Case Of Erythema Elevatum Diutinum Research Articles

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.

A case of erythema elevatum diutinum complicated by ocular damage

A case of erythema elevatum diutinum complicated by ocular damage

Clinical and surgical therapeutic approach in Erithema Elevatum Diutinum - case report

Erythema elevatum diutinum is a rare chronic cutaneous vasculitis which usually affects adults. It is characterized by symmetrical and persistent papules, plaques, and nodules. These lesions are usually located on the extensor surfaces of the extremities. We report a case of erythema elevatum diutinum in which the association of dapsone and surgical excision promoted complete remission of skin lesions, providing a new approach to the treatments described in the literature.

Granuloma Faciale

The pathogenesis of granuloma faciale (GF), framed in the group of cutaneous vasculopathic dermatitis, is poorly understood. The present study investigated whether GF might be part of the spectrum of IgG4-related sclerosing diseases (IgG4-RD). Erythema elevatum diutinum (EED), believed to belong to the same group of disorders as GF, was also studied for comparison. Thirty-one biopsies of GF obtained from 25 patients (18 men, 7 women) and 5 cases of EED (4 women and 1 man) were analyzed morphologically and for the expression of IgG and IgG4 by immunohistochemistry. The distribution of Th1, T regulatory and Th2 T-cell subsets, respectively, identified by anti-T-bet, anti-FoxP3, and anti-GATA-3 antibodies, was also evaluated. The dermal inflammatory infiltrate in GF contained eosinophils and plasma cells in variable proportions. Obliterative venulitis was found in 16 cases, and storiform fibrosis, a typical feature of IgG4-RD, was observed in 8 cases and was prominent in 3 of them. On immunohistochemical analysis 7 of 31 biopsies (22.6%) from 6 GF patients fulfilled the criteria for IgG4-RD (IgG4/IgG ratio >40%, and absolute number of IgG4 per high-power field >50). Interestingly, the 6 patients were male, and 4 showed recurrent and/or multiple lesions. In an additional 5 cases, only the IgG4/IgG ratio was abnormal. None of the 5 EED cases fulfilled the criteria for IgG4-RD. The T-cell subsets in GF were quite variable in number, GATA-3 lymphocytes were generally more abundant, but no relationship with the number of IgG4 plasma cells was found. The study indicates that a significant number of GF cases are associated with an abnormal content of IgG4 plasma cells; this association was particularly obvious in male patients and in cases presenting with multiple or recurrent lesions. As morphologic changes typically found in IgG4-RD, such as obliterative vascular inflammation and storiform sclerosis, are found in GF, we suggest that GF might represent a localized form of IgG4-RD.

Erythema Elevatum Diutinum in Human Immunodeficiency Virus Infected Patient

Erythema elevatum diutinum (EED) is emerging as a specific HIV-associated dermatosis which can be easily misdiagnosed as Kaposi's sarcoma or bacillary angiomatosis. Until now, no case of HIV-associated EED had been reported in Korea. We report a case of EED in a 49-year-old man with HIV infection. The patient was diagnosed with HIVinfection and treated with a combination of anti-retroviral agents and dapsone. Two years after the start of treatment the lesion had regressed.

A case of erythema elevatum diutinum associated with peripheral ulcerative keratitis

A young woman with recurrent painful lesions on the dorsal aspects of her hands associated with arthralgia presented with pain and redness of both eyes. After extensive investigations, a diagnosis of erythema elevatum diutinum accompanied by peripheral ulcerative keratitis was made. The patient was treated with dapsone 50 mg, t.i.d., and responded well.

Erythema elevatum diutinum: An “idiopathic” case

Erythema elevatum diutinum (EED) is a rare condition with an unclear pathogenesis. Initially classified within neutrophilic dermatoses, it is now considered as a leukocytoclastic vasculitis accordingly to its histopathologic pattern. Several clinical presentations as well as many associated diseases are reported in the literature. We report a new case of EED in a 58-year-old man who presented with a three-month history of plaques and nodules on the extensor surfaces of hands, elbows, knees, ankles, forearms, and buttocks. Histology showed a leucocytoclastic vasculitis, suggestive of the diagnosis of EED. Screening for an associated pathology, namely a paraproteinemia or a solid cancer, was negative. Treatment with dapsone leads to amelioration within few weeks.

Erythema elevatum diutinum associated with scleritis

Erythema elevatum diutinum (EED) is a chronic and rare variant of leukocytoclastic vasculitis that is being reported nowadays frequently in association with HIV infection. It clinically manifests as asymptomatic to tender erythematous papules, plaques and nodules, usually with acral distribution and is rarely accompanied by systemic complaints other than arthralgia. The reported associations include preceding upper respiratory infections, hematological malignancies, lymphomas and monoclonal gammopathies. Here we report a 45 year old man with multiple joint pains, tender nodules over palms and soles and ocular pain and congestion, who was subsequently diagnosed as a case of EED and scleritis following histopathological examination. Skin as well as ocular complaints completely subsided with Dapsone monotherapy.

Caso para diagnóstico

Erythema elevatum diutinum is a rare presentation of cutaneous vasculitis. Typical lesions are papules, plaques, and nodules that may coalesce and later turn to yellow-brown color. The extensor surface of the extremities is the most involved area and older lesions may present xanthomatous appearance. Light microscopy of acute lesions shows neutrophilic, perivascular infiltrate with dermal fibrin deposits, endothelial expansion, and leukocytoclasis. We present a case of erythema elevatum diutinum without associated disease.

Forme pseudotumorale d’erythema elevatum diutinum

Erythema elevatum diutinum is a rare, chronic and recurrent dermatosis affecting adults. The disease is characterized by symmetrical, red, brownish-purple, and yellow papules, plaques, and nodules distributed mainly over the extensor surfaces of the extremities. The aetiology is unknown. The condition can occur in association with haematological malignancies (30%), and most frequently with IgA monoclonal gammapathy. Histological diagnosis is sometimes difficult, especially in the late stages of the disease. We describe an unusual case of erythema elevatum diutinum with clinical and histopathological pseudoneoplastic features. A 60-year-old man with no significant medical history consulted for a large nodule of the left knee measuring 2.5cm and red-purple papules symmetrically distributed on the extensor surfaces. Surgical removal of the nodule was performed. The histologic findings were characterized by a predominant concentric fibrosis forming well-circumscribed dermal nodules, composed of small aggregates of spindle cells with palisading and lamellar patterns. Within the nodules, pycnotic polymorphonuclear leucocytes were observed. The nodules were surrounded by a lymphoplasmocytic and histiocytic infiltrate. The diagnosis of erythema elevatum diutinum was thus made and dapsone given, with partial improvement of the lesions. This case illustrates the nodular pseudoneoplastic presentation and the difficulty of histological diagnosis, since long-standing lesions can mimic connective tissue tumours. Clues for histopathological diagnosis are neutrophils scattered throughout the lesion, sometimes associated with vasculitis. Extensive investigation should be made for polymorphonuclear leucocytes that are always present, even in late fibrotic lesions.

A Case of Erythema Elevatum Diutinum associated with Autoimmune Neutropenia and Sjögren Syndrome

A Case of Erythema Elevatum Diutinum associated with Autoimmune Neutropenia and Sjögren Syndrome

A case of erythema elevatum diutinum associated with breast carcinoma

A 53‐year‐old woman diagnosed with invasive ductal‐type breast carcinoma was referred to our clinic with red–purple lesions on the hands and legs. She had neither pruritus nor pain. The first lesion developed on the dorsal hand. In the following days, new lesions appeared on the extensor surface of the legs. The patient had been treated with modified radical mastectomy and three courses of cyclophosphamide, adriamycin, and fluorouracil chemotherapy.Dermatologic examination revealed reddish‐violaceous papules and plaques ranging from a few millimeters to 2 cm in diameter, bilaterally located on the dorsal hands, especially over the metacarpophalangeal and interphalangeal joints (Fig. 1). Multiple red–purple, circumscribed papules and plaques of various diameters were observed bilaterally over the shins (Fig. 2). The largest of these plaques showed an annular configuration. The nails showed distal subungual keratosis and yellow discoloration. The rest of the physical examination was normal.Violaceous papules and plaques on the dorsal handsimageRed–purple, circumscribed, papules and plaques over the shinsimageA biopsy taken from the medial side of the shin revealed a predominantly neutrophilic infiltrate and nuclear dust around the dermal vessels and orthokeratotic stratum corneum (Fig. 3).Predominantly neutrophilic infiltrate and nuclear dust around the dermal vessels and orthokeratotic stratum corneum (hematoxylin and eosin stain, ×100)imageComplete blood count, routine biochemical tests and fasting lipids, serologic tests of bacterial and viral agents, serum electrophoresis, and serologic profiles for autoimmune connective tissue diseases revealed normal results. Mycologic examination of nail clippings did not show any evidence of fungal infection.In the light of our clinical and histopathologic findings, a diagnosis of erythema elevatum diutinum was made, and the patient was given topical clobetasol propionate therapy. Complete clearance was achieved in 3 weeks (Fig. 4). After six courses of cyclophosphamide, adriamycin, and fluorouracil chemotherapy, and radiotherapy, no recurrence of erythema elevatum diutinum lesions was observed.Healed lesion 3 weeks after high‐potency topical glucocorticoidsimage

A case of erythema elevatum diutinum associated with antiphospholipid antibodies

A case of erythema elevatum diutinum associated with antiphospholipid antibodies

Eritema elevatum diutinum

Abstract—Erythema elevatum diutinum is a rare and chronic skin disorder. Clinically is characterized by red, purple, brown or yelow papules, plaques or nodules. These lesions are usually symmetrically distributed on the extensor surfaces of the extremities. Histologically, early lesions of erythema elevatum diutinum show leukocytoclastic vasculitis. We report three cases of erythema elevatum diutinum. These patients are middle aged women, with multiple pruritic lesions of several months'duration.Previous treatment with topical corticosteroids had been unsuccessful. After laboratory studies (erythrocite sedimentation rate, C reactive protein, protein electrophoresis, serological test) and biopsy of lesions we confirmed a diagnosis of erythema elevatum diutinum. The first case had a history of rheumatic disease but the other patients were associated with hepatitis C infections and with elevated levels of Ig A. We established treatment with dapsona and we discussed its evolution. We reviewed the clinical characteristics, histologic, epidemiologic and associated features of the process.

Erythema elevatum diutinum and plastic surgery Case report

Erythema elevatum diutinum (EED) is a chronic form of leucocytoclastic vasculitis that presents with symmetrical plaques, papules, and nodules in an acral distribution. The treatment is conservative, with dapsone being the first choice among the drugs commonly used. However, the late lesions may not respond due to the marked fibrosis, and they are extremely stressful to the patient both symptomatically and cosmetically. A case of EED in a patient with history of splenectomy for idiopathic thrombocytopenic purpura (ITP) is presented, where the late cutaneous lesions were excised and skin grafted. The association with splenectomy and the use of skin grafting as a mode of treatment when all other modalities have failed, have not been reported previously. A review of the relevant literature is presented.

Erythema elevatum diutinum mimicking porphyria cutanea tarda

A case of erythema elevatum diutinum (EED) closely resembling porphyria cutanea tarda (PCT) is reported. The initial skin biopsies were suggestive for PCT but porphyrin levels in the urine, stool and plasma were normal. A further biopsy from an early cutaneous lesion showed a leucocytoclastic vasculitis with fibrinoid necrosis of the vessel walls.

A case of Erythema elevatum diutinum associated with cancer of thyroid gland

A case of Erythema elevatum diutinum associated with cancer of thyroid gland