Presentation
A 55-year-old woman presented with erythematous, violaceous papules, plaques, and annular lesions over her upper and lower limbs since 2 years. [idoj.in]
It presents predominantly in males, in the fourth to sixth decade of life. [plasticsurgerykey.com]
Case report A 32-year-old otherwise healthy man presented with an 8-year history of a tender skin lesion on his right hand. [erar.springeropen.com]
Several clinical presentations as well as many associated diseases are reported in the literature. [escholarship.org]
“Erythema elevatum diutinum presenting with a giant annular pattern”. Int J Dermatol. vol. 48. 2009. pp. 290-2. (An interesting case report with abundant clinical presentation.) Gibson, LE, el-Azhary, RA. “Erythema elevatum diutinum”. [dermatologyadvisor.com]
Entire Body System
- Pain
The patient had no history of other respiratory symptoms, abdominal pain, or joint pain. Skin examination revealed multiple purpuric macules and patches affecting both palms (Figure 1). Involvement of the right palm was more prominent. [dovepress.com]
The patient also reported a constant painful sensation, which worsened in the evening and after exposure to low temperatures. [academic.oup.com]
A young woman with recurrent painful lesions on the dorsal aspects of her hands associated with arthralgia presented with pain and redness of both eyes. [ncbi.nlm.nih.gov]
She denies any other associated symptom as fever sensation, headache, chest pain, dyspnea, abdominal pain, vomiting, diarrhea, muscle ache or any other. [odermatol.com]
- Recurrent Bacterial Infection
Both patients developed EED in the setting of chronic recurrent bacterial infections. [ncbi.nlm.nih.gov]
The cause of EED is not yet defined, but it has been associated with the following conditions: Granuloma faciale Recurrent bacterial infections (especially streptococci) Viral infections (including hepatitis B and HIV) Haematological diseases Rheumatological [dermnetnz.org]
Disorders that have been associated with erythema elevatum diutinum include recurrent bacterial infections (especially streptococcal), viral infections (including hepatitis B or HIV), [9] rheumatologic disease (in the Bury type), lupus erythematosus, [emedicine.com]
Respiratoric
- Pneumonia
The patient was treated for interstitial pneumonia and presently has no respiratory complaints. [cdriadvlkn.org]
Polymerase chain reaction (PCR) on nasopharyngeal swabs was negative for adenovirus, rhinovirus, coronavirus, influenza viruses, respiratory syncytial virus, and Mycoplasma pneumoniae. Blood cytomegalovirus PCR was negative. [dovepress.com]
During this period, the patient had multiple infections with progressive deterioration of performance status, culminating in his death in January 2015 with pneumonia and AML refractory to intensive chemotherapy induction and 5-azacitidine. [htct.com.br]
Past medical history included breast cancer ( high grade ductal carcinoma-in-situ), intraductal papillary-mucin neoplasia of the pancreas and recurrent pneumonia. [gavinpublishers.com]
Jaw & Teeth
- Oral Ulcers
Occasionally, EED may present with oral ulcerations. [drwarrenrheymann.com]
Maruthappu T, Tharakaram S, Calonje E, Shirlaw PJ y Setterfield J, Erythema elevatum diutinum with oral ulceration, bjd 2012; 167:222-4. [medigraphic.com]
[…] loss of the uvula. ( 28443312 ) Kentley J....Bewley A. 2017 15 Erythema elevatum diutinum involving palms and soles: a case report and literature review. ( 28469800 ) Keyal U....Liu Y. 2017 16 Complete resolution of erythema elevatum diutinum using oral [malacards.org]
Maruthappu T, Tharakaram S, Calonje E, Shirlaw PJ, Setterfield J (2012) Erythema elevatum diutinum with oral ulceration. Br J Dermatol 167(1): 222-224. [biomedres.us]
Erythema elevatum diutinum with oral ulceration. Br J Dermatol. 2012;167(1):222–224. doi:10.1111/j.1365-2133.2012.10851.x 21. Davatchi CC, Farsinejad K, Nikoo A, Akhyani M, Daneshpazhooh M, Barzegar M. [dovepress.com]
Cardiovascular
- Hypertension
His family history included hypertension and a recently diagnosed polymyalgia rheumatica in his mother. [erar.springeropen.com]
There was no history of respiratory tract infections, gastrointestinal disturbances, photosensitivity, diabetes, hypertension and tuberculosis. She was on dapsone (100 mg daily) since 7 years for the raised lesions. [ijdvl.com]
[…] high potency topical corticosteroids, sulfapyridine, and niacinamide combined to tetracycline can be tried. 1, 5 Residual hyperpigmentation with occasional atrophy is common after regression of the lesions. 3 A 58-year-old mulatto male patient, with hypertension [scielo.br]
Delivery Models Health Care Economics, Insurance, Payment Health Care Policy Health Care Quality Health Care Reform Health Care Safety Health Care Workforce Health Disparities Health Informatics Health Policy Hematology History of Medicine Humanities Hypertension [jamanetwork.com]
- Vascular Disease
Peripheral ulcerative keratitis (PUK) is a complication of collagen-vascular diseases such as rheumatoid arthritis (RA) and other systemic vasculitides. We report three cases of erythema elevatum diutinum with PUK. [ncbi.nlm.nih.gov]
Skin disease Vascular disease Vasculitis Keyword (es) Dermatología Eritema elevado diutinum Dermatosis neutrófila Aparato circulatorio patología Piel patología Vaso sanguíneo patología Vasculitis Classification Pascal 002 Biological and medical sciences [pascal-francis.inist.fr]
recurrent streptococcal bacterial infections, hepatitis B and HIV, rheumatological disease, collagen vascular diseases, monoclonal gammopathies and lymphomas. [journals.lww.com]
collagen vascular diseases, hematological disorders, inflammatory bowel disease, B-Cell lymphomas, Myeloproliferative disorders.[ 2 ] Unilateral EED is a rare occurrence. [edoj.org.eg]
Musculoskeletal
- Joint Swelling
[…] history of migratory large joints (wrists, elbows, knees, and ankles) swelling and nodules on elbows and hands was admitted for pain control and nodules on hands. [vgrd.org]
Skin
- Papule
Figure 1: Multiple erythematous to violaceous papules, annular plaques over upper limbs Click here to view Figure 2: Multiple erythematous to violaceous papules, annular plaques and purpura, healed hyperpigmentation and atrophy over lower limbs Click [idoj.in]
Figure 165-2 Multiple erythematous scaly papules coalescing into plaques on extensor elbow. Figure 165-3 Crusted brown and violaceous papules over knee and anterior thigh. [plasticsurgerykey.com]
Created: 5th May 2016 | Last Updated: 23rd May 2016 Introduction 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 [pcds.org.uk]
Physical examination revealed erythematous to skin colored papules and nodules on her both elbows and forearm. Moreover, she had bilateral reddish to purple macules and papules on her legs. [jaad.org]
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]
- Dermatitis
[…] by the direct immunofluorescence in the perilesional skin of patients with dermatitis herpetiformis. [5] The close association of IgA with erythema elevatum diutinum and dermatitis herpetiformis is well known. [idoj.in]
We report a patient with dermatitis herpetiformis who developed characteristic erythema elevatum diutinum lesions on the elbows and knees despite sulfone therapy. [ncbi.nlm.nih.gov]
Convert to ICD-10-CM : 695.89 converts approximately to: 2015/16 ICD-10-CM L26 Exfoliative dermatitis Or: 2015/16 ICD-10-CM L30.4 Erythema intertrigo Or: 2015/16 ICD-10-CM L53.8 Other specified erythematous conditions Or: 2015/16 ICD-10-CM L92.0 Granuloma [icd9data.com]
- 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]
The onset of new lesions may be associated with symptoms of pruritus, burning, stinging sensations, paresthesias, or may be completely asymptomatic. [plasticsurgerykey.com]
The lesions of EED are usually asymptomatic, but pruritus, pain, and arthralgia of the involved joints have been reported. [escholarship.org]
- Skin Rash
However she developed a hypersensitivity syndrome compatible with DRESS with fever, skin rash and acute hepatitis confirmed in vitro by the presence of circulating dapsone- specific T cells. [ncbi.nlm.nih.gov]
Since then she had had intermittent episodes of arthritis with significant morning stiffness and skin rashes mainly over the extensor aspects. [edoj.org.eg]
Showing of 8 | 80%-99% of people have these symptoms Arthralgia Joint pain 0002829 Skin rash 0000988 Vasculitis in the skin 0200029 30%-79% of people have these symptoms Abnormal blistering of the skin Blistering, generalized Blisters [ more ] 0008066 [rarediseases.info.nih.gov]
Psychiatrical
- Suggestibility
Intradermal injection of streptococcal antigens into normal skin of EED patients has been reported to elicit vasculitis, which may suggest an abnormal chemotaxic response in these patients. [dermatologyadvisor.com]
Histology showed a leucocytoclastic vasculitis, suggestive of the diagnosis of EED. Screening for an associated pathology, namely a paraproteinemia or a solid cancer, was negative. Treatment with dapsone leads to amelioration within few weeks. [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]
Consequently, diagnosis of leukocytoclastic vasculitis with the suggestion of early EED was made and vasculitis workup was performed. [dovepress.com]
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]
Other Pathologies
- Fibrinoid Necrosis
Histopathologic findings are predominantly those of a chronic LCV, with thickening of blood vessel walls, mural and luminal neutrophilia, vascular occlusion, fibrinoid necrosis of vessel walls, endothelial cell swelling, leukocytoclasia, and dermal neutrophilia [plasticsurgerykey.com]
Histologically, there is leukocytoclastic vasculitis with fibrinoid necrosis of the upper and mid dermal vessel walls. [jddonline.com]
A biopsy of the skin lesion showed a significant increase of neutrophils, fragmented nuclei, and fibrinoid necrosis of vascular tissue in the middle and deep layers of the dermis. The patient's condition was stabilized after treatment of IVIg. [authorea.com]
In addition to the characteristic polymorphonuclear fragmentation, endothelial swelling, extravasation of erythrocytes and fibrinoid necrosis of vessel walls, dermal nodules of the disease contain spindle cells and fibrosis. [dvkeywords.blogspot.com]
- Lymphocytic Infiltrate
[…] papules or plaques Histology: Dense lymphocytic perivascular infiltrate, superficial and deep. [atlases.muni.cz]
Treatment
B Significant reduction of the size of the lesion during treatment. C Near-complete resolution of the lesion after 2 weeks of treatment with dapsone 100 mg BID without any recurrence after 2 years of follow-up. [erar.springeropen.com]
Patient Management Treatment of underlying condition, if any is found, is encouraged and may improve response to specific therapy. [dermatologyadvisor.com]
Colchicine treatment was started and a complete response was obtained. [archivesofrheumatology.org]
Treatment was started with dapsone 100 mg/day orally. Healing of lesions was observed after 48 h of treatment [Figure 7] and [Figure 8]. Lesions recurred after 2 weeks, and she was started on doxycycline 100 mg/day. [idoj.in]
Treatment was started with dapsone 50 mg/day orally. After 4 weeks of treatment, we noticed a decrease in the thickness of plaques and nodules and a regression of pruritus and tenderness. [escholarship.org]
Prognosis
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]
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. [orpha.net]
Introduction Define/describe the condition Anatomy Describe the pertinent anatomy Pathogenesis Describe the biomechanics/biologic basis of the disorder or the mechanism of injury Natural History Describe the natural history, epidemiology and prognosis [orthopaedicsone.com]
Etiology
Case Report Open Access Published: 26 July 2022 Egyptian Rheumatology and Rehabilitation volume 49, Article number: 45 (2022) Cite this article Abstract Background Erythema elevatum diutinum (EED) is a rare disease with unknown etiology, various presentations [erar.springeropen.com]
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
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]
Introduction Define/describe the condition Anatomy Describe the pertinent anatomy Pathogenesis Describe the biomechanics/biologic basis of the disorder or the mechanism of injury Natural History Describe the natural history, epidemiology and prognosis [orthopaedicsone.com]
EPIDEMIOLOGY The incidence of EED is unknown, with only about 250 cases reported in the literature.7 A case series, culled from a tertiary care center, and spanning 60 years, contained only 13 cases.8 EED may present at any age but is most common in the [accessmedicine.mhmedical.com]
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]
Sections Continuing Education Activity Introduction Etiology Epidemiology Pathophysiology Histopathology History and Physical Evaluation Treatment / Management Differential Diagnosis Enhancing Healthcare Team Outcomes Review Questions References Similar [pubmed.ncbi.nlm.nih.gov]
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]
So, every case of it merits further investigation for better understanding of its causes and pathophysiology. [erar.springeropen.com]
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]
Sections Continuing Education Activity Introduction Etiology Epidemiology Pathophysiology Histopathology History and Physical Evaluation Treatment / Management Differential Diagnosis Enhancing Healthcare Team Outcomes Review Questions References Similar [pubmed.ncbi.nlm.nih.gov]
Prevention
Erythema Prevention lies in what ultimately causes your erythema. [sunsaferx.com]
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]
ABSTRACT: Objectives: The time that drugs for moderate-to-severe psoriasis take to... more The Role of Topical Retinoids in Prevention and Treatment of Atrophic Acne .... ABSTRACT: Atrophic acne scarring is a frequent occurrence among acne patients. [jddonline.com]
Transplantation Substance Use and Addiction Surgery Surgical Innovation Surgical Pearls Teachable Moment Technology and Finance The Rational Clinical Examination Tobacco and e-Cigarettes Toxicology Trauma and Injury Treatment Adherence United States Preventive [jamanetwork.com]