Association of hyperimmunoglobulinaemia D syndrome with erythema eievatum diutinum

Miyagawa, Sachiko; Kitamura, Wataru; Morita, Kenichi; Saishin, Mototsugu; Shirai, Toshihiko

doi:10.1111/j.1365-2133.1993.tb00238.x

Cited by 29 publications

(13 citation statements)

References 9 publications

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“…Henoch-Schö nlein purpura and erythema elevatum diutinum have been also reported in children with HIDS. 40,51 Erysipelaslike lesions are the most distinctive cutaneous lesion associated with FMF. 52 In published studies, 53,54 the observed frequency of these lesions varies from 3% to 46%, and they have been reported to occur in 15% to 20% of children.…”

Section: Commentmentioning

confidence: 99%

Tumor Necrosis Factor Receptor–Associated Periodic Syndrome

Toro

Aksentijevich²,

Hull³

et al. 2000

Arch Dermatol

130

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Background: Tumor necrosis factor receptorassociated periodic syndrome (TRAPS) is an inflammatory disorder characterized by prolonged episodes of periodic fever and localized inflammation and dominantly inherited mutations in TNFRSF1A, the gene encoding the 55-kDa tumor necrosis factor receptor. To our knowledge, the cutaneous pathologic characteristics of TRAPS have not been described previously.Objectives: To characterize the dermatologic manifestations of TRAPS by clinical, microscopic, and molecular methods, and to investigate its immunophenotype.Design, Setting, and Patients: At the National Institutes of Health Clinical Center, Bethesda, Md, a tertiary care referral center, 25 patients with a clinical and molecular diagnosis of TRAPS were evaluated clinically and 10 biopsy specimens of lesional skin were examined by light microscopy and immunohistochemistry. Patients were screened for mutations in TNFRSF1A, the gene coding for the p55 tumor necrosis factor receptor.Main Outcome Measures: Clinical, light microscopic, and immunohistochemical features. Results:The skin eruption usually lasted 4 to 21 days (mean, 13 days). Of 25 patients, 21 (84%) presented with migratory erythematous macules and patches and 10

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Section: Commentmentioning

confidence: 99%

Tumor Necrosis Factor Receptor–Associated Periodic Syndrome

Toro

Aksentijevich²,

Hull³

et al. 2000

Arch Dermatol

130

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“…Hernandez-Cano et al reported the disappearance of EED lesions in a patient with a liver transplantation following a reduction in cyclosporin dosage [12]. However, EED lesions may not respond to dapsone, or if they do, recurrences are quite common [5,11,24,31,39]. It is possible that the marked fibrosis in late lesions may account for the lack of response to dapsone [8].…”

Section: Discussionmentioning

confidence: 91%

“…Other conditions reported to be associated include hyperimmunoglobulinemia D syndrome, cryoglobulinemia, myelodysplastic syndromes, abnormalities in the clotting mechanism, haematological malignancies, prostatic carcinoma, testicular lymphoma, viral and bacterial infections, rheumatic fever, HIV infection, inflammatory bowel disease, rheumatoid arthritis and peripheral ulcerative keratitis, relapsing polychondritis, Wegener's granulomatosis, and acro-osteolysis [1,9,10,16,18,23,24,37,39,40,41,42].…”

Section: Discussionmentioning

confidence: 96%

Erythema elevatum diutinum and plastic surgery Case report

Lari¹,

George

Al-Saeed

2002

European Journal of Plastic Surgery

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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.

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“…Ig have been reported in some cases, no uniformly successful treatment has been determined 3 . Notably, in a case accompanied with EED, dapson had a better effect than corticosteroid 27 . In 1999, the mevalonate kinase ( MVK ) gene on chromosome 12q24 was identified to be responsible for this disease 28,29 .…”

Section: Hereditary Periodic Fever Syndromesmentioning

confidence: 99%

Autoinflammatory syndromes with a dermatological perspective

Kanazawa

Furukawa

2007

The Journal of Dermatology

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The term autoinflammatory syndromes describes a distinct group of systemic inflammatory diseases apparently different from infectious, autoimmune, allergic and immunodeficient ones. Originally, it was almost synonymous with clinically defined hereditary periodic fever syndromes, including familial Mediterranean fever, hyper immunoglobulin D syndrome with periodic fever and tumor necrosis factor receptor-associated periodic syndrome. Similar but distinct periodic fever syndromes accompanied by urticarial rash, familial cold autoinflammatory syndrome, Muckle-Wells syndrome and chronic infantile neurological cutaneous articular syndrome, have all been reportedly associated with CIAS1 mutations and are collectively called cryopyrin-associated periodic syndromes. Consequently, the concept of autoinflammatory syndromes has been spread to contain other systemic inflammatory diseases: rare hereditary diseases with or without periodic fevers, such as pyogenic sterile arthritis, pyoderma gangrenosum and acne syndrome, Blau syndrome and chronic recurrent multifocal osteomyelitis, and the more common collagen disease-like diseases, such as Behcet's disease, Crohn's disease, sarcoidosis and psoriatic arthritis. These diseases are all caused by or associated with mutations of genes regulating innate immunity and have common clinical features accompanied with activation of neutrophils and/or monocytes/macrophages. In this review, major autoinflammatory syndromes are summarized and the pathophysiology of related skin disorders is discussed in association with dysregulated innate immune signaling.

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Association of hyperimmunoglobulinaemia D syndrome with erythema eievatum diutinum

Abstract: Hyperimmunoglobulinaemia D and periodic fever syndrome was observed in a female patient with erythema elevatum diutinum. The association of this skin disease with hyperimmunoglobulinaemia D may indicate a pathogenetic relationship. The skin lesions responded to dapsone therapy.

Cited by 29 publications

References 9 publications

Tumor Necrosis Factor Receptor–Associated Periodic Syndrome

Tumor Necrosis Factor Receptor–Associated Periodic Syndrome

Erythema elevatum diutinum and plastic surgery Case report

Autoinflammatory syndromes with a dermatological perspective

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