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Background: Orf virus (ORFV) is the pathogen responsible for Orf, a zoonotic viral infection that can be spread to humans from sheep and goats. Here, we present a case of human Orf complicated by an immune-related reaction, to raise awareness of this under-recognized disease avoiding unnecessary investigations and overtreatment., Case Report: A 51-year-old woman with no previous medical history presented with a one-week history of three asymptomatic swelling nodules with a grey necrotic center and red outer halo on her index finger. At physical examination there was also a pruritic papulovesicular eruption on her hands and feet. She reported a recent contact with a goat which had a similar nodular lesion in its mouth. A biopsy of the lesions was performed and a diagnosis of Orf complicated by widespread erythema multiforme was made based on the clinical and histopathological features. The lesions spontaneously resolved within the next 2 weeks., Conclusions: Orf is not very prevalent in our region, so we performed a biopsy of the lesion to guide us toward a diagnosis. However, we should remember that the diagnosis of ecthyma relies on clinical evaluation and epidemiological criteria., (© 2024. The Author(s).)

Background: Since the 2002 SCAR study, erythema multiforme (EM), a post-infectious disease, has been distinguished from Stevens-Johnson syndrome (SJS), drug-induced. Nevertheless, EM cases are still reported in the French pharmacovigilance database (FPDB)., Objectives: To describe EM reported in the FPDB and to compare the quality and the characteristics of the reports., Methods: This retrospective observational study selected all EM cases reported in the FPDB over two periods: period 1 (P1, 2008-2009) and period 2 (P2, 2018-2019). Inclusion criteria were 1) a diagnosis of clinically typical EM and/or validated by a dermatologist; 2) a reported date of onset of the reaction; and 3) a precise chronology of drug exposure. Cases were classified confirmed EM (typical acral target lesions and/or validation by a dermatologist) and possible EM (not-otherwise-specified target lesions, isolated mucosal involvement, doubtful with SJS). We concluded possible drug-induced EM when EM was confirmed, with onset ranging from 5 to 28 days without an alternative cause., Results: Among 182 selected reports, 140 (77%) were analyzed. Of these, 67 (48%) presented a more likely alternative diagnosis than EM. Of the 73 reports of EM cases finally included (P1, n=41; P2, n=32), 36 (49%) had a probable non-drug cause and 28 (38%) were associated with only drugs with an onset time ≤4 days and/or ≥29 days. Possible drug-induced EM was retained in 9 cases (6% of evaluable reports). Etiological work-up was more often performed in period 2 than 1 (53.1% vs 29.3%, P=0.04), and the time to onset from 5 to 28 days was more frequent in period 2 (59.2% vs 40%, P=0.04)., Conclusions: This study suggests that possible drug-induced EM is rare. Many reports describe "polymorphic" rashes inappropriately concluded as EM or post-infectious EM with unsuitable drug accountability subject to protopathic bias., (Copyright © 2023 Société française de pharmacologie et de thérapeutique. Published by Elsevier Masson SAS. All rights reserved.)

Cutaneous immune-related adverse events (cirAEs) are the most prevalent complication to arise from immunotherapy and cause significant morbidity. We aimed to determine the spectrum, timing, clinical features, and outcomes of cirAEs by conducting an observational pharmacovigilance study using VigiBase, the World Health Organization's global database of individual case safety reports from over 130 member countries (ClinicalTrials.gov, number NCT04898751). We compared adverse event reporting in patients who received immune checkpoint inhibitors (91,323 adverse events) with those of the full reporting database (18,919,358 adverse events). There were 10,933 cases of cirAEs within 51 distinct dermatologic types, with 27 specific eruptions with disproportionate signal represented (information component [IC] 025 > 0). Of these 27 eruptions, there were eight cirAEs with n > 100 reports, including vitiligo (IC 025  = 4.87), bullous pemphigoid (IC 025  = 4.08), lichenoid dermatitis (IC 025  = 3.69), erythema multiforme (IC 025  = 1.03), toxic epidermal necrolysis (IC 025  = 0.95), Stevens‒Johnson syndrome (IC 025  = 0.41), drug eruption (IC 025  = 0.11), and eczematous dermatitis (IC 025  = 0.11). There were differences in time to onset after immune checkpoint inhibitor initiation, with a median of approximately 1 month (erythema multiforme, Stevens‒Johnson syndrome, and toxic epidermal necrolysis), 2 months (drug eruption and eczematous dermatitis), 4 months (lichenoid dermatitis), and 5‒6 months (bullous pemphigoid and vitiligo). CirAEs are diverse, dependent on cancer type, and have distinct and different onset times that are linked to the cirAE subtype., (Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.)

A 9-year-old previously healthy boy presented with high-grade intermittent fever, severe headache associated with neck stiffness for 5 days, rash over trunk and extremities for 4 days, vomiting for 3 days and diplopia for 2 days. There was no history of seizures, abnormal body movements, altered sensorium or focal deficits. On examination, he had maculopapular erythematous rashes over the trunk and extremities and erythema multiforme. He had bilateral abducens nerve palsy and the rest of the cranial nerve, sensory and motor examination was normal. He had neck stiffness and positive Kernig's sign. Fundus examination showed grade 4 papilledema. Cerebrospinal fluid workup revealed elevated opening pressure, lymphocytic pleocytosis, normal protein and glucose levels. Neuroimaging showed features suggestive of intracranial hypertension. Borrelia IgM and IgG antibodies came positive. The uniqueness of our case lies with two rare presenting manifestations of Lyme neuroborreliosis in the same child., (© The Author(s) [2022]. Published by Oxford University Press. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Delayed-onset T-cell-mediated cutaneous adverse drug reactions are an uncommon but potentially serious result of medication exposures. Identification of culprit medications is crucial, but clinical diagnosis is often difficult. Patch tests and interferon-gamma release assays (IFNγ-RA) were previously reported as potentially useful ancillary tests, while rechallenges remain the reference standard test. We compared the number of positive test results with patch testing and IFNγ-RA for drugs implicated as possible causes of cutaneous reactions. Fifty-one patients with a suspected cutaneous drug eruption underwent patch testing and IFNγ-RA for suspected drugs. Participants were followed up at least 9 months after the onset of the rash with results compared with the clinical diagnosis. Forty-two patients presented with morbilliform/eczematous eruptions; five were diagnosed with fixed drug eruption (FDE) and four with erythema multiforme. None had positive patch testing to the drugs tested. A total of 8/51 (15.6%) patients had positive reaction by the IFNγ-RA, and an additional 11 (21.6%) patients had borderline results. Positive or borderline results were more likely in patients with FDE (80%) than morbilliform/eczematous eruptions (30.9%) or erythema multiforme (25%). Our study emphasizes the necessity of additional effective ancillary tests in the evaluation of drug eruptions and supports the use of IFNγ-RA for drug testing as a tool for identifying medications associated with cutaneous drug eruptions., (© 2021 Société Française de Pharmacologie et de Thérapeutique.)

A variety of acute oral lesions may be encountered in the scope of dermatology. Oral lesions may be single or multiple; may arise secondary to infectious, immune, congenital, medication use, or idiopathic causes; and may take a variety of forms. A thorough evaluation of the oral cavity is required to assess patients with oral lesions. Affected patients may be monitored, treated, or referred to an appropriate specialist for further management as needed. Many acute oral lesions are self-limiting in nature and patients may require only assessment and reassurance. Several common acute oral lesions are discussed in this article., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Good's syndrome (GS) is a rare, adult-onset combined B cell and T cell immunodeficiency with an associated thymoma. These patients have an increased risk of bacterial, fungal, viral and opportunistic infections. This report describes a 75-year-old female patient who presented with a full body rash and an anterior mediastinal mass. She underwent a biopsy of her rash and mass, which revealed erythema multiforme and WHO Type A thymoma, respectively. During her hospitalisation, she was also found to have oropharyngeal candidiasis, methicillin-susceptible Staphylococcus aureus bacteraemia and herpes simplex virus type 2 (HSV-2) skin lesions. Based on the number of infections and severity of her rash, an immunocompromised state was suspected. Immunological testing revealed a B cell and T cell deficiency as well as low serum immunoglobulins. This combination of hypogammaglobulinaemia and thymoma led to a diagnosis of GS. While there have been many case reports of GS, this is the first report of the immunodeficiency presenting with erythema multiforme., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

We have explored the rash that appears as target lesions, with the central and dominant diseases belonging to the Stevens-Johnson syndrome/toxic epidermal necrolysis group. After presenting the clinical patterns of an individual target lesion and classifying them into different types of lesions, the contribution has been organized with groups characterized by such specific findings according to the type of lesion: flat or raised, typical or atypical, presence or absence of fever, presence or absence of mucosal ulcerations, presence or absence of arthralgias, and/or internal organ involvement. Other specific features, such as histologic appearance, immunofluorescence findings, and laboratory changes, are considered. We provide clinicians with an algorithmic, systematic, and logical approach to diagnose the condition of the patients who present with targetoid lesions, and enable them to differentiate between those with serious systemic and life-threatening diseases from others with ordinary skin ailments., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Painful oral vesiculoerosive diseases (OVD) include lichen planus, pemphigus vulgaris, mucous membrane pemphigoid, erythema multiforme, and recurrent aphthous stomatitis. OVD lesions have an immunopathic cause. Treatment is aimed at reducing the immunologic and the following inflammatory response. The mainstay of OVD management is topical or systemic corticosteroids to include topical triamcinolone, fluocinonide, and clobetasol, whereas systemic medications used in practice can include dexamethasone, prednisone, and prednisolone. Oral herpetic lesions can be primary or recurrent. If management is desired, they can be treated by topical or systemic antiviral drugs. Topical antiviral creams include prescription acyclovir, penciclovir and over-the-counter docosanol., (Published by Elsevier Inc.)

The cutaneous manifestations of Crohn's disease are myriad. A 15-year-old girl presented with recurrent lip swelling and eventual development of diarrhea and targetoid macules on the palms, feet, and back. She was finally diagnosed with Crohn's disease in the setting of a clinical presentation and histopathology consistent with orofacial granulomatosis and erythema multiforme. We review the literature and summarize reported occurrences of these cutaneous diseases in children with Crohn's disease., (© 2018 Wiley Periodicals, Inc.)

We are currently facing a worldwide epidemic of syphilis. Clinical manifestations that are rarely seen have been encountered, leading the dermatologist to confront unusual clinical conditions in daily practice. Erythema multiforme triggered by syphilis is very rare and is also seldom reported in the literature. We report a case of secondary syphilis in an HIV-positive patient, whose clinical, pathologic and serologic features were consistent with the diagnosis of erythema multiforme triggered by syphilis.

Drug reactions and systemic diseases often involve the skin. In particular, most drug-induced reactions and many infectious diseases present with dermatologic manifestations localized acrally, that is on distal portions of limbs (hand, foot) and head (ears, nose). A detailed review of all acral dermatologic signs of drug reactions and systemic diseases is beyond the scope of this paper, although some of these disorders will be discussed specifically here., (Copyright © 2017 Elsevier Inc. All rights reserved.)

This study describes an erythema multiforme (EM) in breeding sows, after their mixing in the group housing system. Sows at 30-35 days of gestation showed red and raised skin areas, depression, anorexia, fever, respiratory problems, and increased return to estrus. Blood and nasal samples from diseased sows were examined by quantitative polymerase chain reaction and enzyme-linked immunosorbent assay for respiratory pathogens. Hematological and biochemical analyses were performed on the blood samples. From diseased sows, vaginal swabs for microbiological examinations and samples at slaughterhouse for gross and microscopic examinations were collected. Samples from the complete gestation and lactation feed were examined for mycotoxins. All sampled sows were seropositive for Actinobacillus pleuropneumoniae (App) and porcine reproductive and respiratory syndrome virus (PRRSV). No viremia for PRRSV and porcine circovirus type 2 were detected. All nasal samples were positive for Streptococcus suis, one for Swine Influenza Virus and one for App, Hemophilus parasuis, and S. suis. In all vaginal swabs, Escherichia coli and Streptococcus spp. were detected. Diseased sows had moderate leukocytosis, mild anemia, and thrombocytopenia. No mycotoxins were detected in feed. Histopathological examination revealed increased vascularization of the superficial and middle dermis. EM was likely due to illness caused by viral and bacterial infections. This study suggests that stress caused by the sows' mixing might have triggered the problem.

The objective of the current study was to characterize the epidemiology and resource use of U.S. children hospitalized with ophthalmologic disease secondary to erythema multiforme (EM), Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN). We studied children ages 5 to 19 years hospitalized in 2005 in 11 states, encompassing 38% of the U.S. pediatric population. Using International Classification of Diseases, Ninth Revision, Clinical Modification codes, we identified admissions of children with EM, SJS, or TEN and the presence of concurrent ophthalmologic disease, analyzed patient and hospitalization characteristics, and generated age- and sex-adjusted national estimates. We identified 460 children admitted with EM, SJS, or TEN, corresponding to 1,229 U.S. hospitalizations in 2005. Of the children with EM, SJS, or TEN, 60 (13.0%) had ophthalmologic disease, primarily (90.0%) disorders of the conjunctiva. Children with the highest proportions of ophthalmologic disease included those with mycoplasma pneumonia (26.7%), herpes simplex virus (15.6%), upper respiratory infection (13.9%), and lower respiratory infection (13.7%). Individuals with EM, SJS, or TEN and ophthalmologic disease were more likely than those without ophthalmologic disease to receive intensive care unit care (28.3% vs 17.0%, p = 0.03) and to be admitted to a children's hospital (63.3% vs 48.8%, p = 0.03). Ophthalmologic disease was also associated with a significantly longer median length of stay (6.0 days, interquartile range [IQR] 3-9 days vs 3.0 days, IQR 2-6 days, p < 0.001) and median hospital cost ($7,868, IQR $3,539-$17,440 vs $2,969, IQR $1,603-$8,656, p < 0.001). In children with EM, SJS, or TEN, ophthalmologic disease was most common in those with concurrent Mycoplasma pneumoniae and herpes simplex virus infections. Ophthalmologic disease was associated with considerably higher inpatient resource use in this population. Children with EM, SJS, or TEN should be screened and treated early for ophthalmologic disease to prevent morbidity and minimize long-term sequellae., (© 2013 Wiley Periodicals, Inc.)

The article reports survey data on 23 patients with erythemas, including 19 patients with herpes-associated erythema multiforme (HAEM) and 4 patients with Darier's erythema annulare centrifugum (DEAC). Patients in the initial state (baseline) and after two weeks of therapy with proteflazid were characterized by measuring the levels of Toll-like receptor (TLR) expression in peripheral blood mononuclear cells (PBMC) and in epithelial cells of the throat and the skin. The TLR expression in PBMC and skin was assessed by flow cytometry with monoclonal antibodies (ICA) (Caltag Laboratories, USA; Hycult Biotech, Netherlands) against relevant antigens. In addition, patients were also characterized by the content of subpopulations of lymphocytes expressing surface markers CD3, CD4, CD8, CD16, CD21, CD23, CD72, CD25, and HLA-DR in the peripheral blood, which was measured by flow cytometry. The therapy with proteflazid in patients with both HAEM and DEAC led to normalization of the level of both T-cell and B-cell immunity, which was manifested by an increase in the total number of lymphocytes, CD3+, CD4+, CD21+, and CD72+. Measurements of the dynamics of TLR expression in the course of immunotherapy showed an increase in the number of TLR 2, 3, 4, 7, 8, and 9 in PBMC (which was especially pronounced for TLR2) and in epithelium of the pharyngeal mucosa and skin (increased expression of TLR3, 7, and 9).

Background: Mucosal erosions in bullous diseases and leading to mucosal sequelae are widely described in toxic epidermal necrolysis (TEN). These complications cause disfigurement and functional impairment. They are more rarely reported in erythema multiforme (EM). We report a case of lip adhesion following EM induced by Mycoplasma pneumoniae., Patient and Methods: A 12-year-old boy was hospitalized in a paediatric intensive care unit. He had a prominent target skin rash on the palms and soles. Mucosal injury was associated with conjunctivitis, balanitis without dysuria and hyperalgesic stomatitis. M. pneumoniae serology was positive with immunoglobulin M. We made a diagnosis of EM secondary to M. pneumoniae infection. Two months later, the skin lesions had completely disappeared but the patient's mouth opening was limited to 25 mm and he presented bilateral adhesions between the upper and lower lips of 5mm on the right and 8mm on the left resulting in aesthetic and functional damage., Discussion: Mucosal damage and its sequelae have been widely described in TEN. Ophthalmic sequelae are more frequent. A case of labial synechiae secondary to TEN has been reported. In EM, mucosal lesions occur in 100% of cases with a further mucosal problem being present in 50% of patients. Mucosal damage has been reported during EM flares but there are no studies of side-effects after the acute episode. Oral mucosal adhesions can cause cosmetic sequelae, but above all they hinder functional prognosis. These complications must be prevented by making gutters of vestibular deepening and lip movements with maximum mouth opening several times a day, starting as soon as possible. Appropriate pain management should be undertaken to ensure patient comfort and avoid the need for analgesics and restriction of movement., Conclusion: Mucosal sequelae exist in EM. Whatever their cause, complications involving the mucosa must be prevented through early, tailored and multidisciplinary treatment. Adequate pain management must not be overlooked., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Herpes Zoster (HZ) is a Cutaneous Viral infection caused by Varicella zoster virus (VZV). Lesions of HZ are usually limited to one dermatome only but sometimes, there can be dissemination of lesions. The present case describes the role of proper examination of HZ case, which presents with disseminated lesions.

Case Study: A previously well male, 18 years of age, from a rural community, presented with three painful, itchy nodules on the fingers of his left hand, which had been present for 1 week. He had been prescribed amoxicillin clavulanate but presented again when there was no improvement after 4 days of taking antibiotics. Examination revealed three erythematous and umbilicated nodules without any halo, but with a central depression with exudate (Figure 1a). No specific treatment was instituted at this visit. One week later the patient re-presented with new erythematous lesions on the palms and dorsum of his hands. The original three lesions had improved and were drier than previously (Figure 1b, c). The new lesions disappeared after 2 weeks and the original lesions after 4 weeks, without any other treatment.

Erythema multiforme is characterized by itching macules, papules and bullae, symmetrically distributed on the dorsum of the hands. They can follow the administration of several drugs or infections with various agents, and in particular with herpes simplex virus. The recurrent variant is very rare, especially in the paediatric age group. We describe the case of a male adolescent with recurrent erythema multiforme caused by herpes virus and transient natural killer deficiency.

Alachlor and butachlor are commonly used chloroacetanilide herbicides. They are cytotoxic, but there have been rare reported cases of alachlor or butachlor induced erythema multiforme major. We report the case of a 38-year-old farmer with erythema multiforme major due to the occupational exposure to alachlor/butachlor. The patient presented to the ED because of itching. Confluent erythematous to violaceous maculopatches with bullae and erosions were seen on the trunk, both upper extremities and both lower extremities. He had no relevant past or family history of a similar skin disease. He had used alachlor/butachlor for 3 days before he developed the itch. We performed a skin incisional biopsy and found diffuse hydropic degeneration with many necrotic keratinocytes in the epidermis and mild to moderate superficial perivascular lymphocytic infiltrate admixed with neutrophils and eosinophils in the upper dermis. These results confirmed the diagnosis of erythema multiforme major. The patient was admitted and received systemic and topical steroids. After 18 days, most lesions had healed, and he was discharged., (© 2011 The Authors. EMA © 2011 Australasian College for Emergency Medicine and Australasian Society for Emergency Medicine.)

Background: Erythema multiforme (EM) is an acute disorder of the skin and mucosal membranes manifesting in the oral cavity (60-70% of all patients) as polymorphic erosive, ampullar, and bloodstained crusts. The etiology is unclear, although an autoimmune mechanism is involved. Infections and drugs have been implicated in the etiopathogenesis. With the exception of corticosteroids, no specific treatment for EM is available., Methods: Data were collected on the clinical manifestations, antecedents of viral infection, and the use of drugs substances as possible etiological factors, treatment, and response to topical and systemic corticotherapy., Results: A total of 22 patients were studied (14 males and 8 females), with a mean age of 47±20.4 years. A relationship was clearly suspected between drug use and lesion outbreak in 6 patients (27.2%). On the contrary, in 7 patients (31.8%) the triggering factor could have been herpes virus infection (herpes labialis). One half of the patients (11 cases) were classified as presenting minor EM, 36.4% (8 cases) presented major forms of the disease, and 13.6% (3 cases) were classified as corresponding to Stevens-Johnson syndrome. Systemic and/or topical corticosteroids proved effective in controlling the outbreaks in all of our patients., Conclusion: The oral mucosa is the most affected mucosal region in EM, with a predilection for the lip mucosa, erosive forms and bloodstained crusts. Systemic corticosteroids are effective in controlling the outbreaks, although their use as maintenance therapy is not clearly indicated., (© 2010 John Wiley & Sons A/S.)

Erythema multiforme (EM) is usually caused by drug reactions or virus infection. We report a case of secondary syphilis presenting as EM in an HIV-infected patient, proved by immunohistochemical staining, which is rare in the literature. It is valuable to determine the etiology of EM to optimize treatment.