic panniculitis, and aseptic abscesses, their association with underlying diseases, differential diagnosis, treatment options, and prognosis

Erythema elevatum diutinum is a rare neutrophilic dermatosis with vasculitis, which presents as persistent, symmetrical, purple or brownish papules and nodules, mainly in the extensor surface of the limbs. We describe a case of erythema elevatum diutinum and polyarthritis as initial manifestations of Crohn's disea se associated spondyloarthritis. A 51-year-old man, from São Tomé e Príncipe, with previous history of treated tuberculosis and chronic hepatitis B infection, was admitted due to 4 months history of polyarthritis, hyperpigmented papules on the extensor surfaces, occasional episodes of bloody mucous diarrhea and significant weight loss. Histology of the skin showed myeloperoxidase positive neutrophilic granulocytes with moderate karyorrhexis, consistent with erythema elevatum diutinum. Colonoscopy showed erosions in sigmoid and rectum. Diagnosis of erythema elevatum diutinum secondary to Crohn's disease with associated peripheral spondyloarthritis was assumed. The patient was treated with prednisolone, sulphasalazine, metronidazole, azathioprine and tenofovir with good clinical response. As erythema elevatum diutinum can be secondary to multiple systemic diseases, including rheumatic diseases and inflammatory bowel disease, being aware and re - cognizing this entity can be of great importance for rheumatologists. [ABSTRACT FROM AUTHOR]

Erythema elevatum diutinum (EED) has been emerging as a specific Human Immunodeficiency Virus (HIV) associated dermatosis in recent times. It is an extremely rare chronic disease of unknown origin and part of the spectrum of leukocytoclastic vasculitis. We describe a case of EED simulating Kaposi′s sarcoma in a 52-year-old HIV infected female patient with no previous opportunistic infections and CD4+ count of 164/mm 3 . Therapy with oral dapsone (100 mg/day) for two weeks resulted in resolution of some lesions.

Erythema elevatum diutinum (EED) is a rare skin disease that initially presents as leucocytoclastic vasculitis and later resolves with fibrosis. Dermatitis herpetiformis is an autoimmune blistering disease characterized by granular deposits of immunoglobulin A (IgA) in dermal papillae. We report a rare association of these two disorders.

Eosinophilic angiocentric fibrosis (EAF) is a rare tumefactive fibroinflammatory disease with predilection for the upper respiratory tract, characterized by concentric (onionskin) fibrosis around small arterioles with variable intervening storiform fibrosis admixed with chronic inflammatory infiltrates rich in eosinophils. Erythema elevatum diutinum (EED), another autoimmunological disorder that mainly affects acral sites and extensor surfaces, is characterized by neutrophilic leukocytoclastic vasculitis. Rarely, older EED lesions may present as tumefactive nodular (pseudotumoral) fibrous masses closely mimicking EAF. We herein describe four patients (all males) aged 66–70 years who presented with large (median, 7 cm) tumor-like fibrous lesions in the paravertebral region not associated with a known clinical autoimmune disease. All cases were resected surgically with the suspicion of a neoplasm. They displayed a strikingly similar histological appearance with combined features of EAF and nodular fibrous EED. None had evidence of obliterative phlebitis or increased IgG4: IgG ratio. The etiology of this distinctive lesion and its predilection for the paravertebral area of males remains obscure. A distinctive tumefactive localized reaction to trauma caused by degenerative disease of adjacent vertebrae might be a possible explanation. • Four cases (all older males) of a distinctive paravertebral fibroinflammatory pseudotumor overlapping with EAF and tumoral EED are presented. • Their uniform sites and histology argue for a shared etiology. • Lack of known autoimmune disorders suggests association with degenerative vertebral disease. • This variant of pseudotumor should be distinguished from fibroblastic neoplasms, IgG4-RD and other specific etiologies. [ABSTRACT FROM AUTHOR]