HistoryThis section has been translated automatically.
Hutchinson 1888; Bury 1889; Radcliffe Crocker and Williams 1894
DefinitionThis section has been translated automatically.
Rare, harmless, albeit eminently chronic (diutinum!), inflammatory disease of the skin with the characteristic histologic picture of leukocytoclastic "small vessel" vasculitis with angiocentric and storiform fibrosis (Sandhu JK et al. 2019).
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Occurrence/EpidemiologyThis section has been translated automatically.
Rare; 100-200 cases have been described in the literature worldwide
EtiopathogenesisThis section has been translated automatically.
Unexplained, probably infectious allergic vasculitis of small vessels; occasional detection of monoclonal immunoglobulins, most frequently IgA gammopathy (less frequently IgG gammopathy), multiple myeloma, possibly cryoglobulinemia.
Associations with infections (e.g. HIV, hepatitis B,hepatitis C, HIV), autoimmune diseases, especially rheumatoid arthritis, as well as Crohn's disease, ulcerative colitis, Wegener's granulomatosis, myelodysplasias have been described.
The presence of such extracutaneous manifestations in patients with erythema elevatum diutinum (EED) suggests that EED may be a multiorgan entity. Extracutaneous manifestations in EED may be associated with the deposition of circulating immune complexes; therefore, patients with EED should be evaluated for systemic manifestations to ensure targeted treatment.
ManifestationThis section has been translated automatically.
LocalizationThis section has been translated automatically.
Mainly over the extensor sides of the extremity joints (fingers, hands, knees), also buttocks, genitals, face and neck.
Clinical featuresThis section has been translated automatically.
Subacute development of symmetrically arranged, initially soft, later firm, roundish, polycyclic, nodular, blue-red or red-brown, also hemorrhagic, succulent large plaques. (Note: A purpuric component is not always clearly evident because the small-focal hemorrhages are episodic). Frequent central indentation, change in consistency, possibly stabbing pain and burning, itching. Early lesions tend to be bright red, whereas older lesions tend to be livid red to brownish red. Plate-like, keloid lesions are described. These are apparently completely refractory to therapy, so that surgical methods are also indicated (Awan BE et al. 2021/s.fig.).
Possible syntropia with gout, arthralgias, scleritis, panuveitis, peripheral ulcerative keratitis, oral and penile ulcers.
Lesions may heal after years (periods of 5-35 years are reported) leaving a hyperpigmented scar.
HistologyThis section has been translated automatically.
At an early stage the signs of leukocytoclastic vasculitis can be detected with perivascularly oriented neutrophil leukocytes and nuclear dust and fibrin in the vessel walls.
In typical "fully developed" lesions, a dense, diffuse infiltrate is found in the upper and middle dermis. Vascular orientation is usually not (no longer) detectable. Epidermis and skin appendages remain unaffected. Infiltrates consist of lymphocytes, neutrophilic leukocytes and nuclear dust, eosinophilic leukocytes, histiocytes and plasma cells. In addition, proliferation of fibrocytes and collagen fibres.
The following algorithm can be schematized:
|Accentuated around post-capillary venules|
|Capillaries left out of the less strongly involved|
|Damage to endothelial cells|
|Fibrin in/around vessel walls|
|Perivascular extravasation of erythrocytes|
|Edema in the papillary dermis|
|Variable number of eosinophils|
|Plasma cells and fibrosclerosis|
Differential diagnosisThis section has been translated automatically.
TherapyThis section has been translated automatically.
If necessary, treatment of the underlying disease.
The best results are described with DADPS. Patients respond to varying doses (50-150 mg/day). Improvement is immediate in some patients within days, in others skin lesions take months to heal. Treatment failures are known.
Glucocorticoids are usually less responsive. However, a therapy cycle of 4-6 weeks is recommended (initially 50mg prednisolone/day; gradual reduction over the indicated period).
The use of nicotinamide (e.g. Nicobion 1 tbl./day) is described as successful in individual cases.
Progression/forecastThis section has been translated automatically.
Chronic course. Spontaneous healing is possible after years, leaving a hpo- or hyperpigmented scar.
LiteratureThis section has been translated automatically.
Awan BE et al (2021) A Case of Erythema Elevatum Diutinum (EED) Exhibiting A Keloid-Like Appearance. Dermatol Ther (Heidelb) 11:2235-2240.
- Burnett PE et al (2003) Erythema elevatum diutinum. Dermatol Online J 9: 37
- Bury JS (1889) A case of erythema with remarkable nodular thickening and induration of the skin associated with intermittent albuminuria. Illus Med News 3: 145
Chen KR et al (2002) Clinical and histopathological spectrum of cutaneous vasculitis in rheumatoid arthritis. Br J Dermatol 147: 905-913.
- High WA et al (2003) Late-stage nodular erythema elevatum diutinum. J Am Acad Dermatol 49: 764-767.
- Hutchinson J (1888) On two remarkable cases of symmetrical purple congestion of the skin in patches, with induration. Br J Dermatol 1: 10-15
- Kim H (2003) Erythema elevatum diutinum in an HIV-positive patient.
J Drugs Dermatol 2:411-412.
- Lisi S et al (2003) A case of erythema elevatum diutinum associated with antiphospholipid antibodies. J Am Acad Dermatol 49: 963-964.
- Manni E et al (2014) Case of erythema elevatum diutinum associated with IgA paraproteinemia successfully controlled with thalidomide andplasma
exchange. Ther Apher Dial 19:195-196
- Momen SE egt al. (2014) Erythema elevatum diutinum: a review of presentation and treatment. J Eur Acad Dermatol Venereol 28:1594-1602.
- Radcliffe-Crocker HW, Williams C (1894) Erythema elevatum diutinum. Br J Dermatol 6: 1-9 & 53-5.
- Ratzinger G et al (2015) The vasculitis wheel-an algorithmic approach to cutaneous vasculitides. JDDG 1092-1118
Sandhu JK et al (2019) Erythema elevatum et diutinum as a systemic disease. Clin Dermatol 37:679-683.
Incoming links (17)Cholesterol, extracellular; Crocker, radcliffe henry; Cryoglobulins and skin; Dadps; Dermatitis-arthritis syndromes; Erythema; Erythema figuratum perstans; Erythema microgyratum persistens; Granulomatous interstitial Dermatitis with arthritis; Hutchinson, jonathan; ... Show all
Outgoing links (19)Antihistamines; Crohn disease, skin alterations; Cryoglobulins and skin; Dadps; Desloratadine; Erythema anulare centrifugum; Erythema multiforme; Facial granuloma; Glucocorticosteroids systemic; Gout; ... Show all
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