Erythema elevatum diutinum L95.1

Hutchinson 1888; Bury 1889; Radcliffe Crocker and Williams 1894

Rare, harmless, eminently chronic (diutinum!), inflammatory disease of the skin with the characteristic histological picture of leukocytoclastic "small vessel" vasculitis with angiocentric and storiform fibrosis.

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

Unexplained, probably infect-allergic vasculitis of small vessels; occasional detection of monoclonal immunoglobulins, most frequently IgA-gammopathies, 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, myelodysplasia have been described.

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

Subacute development of symmetrically arranged, initially soft, later firm, roundish, polycyclic, nodular, blue-red or red-brown, also haemorrhagic, succulent large-area plaques. (Remark: A purple component is not always clearly visible because the small focal bleedings occur intermittently). Accumulates central indentation, change of consistency, possibly stabbing pain and burning, itching.

Possible syntropia with gout and arthralgia.

The lesions can heal after years (periods of 5-35 years are given) leaving a hyperpigmented scar.

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:

If necessary, treatment of the underlying disease.

The best results are described with DADPS. Patients respond to different doses (50-150 mg/day). Improvement occurs immediately within days in some patients, in others the skin changes only heal over months. Therapy failures are known.

The clinical picture usually responds less to glucocorticoids.

The use of nicotinamide (e.g. Nicobion 1 tbl/day) is described as successful in individual cases.

Symptomatic therapy of itching with _H1 antagonists, e.g. desloratadine (Aerius) 1 tbl/day p.o. or levocetirizine (Xusal) 1 tbl/day p.o.

1. Burnett PE et al (2003) Erythema elevatum diutinum. Dermatol Online J 9: 37
2. 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
3. Chen KR et al (2002) Clinical and histopathological spectrum of cutaneous vasculitis in rheumatoid arthritis. Br J Dermatol 147: 905-913
4. High WA et al (2003) Late-stage nodular erythema elevatum diutinum. J Am Acad Dermatol 49: 764-767
5. Hutchinson J (1888) On two remarkable cases of symmetrical purple congestion of the skin in patches, with induration. Br J Dermatol 1: 10-15
6. Kim H (2003) Erythema elevatum diutinum in an HIV-positive patient.
   J Drugs Dermatol 2:411-412.
7. Lisi S et al (2003) A case of erythema elevatum diutinum associated with antiphospholipid antibodies. J Am Acad Dermatol 49: 963-964
8. 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
9. Momen SE egt al (2014) Erythema elevatum diutinum: a review of presentation and treatment. J Eur Acad Dermatol Venereol 28:1594-1602
10. Radcliffe Crocker HW, Williams C (1894) Erythema elevatum diutinum. Br J Dermatol 6: 1-9 & 53-5
11. Ratzinger G et al. (2015) The Vasculitis Wheel-an algorithmic approach to cutaneous vasculitis. JDDG 1092-1118