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Erythema Elevatum Diutinum

Eed Eryth Elevatum Diutinum


Presentation

  • We present this case to illustrate the subtle, indolent clinical presentation of EED and demonstrate the uncomplicated use of topical dapsone 5% gel for rapid improvement and subsequent successful treatment of localized disease.[ncbi.nlm.nih.gov]
Generalized Lymphadenopathy
  • Treatment with dapsone was effective for his skin and nails until he developed generalized lymphadenopathy which turned out to be malignant lymphoma. Many haematological diseases, e.g.[ncbi.nlm.nih.gov]
Pseudotumor
  • This case is noteworthy clinically due to its location and its histopathological presentation that comprises a wide differential diagnosis, including inflammatory pseudotumor, dermatofibrosarcoma protuberans, superficial nodular fasciitis, hyalinized[ncbi.nlm.nih.gov]
Gangrene
  • […] vasculitis Erythema elevatum diutinum Capillaritis Urticarial vasculitis Nodular vasculitis Microvascular occlusion Calciphylaxis Cryoglobulinemic purpura / Cryoglobulinemic vasculitis vascular coagulopathy : Livedoid vasculitis Livedoid dermatitis Perinatal gangrene[en.wikipedia.org]
Disability
  • We report a patient with unusually widespread and disabling EED that had been unresponsive to corticosteroids and antibiotics, but resolved on dapsone.[ncbi.nlm.nih.gov]
Papule
  • Asymptomatic papules and plaques measuring from 0.7 to 9.0 cm were seen over the interphalangeal joints, elbows, knees, and ankles.[ncbi.nlm.nih.gov]
Pruritus
  • 678 206 Pruritus ani 686 207 Pruritus vulvae 688 208 Pseudofolliculitis barbae 691 209 Pseudoxanthoma elasticum 695 210 Psoriasis 697 211 Psychogenic excoriation 708 212 Pyoderma gangrenosum 711 213 Pyogenic granuloma 716 214 Radiation dermatitis 719[books.google.de]
  • […] herpetiformis Inflammatory Dermatitis and eczema Atopic dermatitis · Seborrhoeic dermatitis ( Dandruff, Cradle cap ) · Contact dermatitis ( Diaper rash, Urushiol-induced contact dermatitis ) · Erythroderma · Lichen simplex chronicus / Prurigo nodularis · Itch ( Pruritus[wikidoc.org]
  • Under this therapy regime, she achieved only a partial remission of the skin lesions and pruritus remained unresolved. In 2015 the patient presented with a severe relapse of the EED despite continuous treatment.[gavinpublishers.com]
  • .), or as a primary or idiopathic disease; often associated with the loss of hair and nails, hyperkeratosis of the palms and soles, and pruritus 695.89 Excludes Applies To Erythema intertrigo Intertrigo Pityriasis rubra (Hebra) ICD-9-CM Volume 2 Index[icd9data.com]
  • The lesions of EED are usually asymptomatic, but pruritus, pain, and arthralgia of the involved joints have been reported.[escholarship.org]
Exanthema
  • 709 ) Infections Bacterial skin disease Staphylococcus ( Staphylococcal scalded skin syndrome, Impetigo, Boil, Carbuncle ) · Strep ( Impetigo ) · Corynebacterium ( Erythrasma ) Viral skin disease Wart · Molluscum contagiosum · Erythema infectiosum · Exanthema[wikidoc.org]
  • […] acute attacs disappear within sevetal hours chronic forms recurr pruritus papules, wheals, edematous plaques, angioedema Pictures Red, plastic dermographism at drug reaction: Urticaria with superficial infiltrate, CLINIC (715) Urticarial toxoallergic exanthema[atlases.muni.cz]
Subcutaneous Nodule
  • The histologic findings were characterized by predominant concentric fibrosis that formed well-circumscribed dermal and subcutaneous nodules. Awareness of this unusual presentation of EED helps to avoid misdiagnosis as cutaneous neoplasms.[ncbi.nlm.nih.gov]
Subcutaneous Mass
  • One, whose histology resembled GF, had a large multinodular dermal and subcutaneous mass that persisted despite attempted resection.[ncbi.nlm.nih.gov]

Workup

  • An extensive workup was positive for underlying hepatitis B infection. Our case represents the clinicopathologic spectrum that EED can present and emphasizes the importance of searching for an underlying etiology.[ncbi.nlm.nih.gov]
  • This workup should include complete blood counts, urine analysis, and age appropriate cancer screening. CT scan of the chest, abdomen, and pelvis must also be considered if other tests are not diagnostic.[ozarkderm.com]
  • An additional infectious workup was negative for hepatitis C, streptococcus, syphilis, tuberculosis, and HIV.[mdedge.com]

Treatment

  • Treatment of these lesions is notoriously difficult. Dapsone is used as the mainstay of treatment, however other options exist. We have highlighted different treatment options and suggested a treatment algorithm.[ncbi.nlm.nih.gov]

Prognosis

  • It has many histological mimics and is often associated with a variety of underlying systemic diseases, when these are present the management and prognosis dictates the course of the EED.[ncbi.nlm.nih.gov]
  • Prognosis Although EED is a chronic condition, it is usually sensitive to treatment and the prognosis is good. The documents contained in this web site are presented for information purposes only.[orpha.net]
  • What is the Prognosis of Erythema Elevatum Diutinum? (Outcomes/Resolutions) The prognosis of Erythema Elevatum Diutinum is dependent upon the associated disorder/condition, severity of the symptoms, and the complications it presents.[dovemed.com]
  • The absence of hematological and cytogenetic response to 5-azacitidine, and consequently the progression of the AML, could explain the appearance of another neutrophilic dermatosis (EED) portending a poor prognosis.[htct.com.br]
  • Prognosis Erythema elevatum diutinum is a chronic disease that usually evolves over a 5- to 10-year period, at which point it may resolve.[emedicine.com]

Etiology

  • Erythema elevatum diutinum is a rare chronic inflammatory skin disease of unknown etiology with a histopathology of a leukocytoclastic vasculitis.[ncbi.nlm.nih.gov]

Epidemiology

  • Summary Epidemiology It is very rare, although the exact prevalence is unknown. Clinical description The disease manifests as chronic, red to purple papules, located mainly on the dorsal aspect of the main joints (knees, elbows, hands and feet).[orpha.net]
  • Epidemiology The incidence of EED is unknown; however, it appears to be a rare disease. There is no significant mortality associated with EED. It presents predominantly in males, in the fourth to sixth decade of life.[plasticsurgerykey.com]
  • Erythema elevatum diutinum has also been reported after the administration of erythropoietin. [14] Epidemiology Frequency Erythema elevatum diutinum is a rare disease.[emedicine.com]
  • This leads to repetitive damage to the vessels and ultimately develops into fibrosis. [3] [2] [1] Epidemiology EED is rare, with descriptions of only several hundred cases in the literature.[ncbi.nlm.nih.gov]
  • EPIDEMIOLOGY The incidence of EED is unknown; however, it appears to be a rare disease. There is no significant mortality associated with EED. It presents predominantly in males, in the fourth to sixth decade of life.[dermaamin.com]
Sex distribution
Age distribution

Pathophysiology

  • CONCLUSIONS: The ANCAs, particularly of IgA class, may prove to be a helpful paraclinical marker in erythema elevatum diutinum and an interesting perspective for understanding the pathophysiology of the disease.[ncbi.nlm.nih.gov]
  • Pathophysiology The pathophysiology of erythema elevatum diutinum is not well understood. According to Gibson and Su, [5] the lesions are thought to be caused by the deposition of immune complexes in small blood vessels.[emedicine.com]

Prevention

  • Newly formed vessels in granulation tissue may be more susceptible to damage by immune complexes, and the early formation of granulation tissue in EED may prevent an early resolution of vasculitis .[journals.lww.com]
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