Unusual presentation of erythema elevatum diutinum with. Erythema elevatum diutinum eed is a rare type of necrotising vasculitis that is characterised by red, purple, brown or yellow papules raised spot, plaques, or nodules, found on the backs of the hands, other extensor surfaces overlying joints, and on the buttocks. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. In this conditions, especially in pyoderma gangrenosum pg, positive pathergy may have different. It was later classified as chronic leukocytoclastic vasculitis in 1929. Vascular infiltration in the upper and middermis with predominant neutrophils and fewer lymphocytes, eosinophils and plasma cells figures 3, 4, 5. Barzegar m, davatchi cc, akhyani m, nikoo a, daneshpazhooh m 2009 an atypical presentation of erythema elevatum diutinum involving palms and soles. Erythema elevatum diutinum primary care dermatology society.
Erythema elevatum diutinum annals academy of medicine. Erythema elevatum diutinum eed is a rare, chronic dermatosis that is characterized by redviolet to redbrown papules, plaques, and. Chronic vasculitis with many neutrophils, leukocytoclasia and dense perivascular infiltrate. Special stains for mycobacteria and fungi were negative.
Erythema elevatum diutinum eed is a chronic and rare leukocytoclastic vasculitis, characterized by redpurple plaques, papules and nodules, found mainly over the extensor surfaces of the. Erythema elevatum diutinum is equally seen in both genders. Erythema elevatum diutinum was first described by hutchinson in 1878. Erythema elevatum diutinum is a rare form of lcv that has its major manifestation in the skin. Its incidence peaks between the third and sixth decades of life, and few cases have been reported in pediatric patients. We present a case of eed with widespread lesions involving the hands, extensor arms and legs, and trunk. Patients with erythema elevatum diutinum eed usually present with a history of chronically persistent or intermittently recurrent skin lesions on the extensor surface of limbs and joints that may be asymptomatic, painful or pruritic, and may be associated with systemic symptoms, such as fever and arthralgia. A distinctive vasculitis with acuteonchronic features dear editor, a 53yearold man presented at our institution in june 2012 for a painful rash on his hands and feet.
The most prominent involvement occurs on the extensor surfaces, such as the knees, elbows, and dorsa of the hands fig. These lesions are located mainly on the knees, elbows, hands, feet, face, genitals and buttocks. Jaad im certain that every dermatologist myself included has a diagnostic blind spot. The only systemic manifestation is arthralgia, which occurs in 20% to 40% of patients. These lesions are usually distributed on the extensor surfaces of the body. Erythema elevatum diutinum eed is a rare, chronic, cutaneous small vessel vasculitis of unclear pathogenesis. He had been treated prior to hospitalisation with topical and oral antifungals and antibiotics without success. With the diagnosis of erythema elevatum diutinum he was prescribed dapsone 100 mgd after g6pd estimation was normal. Histology of an erythematous plaque showed leukocytoclastic. The most common association in our series was with hypergammaglobulinemia. First described by hutchinson in 1878, it received. Erythema elevatum diutinum eed is a rare and chronic infl ammatory disorder involving small vessels. Erythema elevatum diutinum is a rare type of chronic cutaneous vasculitis characterized by red, purple, brown or yellow papules, plaques thick, red patches of skin, or nodules.
Histology revealing a chronic psoriasiform dermatitis with verrucous. Histopathological analysis revealed leukocytoclastic vasculitis, confirming the clinical suspicion. Erythema elevatum diutinum eed is a rare chronic dermatosis and majority comprises adult patients. Mar 22, 2019 erythema elevatum diutinum eed is a rare type of leukocytoclastic vasculitis characterized by red, purple, brown, or yellow papules, plaques, or nodules. Erythema elevatum diutinum is a rare chronic leukocytoclastic vasculitis of unknown etiology. Erythema elevatum diutinum depicted by erythematoustoviolaceous papules and plaques affecting the extensor surfaces of the patients arms, legs, and buttocks. Granuloma faciale gf and erythema elevatum diutinum eed are two rare related dermatoses with unknown pathogenesis. Erythema elevatum diutinum is a rare chronic leukocytoclastic vasculitis of. In erythema elevatum diutinum, there is sparing of the papillary and periadnexal dermis figures 1, 2.
Elazhary, md, phd e rythema elevatum diutinum eed is a distinctive form of chronic cutaneous vasculitis. Jul 01, 2011 skin nontumor erythema elevatum diutinum. Described over 100 years ago, the early descriptions are still an excellent source for information regarding the symptoms and signs of this unusual disease. Erythema elevatum diutinum erythema elevatum diutinum nodular lesions of erythema elevatum diutinum in patients infected with the human immunodeficiency virus erythema elevatum diutinum in a patient with crohns disease the evolution of lesions in erythema elevatum diutinum. Granuloma faciale and erythema elevatum diutinum in relation. Focal leukocytoclastic vasculitis with a fairly dense perivascular and interstitial infiltrate composed of neutrophils, lymphocytes, histiocytes, and leukocytoclastic debris. The primer will educate trainees, update established clini cians, and help health care providers from all walks of the profession provide better. Erythema elevatum diutinum is a rare condition representing a chronic leuko cytoclastic vasculitis.
Histologic leukocytoclastic vasculitis is a key confirmatory diagnostic feature. On this basis, we confirmed the clinical suspicion of erythema elevatum diutinum eed. In women, it usually occurs at an earlier age and is accompanied by a rheumatologic disease. Erythema elevatum diutinum is typically characterized by acute histological features which contrast with the chronic clinical course. Erythema elevatum diutinum an overview sciencedirect.
Erythema elevatum diutinum eed is a rare form of cutaneous small vessel vasculitis characterised by red, purple, brown, or yellow papules, plaques, or nodules, presenting symmetrically on extensor surfaces, most commonly the hands, elbows, knees, and achilles tendons. Classically, lesions present as symmetric red to purple plaques, papules, and nodules overlying joints. Figure 1655 early stage lesion of erythema elevatum diutinum. Erythema elevatum diutinum is an unusual form of small vessel vasculitis that is characterized by erythematous or violaceous papules, plaques, and nodules over the dorsal hands, ears, knees, heel, and buttocks.
Erythema elevatum diutinum genetic and rare diseases. Erythema elevatum diutinum is a syndrome of vasculitis in which lesions, typically over the extensor surfaces, show a mixed inflammatory infiltrate on biopsy. Erythema elevatum diutinum is a rare, chronic cutaneous vasculitis that presents with plaques or nodules on the extensor surfaces of extremities. A 5mm punch biopsy was performed and histological examination revealed a leukocytoclastic vasculitis with neutrophilic perivascular infiltrates, dermal fibrin deposits and endothelial expansion.
Pathology of erythema elevatum diutinum dr sampurna roy md. Erythema elevatum diutinum an overview sciencedirect topics. A clinical and histopathologic study of patients james a. Erythema elevatum diutinum eed is a chronic form of cutaneous small vessel vasculitis consisting of violaceous, redbrown, or yellowish papules, plaques, or nodules that favor the extensor surfaces picture 1ad. Dr harriet cheng, dermatologist, waikato hospital, hamilton, new zealand. Lesions are usually asymptomatic, although burning and itchiness can. Erythema elevatum diutinum in a patient with relapsing polychondritis. Erythema elevatum diutinum eed is a chronic form of leukocytoclastic vasculitis consisting of violaceous, redbrown, or yellowish papules. There are reports in the literature documenting the occurrence of iga ancas in patients with neutrophildriven dermatoses such as henochschonlein purpura, inflammatory bowel disease, and various cutaneous vasculitides, including eed. Introduction erythema elevatum diutinum eed is a rare. Cockerell, nodular lesions of erythema elevatum diutinum in patients infected with the human immunodeficiency virus, journal of the american academy of dermatology, 28, 6, 919, 1993. Histopathological evaluation of behcets disease and.
Characteristic skin lesions are asymptomatic pain is occasional, plum colored erythemtous nodules, and plaques of variable size distributed symmetrically over extensors of extremities particularly over the joints, dorsal hands and feet, knees and elbows, buttocks and achilles tendon, and. Erythema elevatum diutinum eed is a rare chronic cuta neous vasculitis that affects adults between 30 and 60 years of age, with no race or gender predilection. Neutrophilic dermatoses pyoderma gangrenosum, sweet syndrome, and erythema elevatum diutinum are also known for positive pathergy reaction. Erythema elevatum et diutinum as a systemic disease. Erythema elevatum diutinum is a disease characterized by red, pink, purple and yellow cutaneous papules, nodules, and plaques distributed mainly over extensor surfaces.
It is a rare, chronic and progressive disease affecting mostly extensor surfaces and skin overlying the joints. Histology epidermis is slightly acanthotic, sometimes infiltrated with neutrophils. Oct 18, 2011 pathergy is one of the diagnostic criteria for bd and accepted as a sign for the active disease. Rare presentation of erythema elevatum diutinum charlotte paugam, msc,a othman laghmari, md,b jeanjacques renaut, md,b and sebastien barbarot, md, phda nantes, france key words.
The term diutinum is the latin word for long lasting. Erythema elevatum diutinum was first described in 1888 by hutchinson and in 1889 by bury. Histopathology revealed dermal infiltrates of histiocytes. Erythema elevatum diutinum eed is classified within the small vessel vasculitis. Erythema elevatum diutinum sharma v, mahajan vk, mehta ks. Apr, 2010 erythema elevatum diutinum is a rare type of chronic cutaneous vasculitis characterized by red, purple, brown or yellow papules, plaques thick, red patches of skin, or nodules.
678 1214 1599 90 1130 524 46 400 133 421 722 172 357 1114 1340 1525 629 1564 873 972 1155 518 479 810 1222 1393 249 1200 1393 1420 152 261 1096 749 1131 761 872 685