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]
- Asymptomatic
Serum M-proteins are identified in 1% of asymptomatic healthy persons older than 50 years. This paraproteinemia without any signs and symptoms of multiple myeloma is called monoclonal gammopathy (IgA paraproteinemia). [idoj.in]
Although the lesion was otherwise asymptomatic, further investigation demonstrated an underlying IgA paraproteinaemia. [ncbi.nlm.nih.gov]
- Fever
Patient was under evaluation as an in-patient in the department of internal medicine for recurrent polyarthritis and fever. [edoj.org.eg]
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]
Systemic Implications and Complications Fever and arthralgias are reported, but extracutaneous involvement is extremely rare. Underlying conditions, as previously mentioned, are frequent but not a rule. [dermatologyadvisor.com]
No therapy was initiated for the throat infection since it was already treated with empirical antibiotics and there were no upper respiratory symptoms or fever. [dovepress.com]
- Streptococcal Infection
A streptococcal infection seemed to be the trigger factor in four of our patients. Partial control of the cutaneous lesions was achieved by the use of antibiotics. [ncbi.nlm.nih.gov]
However, screening for infectious diseases, such as HIV, tuberculosis and streptococcal infections, as well as for autoimmune disorders and hematologic conditions (especially IgA gammopathy) should be considered, even though the lack of an underlying [dermatologyadvisor.com]
Streptococcal infection was the first disorder associated with EED after the isolation of streptococci from affected skin and reproduction of lesions after intradermal injection of streptococcal antigen. [escholarship.org]
Bacterial infections, especially streptococcal infections, have been associated with EED, and typical skin lesions have been reproduced or exacerbated by the intradermal injection of streptococcal antigens. [dermaamin.com]
On this basis, a prior diagnosis of nontuberculous mycobacterial infection was made, but systemic clarithromycin was ineffective. [academic.oup.com]
- Burning Pain
There are reports of burning pain in the evening in the involved cutaneous areas. 2, 5 Histology findings vary according to the duration of the disease. [scielo.br]
Gastrointestinal
- Intestinal Disease
(tuberculosis, streptococcal infection, HIV), immunological disorders (inflammatory intestinal diseases, rheumatoid arthritis) and certain malignancies (B-cell lymphoma, squamous cell carcinoma, breast carcinoma). 2, 5 The differential diagnosis of EED [academic.oup.com]
Musculoskeletal
- Arthritis
Antineutrophil cytoplasmic antibodies in rheumatoid arthritis. Characterization and clinical correlations. Arthritis Rheum 1993; 36: 1054- 60. 13. Bosch X, Llena J, Collado A, Font J, Mirapeix E, Ingelmo M, et al. [edoj.org.eg]
Atypical presentations of the disease have also been reported in the literature such as involvement of the palms and soles and a solitary lesion in a patient with rheumatoid arthritis [3, 4]. [erar.springeropen.com]
A 53-year-old female developed erythema elevatum diutinum (EED) twelve years after the onset of rheumatoid arthritis. The arthritis had been well controlled for the last several years. [ncbi.nlm.nih.gov]
Erythema elevatum diutinum-A solitary lesion in a patient with rheumatoid arthritis. Clin Exp Dermatol 1990;15:394-5. 4. Dronda F, González-López A, Lecona M, Barros C. [idoj.in]
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]
- Ulcer
Epidermal changes of hyperkeratosis, acanthosis, ulceration, subepidermal edema, and bulla formation may also be observed. 1, 3, 16 Only gold members can continue reading. [plasticsurgerykey.com]
Peripheral ulcerative keratitis seems to be the most common ocular finding and may be the initial presenting feature of EED. [ncbi.nlm.nih.gov]
Venous ulcer Arterial insufficiency ulcer Hematopoietic ulcer Neuropathic ulcer Acroangiodermatitis Lymphedema see Template:Lymphatic vessel disease Ungrouped vascular-related cutaneous conditions Raynaud's phenomenon Thromboangiitis obliterans Erythromelalgia [en.wikipedia.org]
Occasionally, EED may present with oral ulcerations. [drwarrenrheymann.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]
- Blister
Dermatitis herpetiformis is an autoimmune blistering disease characterized by granular deposits of immunoglobulin A (IgA) in dermal papillae. Erythema elevatum diutinum is a chronic form of vasculitis associated with IgA paraproteinemia. [ncbi.nlm.nih.gov]
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]
- 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]
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]
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]