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We report a case of a patient with erythema multiforme major following COVID-19 (coronavirus disease 2019) vaccination. Lesions on skin and mucous membranes developed 48 h after the second dose of the mRNA-vaccine BNT162b2 (Tozinameran, Comirnaty®). Under the application of external glucocorticoids complete resolution was achieved within 3 weeks.Es wird über den Fall eines ausgeprägten Erythema exsudativum multiforme infolge einer COVID-19-Impfung berichtet. Die Effloreszenzen an Haut und Schleimhaut entwickelten sich 48 h nach der zweiten Dosis des mRNA-Impfstoffs BNT162b2 (Tozinameran, Comirnaty®). Unter der Anwendung glukokortikoidhaltiger Externa kam es innerhalb von 3 Wochen zu einer folgenlosen Abheilung.

Der Begriff „Allergie“ wurde 1906 von dem Wiener Padiater Clemens von Pirquet gepragt. Die vielfaltigen pathophysiologischen Reaktionen im Rahmen immunologischer Ablaufe von Allergien wurden jedoch erst 57 Jahre spater, 1963, durch Robert R.A. Coombs und Philip G.H. Gell [1] durch die Einteilung in 4 Typen geordnet. Die Typen I bis III sind humoral vermittelt, wahrend der Typ IV zellular, durch sensibilisierte T-Lymphozyten bedingt ist. T-Lymphozyten wirken uber entzundungsfordernde „Lymphokine“. Die von Stevens und Johnson beschriebenen Haut- und Schleimhautveranderungen werden zwischenzeitlich als Reaktionen im Sinne einer Typ-IV-Allergie eingestuft. Nachfolgend berichten die Autoren uber zwei Jungen im Alter von 7 und 8 Jahren, die mit auffalligen Hauteffloreszenzen, Fieber und entzundlich veranderter Mundschleimhaut in das Bellevue Hospital New York aufgenommen worden waren. Es ist die Beschreibung des einen 8-jahrigen Jungen wiedergegeben.

Die Schleimhaute sind an vielen entzundlichen Erkrankungen beteiligt. Es wird eine Ubersicht uber klinisches Bild, Diagnostik und Therapie der entzundlichen Schleimhauterkrankungen gegeben. Die Diagnostik basiert auf Anamnese, klinischem Bild, Histologie und Serologie. Mangelnde Mundhygiene ist der wichtigste pathogenetische Faktor. Die Therapie ist von der Diagnose, der Ursache und der Ausdehnung der Schleimhautveranderungen abhangig. Geeignet sind sowohl topische als auch systemische immunsuppressive bzw. immunmodulatorische Masnahmen. Die Diagnostik und die Behandlung entzundlicher Schleimhauterkrankungen sind meistens etwas schwieriger durchzufuhren als die der Hauterkrankungen und erfordern haufig eine interdisziplinare Kooperation.

Erythema exsudativum multiforme is an immunologically mediated skin condition caused by viruses, bacteria, food, and drugs. There are different forms, and depending on the severity of the disease there is a major and minor form. Whereas the minor form passes without consequences, the major form and Stevens-Johnson syndrome affect the mucosa and may result in death. The disease affects all age groups but is more often observed in young individuals. Typical signs of the disease are skin lesions termed herpes iris. Taurine is an organic compound used in energy drinks and food that can cause many forms of hypersensitivity reactions, and one of these is erythema exsudativum multiforme. As consumption of energy drinks containing taurine increases, the problem of an increase in cases presenting with various forms of hypersensitivity reactions should be considered. Here we present the case of a 19-year-old man with erythema exsudativum multiforme caused by a drink containing taurine. We excluded all other factors that may have caused erythema multiforme and the patient was hospitalized, having been referred to us for the second time presenting with the same problem caused twice by the same drink.

Hintergrund: Der selektive COX-2-Inhibitor Etoricoxib stellt eine wichtige Alternative fur Patienten mit Analgetikaintoleranz dar. Etoricoxib selbst kann aber auch Ausloser von Unvertraglichkeitsreaktionen sein.

In dit artikel worden enkele huidaandoeningen besproken die relatief weinig voorkomen en, doordat ze zich onder verschillende beelden kunnen presenteren, vaak moeilijk te herkennen zijn: erythema exsudativum multiforme, erythema nodosum, lichen planus en granuloma annulare.

We report two cases of erythema exsudativum multiforme (EEM) that we concluded were caused by infections with Chlamydia pneumoniae. High titers of IgG antibody for Chlamydia pneumoniae were shown in the sera of both cases. One case showed the classical symptoms of pneumonia together with radiological changes in the chest; the other case did not show these symptoms. To the best of our knowledge, only three cases of erythema multiforme associated with Chlamydia pneumoniae infection have been reported.

Stevens-Johnson Syndrome is an adverse drug reaction, considered a severe type of erythema exsudativum multiforme, fatal in about 5%. Characterized by erythema with vesiculobullous lesions in skin and mucous membranes. Implicated drugs are mainly anticonvulsivants and sulphonamides. Prompt recognition and withdrawal of the suspected drug is essential in treatment. Denuded skin areas must be treated with the same principles of burns. Systemic immunosuppression, mainly corticosteroids, remain controversial. We report a case of Stevens-Johnson Syndrome in a 43 year-old male who developed disuria and genital lesions with a detachment of foreskin and glans penis.

In diesem Beitrag werden anhand eines Fallbeispiels die verschiedenen Varianten arzneimittelinduzierter schwerer Hautreaktionen besprochen, zu denen das Erythema exsudativum multiforme majus, das Stevens-Johnson-Syndrom und die toxisch epidermale Nekrolyse gezahlt werden. Alle diese Hauterkrankungen sind gekennzeichnet durch das rasche Auftreten von Erythemen mit einer mehr oder weniger grosflachigen zusatzlichen Blasenbildung. Der Verlauf kann in manchen Fallen fulminant sein, die schnelle Diagnose und der rasche Therapieeinsatz sind von groser Bedeutung. Besondere Aufmerksamkeit wird einer ausgepragten hamorrhagischen Variante und der Assoziation mit der HIV-Infektion geschenkt.

58歳の女性。3年前から両下腿にそう痒を伴った紅色皮疹が出没するようになった。臨床症状および病理組織学的所見より多形滲出性紅斑と診断した。当院歯科口腔外科にて左上顎部第1大臼歯の内歯瘻を指摘され同部の抜歯術を施行されたところ一過性の増悪がみられた後, 両下腿の皮疹は速やかに消退した。抜歯前は末梢血球数, 血沈値, CRP, ASO値などは正常であったが抜歯後, 皮疹の増悪時に一致して一過性に異常値を示した。これらのことより自験例は内歯瘻が誘因となったものと思われた。難治の多形滲出性紅斑の症例では病巣感染がある可能性を考えるべきと思われた。

多型滲出性紅斑の経過中に類白血病反応を認めた症例を報告した。43歳の女性, 感冒様症状より始まり, 大腿に拇指頭大までの紅斑が出現。2日後紅斑は全身に拡大し, 一部に水疱形成がみられた。初診時顔面を除くほぼ全身に滲出傾向を伴った紅斑と一部に緊満性水疱を認めた。血液検査にて, 白血球19,300/mm3(血液像: pro my 2.6%, myelo 2.8%, meta my 4.8%, stab 9.2%, seg 19.8%, eosino 24.8%, lympho 10.2%, mono 10%)を示し, 3日後52,400/mm3となった。骨髄生検標本で異型細胞は認められず。病理組織学的所見は多型滲出性紅斑の像に一致した。類白血病反応を呈した多型滲出性紅斑と診断し, プレドニゾロン(PSL)30mgを投与したところ, 全身状態の改善および白血球数の減少を認めた。

In accordance with earlier investigations of other authors, some diseases of the oral mucosa occur predominantly at appointed seasons of the year. Aphthous ulcers, Herpes simplex labialis, and Erythema exsudativum multiforme show peaks of frequency in March/April and August/September/October, while acute necrotizing ulcerative gingivitis (ANUG) has a large peak of frequency in winter time.

Erythema Exsudativum Multiforme Majus with Predominant Mucous Membrane Involvement (Fuchs' Syndrome) Triggered by Ibuprofen. Fuchs' syndrome is a subtype of erythema exsudativum multiforme majus (EEMM). We report on a 46-year old woman who developed this severe disease pattern following Ibuprofen medication.

The severe skin reactions erythema exsudativum multiforme majus (EEM with mucosal involvement, EEMM), Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN) are difficult to study as they are very rare diseases with an incidence of about two cases per 1 million inhabitants per year. We report on the structure of a registry with the aim of ascertaining all hospitalized cases of EEMM, SJS, and TEN in western Germany and Berlin. The registry is structured as an intensive reporting system, regularly contacting more than 1500 departments including 100% of the burn units (n = 34), departments of pediatrics (n = 241), departments of dermatology (n = 106), and 100% of all internal medicine departments in hospitals with intensive care facilities or with more than 200 beds (n = 1161). With a coverage rate up to 95% based on the number of responding departments between April 1, 1990 and December 31, 1992, from a total of 767 reported cases 353 patients with EEMM, SJS, and TEN were finally included in the registry. Most of these patients were directly reported to the registry; only 2.54% (9 of 353) were primarily registered by the German spontaneous reporting systems. Assuming an average population of 64.5 million for western Germany and Berlin an incidence up to 1.89 per 1 million inhabitants per year could be calculated for SJS and TEN.

Arzneimittel konnen eine Vielfalt von Hautreaktio nen auslosen, die fur den Patienten sehr bedrohlich sein konnen. Neben anaphylaktischen Reaktionen, Purpura, Vaskulitis, Marcumarnekrose, Embolia cutis medicamentosa und verschiedensten exanthematischen Reaktionen sind Erythrodermie und exfoliative Dermatitis, sklerodermieartige Reaktionen sowie das Eosinophilie-Myalgie-Syndrom zu nen nen. Besonders schwer verlaufen die bullosen Haut reaktionen, wie Pemphigus vulgaris (PV), bulloses Pemphigoid (BP), generalized bullous fixed drug eruption (generalisiertes bulloses fixes Arzneiexanthem, GBFDE), Erythema exsudativum multiforme majus (EMMM), Stevens-Johnson-Syndrom (SJS) und toxisch-epidermale Nekrolyse (TEN).

Serious cutaneous adverse reactions such as Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) 1-4 are well known in patients with the human immunodeficiency virus. 1-3 Based on a few case series with a selected population, prevalence data have been calculated as 1.7% (2/115) 2 and 0.8% (12/1500). 1 Due to the short duration of these diseases, we prefered to evaluate the incidence of SJS/TEN in patients with the acquired immunodeficiency syndrome (AIDS), which so far has not been reported. Subjects and Methods. The incidence of SJS/TEN in AIDS patients (Centers for Disease Control [Atlanta, Ga]/World Health Organization [Geneva, Switzerland] classification) was calculated for Germany (excluding the former German Democratic Republic) based on the results of the Dokumentationszentrum schwerer Hautreaktionen , a registry for the severe skin reactions erythema exsudativum multiforme majus, SJS, and TEN 5 that covers more than 90% of all SJS/TEN cases, 5 and the AIDS center of

An account is given of 4 cases of toxic epidermal necrolysis, 3 with lethal course. Three of the cases were of primary type, the first caused by Salazopyrin® in combination with staphylococcal infection, the second possibly by barbiturate (Pentymal), and the third by multiple drug provocation, sulphamethoxine (Bayrena®) probably being the decisive factor in the last resort. The salazopyrin patient survived but incurred a severe visual defect. The 2 of these cases with lethal course exhibited toxic injuries in the myocardium, liver and kidneys, in one case leading to uraemia, and both developed pulmonary oedema. The fourth case, also with fatal outcome, was not caused by a drug. It started as an ordinary mucocutaneous syndrome with eruption of erythema exsudativum multiforme type. The syndrome later developed into epidermolysis in connection with secondary staphylococcal infection.Toxic epidermal necrolysis may possibly be classified under the febrile mucocutaneous syndrome, but it has such charact...

Circulating lymphocytes of patients with recurrent herpetic, aphthous and other oral mucosal diseases, as well as of persons with intact oral mucosa were examined with direct immunofluorescence method using HVH and adenovirus hyperimmune conjugated sera. Some of the circulating lymphocytes showed a specific fluorescence both in patients and in persons with intact oral mucosa. Increased herpes-positivity was observed only in erythema exsudativum multiforme and increased adeno-positivity in patients with recurrent aphthous stomatitis, especially in cases with constantly recurring aphthae.

Serum protein fractions of patients with Behcet's syndrome (incomplete type, 3 males and 3 females), recurrent aphthous ulcer (4 males and 2 females), simple aphtha (1 male and 2 females) and erythema exsudativum multiforme (EEM) (1 male and 3 females) were analyzed quantitatively by electrophoresis on cellulose acetate membrane and qualitatively by immuno-electrophoresis on agar gel plate' γ-Globulin and α2-globulin were incressed in Behcet's syndrome. γ-Globulin was also increased significantly in recurrent aphthous ulcer and EEM.By immuno-electrophoretic analysis the precipitation lines of haptoglobin (4 in 6 patients), ceruloplasmin (5), transferrin (4), β1c-globulin (3), IgA (6) and IgG (5) were increased in Behcet's syndrome. In recurrent aphthous ulcer the similar tendency was noticed. In EEM ceruloplasmin, haptoglobin, β1c-globulin, IgA and IgG were increased. Increase in these fractions suggests that some immunolgic mechanism may be involved in the etiology of the diseases.

Ferdinand von Hebra first described a typical skin lesion with aggressive and tissue destructive characteristics in 1845 [1], which — due to its appearance — was named by Pierre Louis Cazanave “lupus [latin wolf] erythematosus” in 1851 [2]. Although the term “lupus” was not confined to a single disorder at that time [3], the peculiarity of the skin lesion was fully recognized, resulting in a variety of descriptive terms (e.g., “butterfly rash” [4]) which are in use until today. It was, however, not until Moritz Kaposi succeeded Hebra to the Chair of the Department of Dermatology at the Vienna University Hospital that the systemic nature of lupus was recognized: Kaposi reported in 1872 that certain patients suffering from lupus presented with a syndrome consisting of fever. arthritis, lymphadenopathy, and anemia [5]. It was this report which led to a clear separation of systemic or disseminated lupus erythemntosus (SLE) from the forms definitely limited to the skin. Publications by Jadassohn from Vienna [6] and by William Osler from Baltimore, Maryland, who discussed visceral complications of “erythema exsudativum multiforme” [7] — which rather would be recognized as SLE today [8] — gave additional evidence for the disseminated nature of the disease, while reports on idiopathic thrombotic thrombocytopenic purpura [9], endocarditis [10], and changes in renal histology [11], culminating in the classic study by Klemperer, Pollack, and Baehr on the pathomorphology of SLE in different organs [12], illustrated its puzzling variety of clinical manifestations.

Die auf Hebra zuruckgehende Bezeichnung Erythema exsudativum multiforme ist heute nur noch im deutschsprachigen Raum gangig. Im internationalen Schrifttum wird das klinisch wohl zu definierende, atiologisch nur teilweise geklarte Krankheitsbild als Erythema multiforme bezeichnet. Es mus hier nicht betont werden, das nicht jedes polymorphe Erythem ein Erythema exsudativum multiforme ist.

Die geschichtliche Entwicklung des Erythembegriffes tritt erst mit Ferdinand Hebra in eine Phase ein, die unser Interesse in groserem Mase verdient. Ich halte es daher fur uberflussig, auf die fruheren Epochen einzugehen. In der Literatur finden sich ausgezeichnete historische Darstellungen. Ich verweise z. B. auf Lewin, der mit kaum zu ubertreffender Grundlichkeit den vielfach verschlungenen Pfaden bis zur altesten Zeit nachgegangen ist.