Erythema Elevatum Diutinum Research Articles

Erythema Elevatum Diutinum in Association with IgA Monoclonal Gammopathy of Undetermined Significance

Erythema Elevatum Diutinum (EED) is a distinctive form of chronic leukocytoclastic vasculitis characterized by red to brown papules, plaques, and nodules that favor the extensor aspect of the extremities. EED is a benign condition but may be associated with several systemic diseases, including hematologic disorders, infections, and autoimmune conditions. EED is a rare entity of vasculitis, with less than 400 cases being reported in world literature to date. We present a rare case of a 61-year-old male with IgA monoclonal gammopathy of undetermined significance (MGUS) and a 17-year history of EED that improved significantly with dapsone treatment.

Erythema elevatum diutinum as the first clinical manifestation of HIV infection: case report

Erythema elevatum diutinum as the first clinical manifestation of HIV infection: case report

Lesional Infiltration of Eosinophils, Basophils, and M2 Macrophages Expressing Eotaxin-1 and Eotaxin-3 in Granuloma Faciale, but not in Erythema Elevatum Diutinum.

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Assessment of specificity of dermatopathologic criteria for IgG4-related skin disease.

IgG4-related disease (IgG4-RD) represents a recently characterized multisystemic fibroinflammatory condition that can manifest a spectrum of skin findings (IgG4-related skin disease; IgG4-RSD). Histopathologic and immunohistochemical criteria have been proposed; however, the specificity of these criteria merits scrutiny given the potential histopathologic overlap of IgG4-RSD and both neoplastic and inflammatory skin conditions featuring lymphoplasmacytic infiltrates (IgG4-RSD mimics). This study sought to assess the specificity of the criteria by quantifying the frequency by which an expanded spectrum of IgG4-RSD mimics meet proposed thresholds. Following IRB approval, a total of 69 cases of IgG4-RD mimics, representing 14 different diagnoses featuring plasma cells, were reviewed and analyzed for the following histopathologic and immunohistochemical features: (i) maximum IgG4+ count/high-powered field (hpf) >200; (ii) IgG4/IgG ratio >0.4 averaged over 3 hpfs; (iii) IgG4+ count >10 per hpf. Screening for IgG4-RSD by histopathologic criteria demonstrated the high frequency of lymphoplasmacytic infiltrates, contrasted with the rarity of storiform fibrosis (only one case of erythema elevatum diutinum [EED]) and obliterative phlebitis (0 cases). By immunohistochemical criteria, the analysis revealed that no cases exceeded 200 IgG4+ cells; 13% (9/69) cases demonstrated an IgG4/IgG ratio of >0.4 averaged over 3 hpfs; and 23% (16/69) cases demonstrated a mean IgG4+ count of >10 per hpf. Application of proposed IgG4-RSD histopathologic criteria to an expanded spectrum of potential IgG4-RSD mimics (to include cutaneous marginal zone lymphoma, syphilis, necrobiosis lipoidica, lichen sclerosus, ALHE, psoriasis, lymphoplasmacytic plaque, EED, and erosive pustular dermatosis), highlights the relative nonspecificity of lymphoplasmacytic infiltrates contrasted with the stringency of storiform fibrosis and obliterative fibrosis. Furthermore, an IgG4+ cell count of >10 per hpf and an IgG4/IgG ratio of >0.4 are not specific to IgG4-RSD alone. In the appropriate clinical context for IgG4-RSD, histopathologic features still represent the entry threshold for diagnosis consideration, which then allows for further screening by immunohistochemical criteria.

A Rare Case of Erythema Elevatum Diutinum with Blister

We report a rare case of erythema elevatum diutinum with blisters, a 58-year-old woman presented with recurrent erythema, nodules, plaques, and blisters on her face, trunk, and extremities without no cause. The biopsy of the blisters on the left lower leg showed Leukoblastic vasculitis and the biopsy of the plaque on the dorsal right hand showed persistent elevated erythema tissue. The vesiculobullous appearance of EED is extremely rare, and the histopathological in both the acute and chronic stage found on skin biopsy are more worth reporting.

Erythema elevatum diutinum: a rare case of atypical presentation involving palms

Erythema elevatum diutinum (EED) is a rare chronic dermatosis which mostly affects adult individuals. The classical skin lesions are asymptomatic, plum colored, erythematous nodules and plaques of variable sizes distributed symmetrically over extensor aspect of extremities. We reported a case of EED in a 50-year-old female presenting with lesions involving palms owing to its rare presentation.

Nodular Syphilis Seen in an HIV-Positive Man: A Case Study.

Syphilis is becoming an increasingly concerning sexually transmitted infection in the primary care setting with a precipitous rise in cases in the USA over the past decade according to the CDC surveillance data. This rise in cases is particularly relevant in military medicine as the greatest increase in cases is among adults in their 20s. Nodular syphilis is a rare presentation of the secondary stage of syphilis, and contrary to the well-known maculopapular rash seen with syphilis infection, little is known about effective treatment of nodular manifestations of the disease. We present a case of a 54-year-old HIV-positive man with nodular secondary syphilis effectively treated with an empiric 2-week course of oral doxycycline. Initial differential diagnosis included erythema nodosum, arthropod vector disease, skin infection, and erythema elevatum diutinum. Biopsy demonstrated granulomatous dermatitis, and serology revealed positive Treponema pallidum antibodies with a rapid plasma reagin of 1:256, confirming the diagnosis of secondary syphilis with granulomatous dermatitis. This case indicates that clinicians should consider this unusual cutaneous presentation of secondary syphilis when evaluating patients with nodular skin lesions, especially in high-risk individuals.

Erythema Elevatum Diutinum: A Case Report of Presentation and Management

Erythema elevatum diutinum (EED) is a rare, chronic necrotizing vasculitis and treatable skin condition, it has many histological mimics and is often association with a variety of underlying diseases. When these are present the management and prognosis dictate the course of EED. This case report highlights the rare nature of the disease, the difficulties encountered in clinching the diagnosis and the treatment and outcomes.

Paravertebral fibrous pseudotumor: Four cases of a distinctive tumefactive lesion overlapping with eosinophilic angiocentric fibrosis and tumoral erythema elevatum diutinum

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.

Erythema Elevatum Diutinum Associated with Tuberculosis

Erythema elevatum diutinum (EED) is a rare small vessel vasculitis with cutaneous manifestations most commonly presenting as red or brown papules, plaques, and nodules on extensor surfaces. It is a chronic, relapsing disease that is often associated with states of immunocompromise, e.g., HIV, gammaglobulinemias, and, more rarely, tuberculosis. Currently oral dapsone is the current treatment of choice, and while most patients respond well, it is imperative that the diagnosis be made early. We report a rare case of EED associated with underlying tuberculosis infection. Recognizing the distinct manifestations of EED and understanding its immunologic and infectious disease associations may enable clinicians to properly recognize, manage, and treat patients with EED.

Neutrophilic dermatoses

Neutrophilic dermatoses (ND) are a group of inflammatory skin conditions characterized by a neutrophilic infiltrate on histopathology with no evidence of infection. ND are classified based upon the localization of neutrophils within the skin and clinical features. Recent findings suggest that ND are due to two main mechanisms: i) a polyclonal hereditary activation of the innate immune system (polygenic or monogenic); or ii) a clonal somatic activation of myeloid cells such as encountered in myelodysplastic syndrome or VEXAS syndrome. ND belong to internal medicine as a great number of patients with ND suffer from an underlying condition (such as hematological malignancy, inflammatory bowel disease, auto-immune and auto-inflammatory diseases). ND are diagnoses of exclusion and physicians should always consider differential diagnoses, particularly skin infections. Here, we review the pathophysiology and classification of the main ND (i.e., subcorneal pustular dermatosis (Sneddon–Wilkinson Disease) and Intercellular IgA dermatoses, aseptic pustulosis of the folds, Sweet syndrome, neutrophilic eccrine hidradenitis, pyoderma gangrenosum, erythema elevatum diutinum, neutrophilic urticarial dermatosis and neutrophilic panniculitis), their clinical and histopathological features, and we highlight the investigations that are useful to identify ND-associated diseases and to exclude the differential diagnoses.

Reactive granulomatous dermatitis: A useful and encompassing term

Reactive granulomatous dermatitis: A useful and encompassing term

Erythema elevatum diutinum - Case report of a rare entity

Erythema elevatum diutinum (EED) is a rare entity of chronic cutaneous vasculitis occurring in association with a variety of conditions including autoimmunity, infectious disease, and hematological abnormalities. The exact pathogenesis of EED is unknown. A 59-year old female presented with multiple, itchy, eczematized erythematous papules and plaques over both upper and lower limbs present since 5 months. Histopathological changes were consistent with EED. Patient was started on oral Dapsone 100mg bid along with topical betamethasone diproprionate cream. Within 6 weeks lesions regressed completely leaving post-inflammatory hyperpigmentation. Dapsone was stopped at the end of 3 months. Minor recurrences are managed by topical steroids. EED is a rare form of vasculitis which is difficult to diagnose clinically, histopathology can be diagnostic. Dapsone is the treatment of choice.

Erythema elevatum diutinum of the hand: a peculiar case, successfully treated with tetracyclines.

article: Erythema elevatum diutinum of the hand: a peculiar case, successfully treated with tetracyclines - Italian Journal of Dermatology and Venereology 2021 December;156(6):722-4 - Minerva Medica - Journals

A Case of Erythema Elevatum Diutinum (EED) Exhibiting A Keloid-Like Appearance.

IntroductionMost 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 PresentationIn 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.DiscussionWhereas 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.ConclusionEED 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.

Concurrent Dermatomyositis, Celiac Disease, and Dermatitis Herpetiformis in a Patient with a History of Morphea

Dermatomyositis is a type of idiopathic inflammatory myopathy (IIM), characterized by an autoimmune response primarily against skin and muscle tissues.1,2 A variety of cutaneous manifestations can be seen in dermatomyositis, although Gottron’s papules, Gottron’s sign, and a heliotrope rash are considered pathognomonic.1,3 Gottron’s papules are flat-topped, red-to-purple papules and plaques involving the skin, metacarpophalangeal, and interphalangeal joints of the dorsal aspect of both hands. These lesions resolve, leaving areas of dyspigmentation, atrophy, and scarring. Gottron’s papules should be differentiated from similar lesions, such as verruca vulgaris, lichen planus, knuckle pads, sarcoidosis, and erythema elevatum diutinum.1,3 If a clinician initially suspects a Gottron’s mimicker and treats with the standard treatment for that diagnosis (i.e., verruca without resolution), a biopsy should be considered. Morphea, dermatomyositis, celiac disease, and dermatitis herpetiformis are autoimmune disorders that share similar pathogenesis and triggers. The diagnosis of multiple autoimmune syndrome (MAS) is given to patients with at least three autoimmune disorders with involvement of one or more autoimmune endocrine disorders.4 We present a case describing a patient with morphea, dermatomyositis, and celiac disease/dermatitis herpetiformis, suggesting a constellation of autoimmune disorders not subclassified by the MAS classification and not reported in the literature.

Extensive erythema elevatum diutinum associated with IgA monoclonal gammopathy: a rare case report

<p class="abstract">Erythema elevatum diutinum (EED) is a rare form of leukocytoclastic vasculitis with an unclear pathogenesis. Almost 250 cases of EED have been reported in the literature, associated with several diseases and presented with various clinical features. Term 'diutinum' means chronic, describes one of the main characteristic features. The duration is very long, varying between one to more than 39 years. We report a rare presentation of EED in a 58-years-old man who presented with extensive and symmetrical, persistent, erythematous to violaceous plaques over trunk and limbs and popular-nodular lesions present over pinna and dorsum of hands with burning sensation and itching over few lesions, in association with IgA monoclonal gammopathy.</p>

First Case Report of Erythema Elevatum Diutinum Involving the Glans Penis of A HIV–Positive Patient: Possible Atypical Manifestation of Immune Reconstitution Inflammatory Syndrome

First Case Report of Erythema Elevatum Diutinum Involving the Glans Penis of A HIV–Positive Patient: Possible Atypical Manifestation of Immune Reconstitution Inflammatory Syndrome

Erythema elevatum diutinum of the hand: a peculiar case, successfully treated with tetracyclines

Erythema elevatum diutinum of the hand: a peculiar case, successfully treated with tetracyclines

Update and Review of the Treatment of Erythema Elevatum Diutinum

Erythema elevatum diutinum (EED) is a rare chronic leukocytoclastic vasculitis dermatosis of unknown aetiology that was described by Hutchinson in 1888 and in 1894 was named by Radcliffe- Crocker and Williams .It presents as brown to red-purplish nodules, papules and plaques on the extensor surface of the extremities [1].