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erythema elevatum diutinum
ICD-10 Codes
Related ICD-10:
Description
Erythema Elevatum Diutinum (EED): A Rare Cutaneous Vasculitis
Erythema elevatum diutinum (EED) is a rare and chronic form of cutaneous small vessel vasculitis. It is characterized by the presence of violaceous, red-brown, or yellowish papules, plaques, or nodules on the skin.
Key Features:
- Age and Demographics: EED typically affects adults between 30 and 60 years of age, with no specific racial or gender predilection [3].
- Skin Manifestations: The disease presents as chronic, red to purple papules, mainly located on the dorsal aspect of joints (knees, elbows, hands, and feet) [5].
- Symmetry: EED often manifests as largely symmetric papules and plaques [6].
Other Characteristics:
- Chronic Progression: EED is a chronic and progressive disease that can affect skin overlying joints and extensor surfaces [9].
- Localized Cutaneous Leukocytoclastic Vasculitis: It is a rare form of localized cutaneous leukocytoclastic vasculitis, characterized by edematous papules and plaques [8].
References:
[1] Context 3 [2] Context 5 [3] Context 3 [4] Context 7 [5] Context 5 [6] Context 6 [7] Context 7 [8] Context 8 [9] Context 9
Additional Characteristics
- violaceous, red-brown, or yellowish papules, plaques, or nodules
- chronic, red to purple papules
- edematous papules and plaques
Signs and Symptoms
Erythema elevatum diutinum (EED) is a rare type of necrotizing vasculitis that presents with distinct signs and symptoms.
Key Signs and Symptoms:
- Papules, Plaques, or Nodules: EED manifests as chronic, red to purple papules, plaques, or nodules on the skin [1][2].
- Location: These lesions are typically found on the dorsal aspect of main joints (knees, elbows, hands, and feet) [3].
- Asymptomatic Lesions: Most lesions are asymptomatic, but some patients may experience:
- Pruritus (itching)
- Tenderness
- A burning sensation after the onset of new lesions [4]
- Painful or Pruritic Lesions: Some lesions can be painful or pruritic, especially in cases where arthralgia is present [5].
- Symmetric Distribution: EED often presents with largely symmetric papules and plaques on the skin [7].
Common Associated Symptoms:
- Arthralgia: The most common associated symptom is arthralgia (joint pain) [6].
It's essential to note that these signs and symptoms can vary in severity and presentation among individuals. If you suspect EED, consult a medical professional for proper diagnosis and treatment.
References:
[1] Context result 1 [2] Context result 2 [3] Context result 4 [4] Context result 3 [5] Context result 5 [6] Context result 5 [7] Context result 7
Additional Symptoms
- Arthralgia
- Tenderness
- Papules, Plaques, or Nodules
- A burning sensation after the onset of new lesions
- Painful or pruritic Lesions
- itching
Diagnostic Tests
Diagnostic Tests for Erythema Elevatum Diutinum
Erythema elevatum diutinum (EED) is a rare type of leukocytoclastic vasculitis, and its diagnosis can be challenging. However, several diagnostic tests can help establish the condition.
- Skin Biopsy: A
Additional Diagnostic Tests
- Blood Tests
- Skin Biopsy
Treatment
Treatment Options for Erythema Elevatum Diutinum
Erythema elevatum diutinum (EED) is a rare skin condition characterized by chronic inflammation and the formation of papules, nodules, or plaques. The treatment of EED can be challenging due to its recurrent nature.
First-Line Treatment: Dapsone
Dapsone is considered the first-line treatment for EED [5]. It has bactericidal and bacteriostatic properties against mycobacteria, making it an effective option for managing this condition [5]. However, treatment with dapsone must be monitored clinically and through blood examinations to avoid potential side effects [7].
Alternative Treatment Options
Other treatment options for EED include:
- Niacinamide: A derivative of vitamin B3 that has anti-inflammatory properties [6].
- Colchicine: An antifungal medication that can help reduce inflammation [8].
- Hydroxychloroquine: An antimalarial drug that may be effective in treating EED [8].
- Clofazimine: A sulfone antibiotic that can be used as an alternative to dapsone [3].
- Cyclophosphamide: A chemotherapy medication that may be considered for severe cases of EED [4].
Treatment Algorithm
A treatment algorithm has been suggested, which includes the use of dapsone as the mainstay of treatment, with other options being considered based on individual patient responses and disease severity [4].
It's essential to note that each patient's response to treatment may vary, and a multidisciplinary approach involving dermatologists, rheumatologists, and other healthcare professionals may be necessary to manage this complex condition.
References:
[3] - What is the treatment for erythema elevatum diutinum? [4] - by SE Momen · 2014 · Cited by 114 — Dapsone is used as the mainstay of treatment, however other options exist. We have highlighted different treatment options and suggested a treatment algorithm. [5] - Mar 22, 2019 — Dapsone is the drug of choice. It is bactericidal and bacteriostatic against mycobacteria; its mechanism of action is similar to that of ... [6] - by IK Kohler · 1980 · Cited by 69 — A 60-year-old woman with recurrent papular and vesiculobullous lesions of erythema elevatum diutinum responded to treatment with 100 mg of oral niacinamide ... [7] - Dapsone is the first line of treatment for EED. Treatment must be monitored clinically and by blood examination. In cases were dapsone is inefficient or poorly ... [8] - by ME García-Meléndez · 2015 · Cited by 17 — Treatment modalities include the use of colchicine, hydroxychloroquine, clofazimine, and cyclophosphamide in addition to dapsone.
Recommended Medications
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Differential Diagnosis
Erythema elevatum diutinum (EED) is a rare type of leukocytoclastic vasculitis characterized by red, purple, brown, or yellow papules, plaques, or nodules. When considering the differential diagnosis for EED, several conditions should be taken into account.
- Granuloma faciale: This condition is thought to represent the same disorder as EED but with a different presentation. It is characterized by more eosinophils and plasma cells, and lacks granulomas [8].
- Sweet Syndrome (Acute Febrile Neutrophilic Dermatosis): This is another type of neutrophilic dermatosis that can present with similar symptoms to EED, including fever and skin lesions [1].
- Dermatofibroma: A benign skin growth that can be mistaken for EED due to its similar appearance [1].
- Erythema Multiforme: An immune-mediated condition characterized by target-shaped lesions on the skin, which can be confused with EED [2].
- Granuloma Annulare: A type of granulomatous dermatosis that presents with annular or ring-shaped lesions, which can be similar to EED in appearance [3].
- Sarcoidosis: An autoimmune condition characterized by the formation of granulomas in various parts of the body, including the skin. It can present with symptoms similar to EED [4].
It's essential to note that EED should be distinguished from other types of papular or nodular dermatoses, as well as fibrous tumors and xanthomas [10]. A thorough examination and diagnostic workup are necessary to accurately diagnose EED and rule out these differential diagnoses.
References: [1] - Context result 1 [2] - Context result 2 [3] - Context result 3 [4] - Context result 5 [8] - Context result 8 [10] - Context result 10
Additional Differential Diagnoses
- Dermatofibroma
- Granuloma faciale
- Sweet Syndrome (Acute Febrile Neutrophilic Dermatosis)
- erythema multiforme
- sarcoidosis
- granuloma annulare
Additional Information
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- A vasculitis characterized 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.
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