Erythema elevatum diutinum L95.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 19.06.2022

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Synonym(s)

EED; Erythema elevatum et diutinum; Erythema figuratum perstans; Erythema microgyratum persistens

History
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Hutchinson 1888; Bury 1889; Radcliffe Crocker and Williams 1894

Definition
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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).

Occurrence/Epidemiology
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Rare; 100-200 cases have been described in the literature worldwide

Etiopathogenesis
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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.

Manifestation
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Mainly occurring in adults between the 3rd and 6th decade of life.

Localization
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Mainly over the extensor sides of the extremity joints (fingers, hands, knees), also buttocks, genitals, face and neck.

Clinical features
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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.

Histology
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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:

Histopathological algorithm of erythema elevatum et diutinum (lowest common denominator: italics, leading symptoms: bold) varies according to Ratzinger et al. 2105
Accentuated around post-capillary venules
Capillaries left out of the less strongly involved
perivascular leukocytoclasia
Damage to endothelial cells
Fibrin in/around vessel walls
Perivascular extravasation of erythrocytes
Edema in the papillary dermis
Collagen degeneration
Variable number of eosinophils
Plasma cells and fibrosclerosis

Differential diagnosis
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Therapy
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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.

Symptomatic therapy of itching with H 1 antagonists, e.g. desloratadine (Aerius) 1 tablet/day p.o. or levocetirizine (Xusal) 1 tablet/day p.o..

Progression/forecast
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Chronic course. Spontaneous healing is possible after years, leaving a hpo- or hyperpigmented scar.

Literature
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  1. Awan BE et al (2021) A Case of Erythema Elevatum Diutinum (EED) Exhibiting A Keloid-Like Appearance. Dermatol Ther (Heidelb) 11:2235-2240.

  2. Burnett PE et al (2003) Erythema elevatum diutinum. Dermatol Online J 9: 37
  3. 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
  4. Chen KR et al (2002) Clinical and histopathological spectrum of cutaneous vasculitis in rheumatoid arthritis. Br J Dermatol 147: 905-913.

  5. High WA et al (2003) Late-stage nodular erythema elevatum diutinum. J Am Acad Dermatol 49: 764-767.
  6. Hutchinson J (1888) On two remarkable cases of symmetrical purple congestion of the skin in patches, with induration. Br J Dermatol 1: 10-15
  7. Kim H (2003) Erythema elevatum diutinum in an HIV-positive patient.
    J Drugs Dermatol 2:411-412.
  8. Lisi S et al (2003) A case of erythema elevatum diutinum associated with antiphospholipid antibodies. J Am Acad Dermatol 49: 963-964.
  9. 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
  10. Momen SE egt al. (2014) Erythema elevatum diutinum: a review of presentation and treatment. J Eur Acad Dermatol Venereol 28:1594-1602.
  11. Radcliffe-Crocker HW, Williams C (1894) Erythema elevatum diutinum. Br J Dermatol 6: 1-9 & 53-5.
  12. Ratzinger G et al (2015) The vasculitis wheel-an algorithmic approach to cutaneous vasculitides. JDDG 1092-1118
  13. Sandhu JK et al (2019) Erythema elevatum et diutinum as a systemic disease. Clin Dermatol 37:679-683.

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Last updated on: 19.06.2022