Severe Erythema Multiforme Research Articles

Guidelines for the management of drug-induced hypersensitivity syndrome 2023.

Drug-induced hypersensitivity syndrome (DIHS) is one of the severe drug eruptions accompanied by fever and multiple organ dysfunction, and it is induced by a relatively limited range of drugs, including anticonvulsants. A distinctive feature of this condition is its association with the reactivation of herpes viruses, particularly human herpesvirus 6. The pathogenesis involves two key factors: drug allergy and herpesvirus reactivation. DIHS is often challenging to diagnose in its early stages, and its clinical course varies widely, ranging from relatively mild to life-threatening cases. Additionally, unexpected complications, such as autoimmune diseases, may occur during the convalescent phase. As a result, diagnosing, treating, and predicting the prognosis of DIHS remain complex issues. In response to these challenges, the Ministry of Health, Labour and Welfare Study Group on Severe Erythema Multiforme has taken the lead in developing new guidelines for the management of DIHS. These guidelines aim to support clinical practice by providing up-to-date, evidence-based information on the diagnosis, treatment, and prognosis of DIHS.

Prevention of recurrent erythema multiforme with lenalidomide: a case series.

Prevention of recurrences of erythema multiforme is a therapeutic challenge. Thalidomide is effective, but its use is limited by adverse effects, especially peripheral neuropathy. We report seven cases of recurrent severe erythema multiforme treated with lenalidomide. In six cases, the drug was effective without significant adverse effects.

Erythema Multiforme after Unilateral Adrenal Adenoma Resection in a Patient with Cushing’s Syndrome

Introduction: Cushing’s syndrome is characterized by endogenously increased production of glucocorticoids. It has been postulated that excessive immune response may occur after an immediate withdrawal of high glucocorticoids after surgical therapy of Cushing’s syndrome. We reported a rare case of severe erythema multiforme after unilateral adrenal adenoma surgery for Cushing’s syndrome.

Abstract P-105: A COMPARATIVE STUDY ON CHILDREN WITH ERYTHEMA MULTIFORME MAJUS SYNDROME

Aims & Objectives: To compare the effect of blood purification therapy with steroid-immunoglobulin therapy in children with severe erythema multiforme majus. Methods The clinical data of 30 children admitted between 2010 and 2016 were retrospectively reviewed. These children were separated into two groups according to the therapeutic method. Results No death occurred in two groups. The results showed that the duration of fever and majus was shorter in blood-purification group comparing with steroid-immunoglobulin group, as well as the duration of mucosal lesions. Conclusions The blood purification therapy is more effective for children with severe erythema multiforme majus. It can decline the mortality.

Successful drug desensitization in patients with delayed-type allergic reactions to anti-tuberculosis drugs

ObjectiveTo evaluate the outcomes of anti-tuberculosis drug desensitization. MethodsThis was a retrospective study. Inclusion criteria were as follows: age >18years, documented tuberculosis infection, a previous cutaneous allergic reaction to anti-tuberculosis drugs, and having undergone drug desensitization between January 2003 and March 2014. The definition of allergic reaction to anti-tuberculosis drugs included (1) a temporal relationship between drug use and the allergic reaction; (2) improvement in the allergic reaction after drug withdrawal; (3) recurrence of the allergic reaction after reintroduction of only the offending drug; and (4) absence of other causes. ResultsA total of 19 desensitization procedures were performed. The drugs used for these procedures were isoniazid (n=7), rifampicin (n=6), or ethambutol (n=6). Of note, severe allergic reactions (Stevens–Johnson syndrome (n=4), erythema multiforme (n=3), and drug rash with eosinophilia and systemic syndrome (n=1)) were included. All patients underwent resolution of the previous allergic reactions before desensitization. The median duration of desensitization was 18 days. The success rate was 78.9%. The allergic reactions following failed desensitization were not severe; most were maculopapular rashes. ConclusionsThe desensitization protocol for anti-tuberculosis drugs was associated with a high success rate, and the individuals who failed desensitization experienced mild allergic reactions.

Abstract: In a Little While: Anticipating Changes in Bundled Payments for the Treatment of Patients with Acute, Life-threatening Dermatologic Emergencies, Through Prevention of Healthcare Associated Infections.

INTRODUCTION: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are part of the same clinical syndrome that represents a medication-induced desquamation disorder. In 1922 Drs. Stevens and Johnson first described SJS as an acute mucocutaneous syndrome presenting in two young boys. 1, 2 Patients admitted to a burn center suffering from the Stevens-Johnson syndrome to Toxic Epidermal Necrolysis (SJS-TEN) spectrum are typically considered to have high hospital morbidity and mortality. Little is known about patients admitted to a burn center suffering from non-bullous Skin Disorders (SD). This group includes severe rashes, non-healing wounds, erythema multiforme, and unknown skin lesions requiring hospitalizations. We compared these two group’s costs, mortality, and the effect of Healthcare-Associated Infections (HAI) on outcomes to better define this patient population.

Case Report 2. Severe erythema multiforme induced by two consecutive anti-angiogenic drugs

Case Report 2. Severe erythema multiforme induced by two consecutive anti-angiogenic drugs

Enzyme-linked immunosorbent assay for detection of peptide-specific human antidesmoplakin autoantibodies

Autoantibodies directed against desmoplakin (Dp) I and II have recently been characterized in a subset of patients with severe erythema multiforme (EM), a recurrent inflammatory skin disease with a broad spectrum of clinical manifestations. These autoantibodies recognize a peptide epitope localized within the extreme end of the carboxy terminal domain of Dp responsible for the assembly of keratin filaments to the desmosomal plaque. Using dot blot analysis with overlapping synthetic peptides, the binding epitope YSYSYS has been identified. To establish an enzyme-linked immunosorbent assay (ELISA) for detection of peptide-specific anti-Dp autoantibodies in sera of patients with EM. A synthetic peptide containing the respective amino acid sequence was used as matrix for ELISA plates. Serum samples from patients with known EM and peptide-specific anti-Dp autoantibodies verified by immunoblotting, immunoprecipitation and epitope mapping were used. Establishing an index value of 42.0, 25 of 25 serum samples from five patients with peptide-specific anti-Dp autoantibodies were positive in the ELISA. From control sera, none of 31 bullous disease sera and only one (1.2%) of 83 normal human sera were positive. These data show that the ELISA presented in this study represents a sensitive and highly specific tool for the detection of peptide-specific anti-Dp autoantibodies in patients with EM.

Systemic immunosuppressant therapy in childhood

The indications for systemic immunosuppressant therapy are much the same in children as in adults. Perhaps the most important difference is the need in a child to consider more carefully the patient's likely future therapy requirements. This need reflects a justifiable anxiety concerning the longer-term toxicity associated with some of these drugs. It is obvious that special attention should be paid to the dosage regimens that are appropriate in children. However, otherwise the principles of treatment are essentially the same as in adults. This talk will focus on the use of azathioprine in atopic eczema, methotrexate in psoriasis and linear morphoea, and intravenous methylprednisolone in severe muco-cutancous erythema multiforme and toxic epidermal necrolysis. The value of azathioprine as a treatment for severe childhood eczema was greatly increased by the elucidation of the metabolic pathways for this drug, and by the development of an assay for thiopurine methyl transferase to allow detection of those at greatest risk of myelosuppression. We now treat children with normal TPMT levels with 3 mg/kg per day with gratifying therapeutic response and limited requirement for monitoring of blood counts and liver function. More recently we have successfully treated TPMTHL heterozygotes with doses of around 1.5 mg/kg per day. We now consider azathioprine as superior to cyclosporin as a systemic therapy for atopic eczema. The value of methotrexate in adults with plaque psoriasis and generalized pustular psoriasis is well established. It is equally useful in children with these disorders, and the most appropriate dosage appears to be in the region of 0.3–0.4 mg/kg as a single weekly dose. Children generally tolerate oral therapy well. Methotrexate also appears helpful in arresting the progression of linear morphoea, both in the case of coup de sabre lesions and progressive hemi-facial atrophy, and in limb lesions that are interfering with joint mobility or are causing profound lipoatrophy. Intravenous methylprednisolone appears to be of value in several acute dermatoses in childhood, but is most commonly used at Great Ormond Street Hospital in the hope of arresting progression of severe muco-cutaneous erythema multiforme and toxic epidermal necrolysis. Various dose regimens are used in children, but in our unit we use a dose of 20–30 mg/kg per day, up to a maximum of 500 mg, for 3 successive days. Each dose is given over period of 2 h with frequent monitoring of vital signs, particularly blood pressure.

Erythema multiforme in dogs and cats: literature review and case material from the Cornell University College of Veterinary Medicine (1988-96).

A review of case material from the Cornell University College of Veterinary Medicine from 1988 to 1996 identified 20 dogs and one cat with definitive or presumed erythema multiforme. An additional 24 dogs and five cats with definitive or presumed erythema multiforme were found in the veterinary literature. Erythema multiforme accounted for only 0.40% and 0.11%, respectively, of all the canine and feline dermatology cases examined over a 9-year period. German Shepherd dogs and Pembroke Welsh Corgis appeared predisposed. The condition was manifested as a vesiculobullous and/or ulcerative dermatosis in the majority of dogs and cats. In dogs, the most commonly affected body sites included the ventrum (especially axillae and groin), mucocutaneous junctions, oral cavity, pinnae, and footpads. Histopathological findings in cutaneous and mucocutaneous biopsy specimens were consistent with previously published criteria. In dogs, erythema multiforme occurred more frequently in patients receiving drug therapy. In cats, all cases of erythema multiforme were presumed to be drug related. Elimination of the associated trigger factor and supportive care usually resulted in resolution of the erythema multiforme within 1-2 weeks. Four dogs with severe idiopathic erythema multiforme were successfully managed with glucocorticoids or azathioprine.

Erythema Multiforme and Stevens - Johnson Syndrome Are Histologically Distinct

Recently, it has been suggested that severe erythema multiforme (EM) and Stevens - Johnson syndrome (SJS) are two distinct entities. That conclusion was based on a study of clinical and epidemiologic data. To determine if the two diseases differ histologically, the authors of the present study retrospectively …

The spectrum of Stevens-Johnson syndrome and toxic epidermal necrolysis: a clinical classification.

The nosology of severe bullous erythema multiforme (EM), Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN) remains controversial. To conduct a prospective case-control study of the etiologic factors of these diseases, we needed to define criteria for classifying the cases. After having reviewed photographs of the skin lesions of more than 200 patients, an international group of dermatologists proposed a classification based on the pattern of "EM-like lesions" (categorized as typical targets, raised or flat atypical targets, and purpuric macules) and on the extent of epidermal detachment. The "consensus" classification in five categories was as follows: bullous erythema multiforme, detachment below 10% of the body surface area (BSA) plus localized typical targets or raised atypical targets; SJS, detachment below 10% of the BSA plus widespread erythematous or purpuric macules or flat atypical targets; overlap SJSTEN, detachment between 10% and 30% of the BSA plus widespread purpuric macules or flat atypical targets; TEN with spots, detachment above 30% of the BSA plus wide-spread purpuric macules or flat atypical targets; TEN without spots, detachment above 10% of the BSA with large epidermal sheets and without any purpuric macules or target. Whether all five categories proposed represent distinct etiopathologic entities will require further epidemiologic and laboratory investigations.

Clinical Classification of Cases of Toxic Epidermal Necrolysis, Stevens-Johnson Syndrome, and Erythema Multiforme

To conduct a prospective case-control study about causative factors of severe bullous erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis, we needed to define criteria for classifying the cases and standardize the collection of data so that cases could be reliably diagnosed according to this classification. Based on review of case histories and photographs of patients, a group of experts proposed a classification based on the pattern of erythema multiforme-like lesions (categorized as typical targets, raised or flat atypical targets, and purpuric macules) and on the extent of epidermal detachment. An atlas illustrating this classification that included photographs and schematic drawings was developed. We compared the evaluations of 28 cases by four nonphysicians relying on the atlas with the evaluations of the same cases by five experts not using the atlas to determine the usefulness of this atlas for classifying cases according to our nosologic schema. The following consensus classification in five categories was proposed: bullous erythema multiforme, detachment below 10% of the body surface area plus localized "typical targets" or "raised atypical targets"; Stevens-Johnson syndrome, detachment below 10% of the body surface area plus widespread erythematous or purpuric macules or flat atypical targets; overlap Stevens-Johnson syndrome-toxic epidermal necrolysis, detachment between 10% and 30% of the body surface area plus widespread purpuric macules or flat atypical targets; toxic epidermal necrolysis with spots, detachment above 30% of the body surface area plus widespread purpuric macules or flat atypical targets; and toxic epidermal necrolysis without spots, detachment above 10% of the body surface area with large epidermal sheets and without any purpuric macule or target. Using the atlas, the nonexperts showed excellent agreement with the experts. This study suggests that an illustrated atlas is a useful tool for standardizing the diagnosis of acute severe bullous disorders that are attributed to drugs or infectious agents. Whether the five categories proposed represent distinct etiopathologic entities will require further epidemiologic and laboratory investigations.

Two Cases of Transfusion-Associated Graft-vs-Host Disease After Open Heart Surgery

Some cases of blood transfusion-associated (TA) graft-vs-host disease (GVHD) in immunocompetent patients have been reported, but those dermatologic findings were not precisely mentioned. We describe patients with clinicopathologically TA-GVHD and compare TA-GVHD and acute GVHD after bone marrow transplantation. Two cases of TA-GVHD after open heart surgery are reported. In both immunocompetent patients, severe erythema multiformelike skin rash developed over the entire body, followed by fever, diarrhea, jaundice, transaminitis, pancytopenia, and marrow alpasia approximately 10 days after operation. The rash in one patient changed from erythema multiformelike to toxic epidermal necrolysis at death. Skin biopsy specimens revealed eosinophilic bodies, basal vacuolation, and exocytosis in the epidermis. Eosinophilic bodies tend to appear in the upper epidermis. Immunohistochemistry studies revealed that infiltrating cells were CD4 and CD8. While acute GVHD in immunosuppressed patients who have undergone bone marrow transplantations often shows lichenoid histologic features, TA-GVHD in our patients who were immunocompetent may resemble severe erythema multiforme or toxic epidermal necrolysis. The difference in TA-GVHD may be related to lack of host modification by immunosuppression. Irradiation of the blood products should be required in open heart surgery, for TA-GVHD in immunocompetent patients is almost always fatal.

Erythema multiforme major in a 2-month-old child with human immunodeficiency virus (HIV) infection

A case is presented of severe erythema multiforme occurring in a 2-month-old boy from Zimbabwe which was probably triggered by phenobarbitone. He later developed the acquired immunodeficiency syndrome and serological tests suggested that it was a congenital HIV-I infection.

Cyclosporine Therapy for Bullous Erythema Multiforme

To the Editor.— Immunosuppressive therapy with cyclosporine introduced in the 1970s dramatically improved the survival of kidney, liver, and heart transplants. The reported use of cyclosporine for treatment of dermatological disease is increasing in frequency, and favorable responses have been observed in the treatment of psoriasis, Behcet's disease, bullous pemphigoid, pemphigus vulgaris, dermatomyositis, polymyositis, ichthyosis, and several other cutaneous disorders.1We report on the successful treatment of a patient with severe bullous erythema multiforme that had previously responded only partially to high dosages of systemic corticosteroids. Report of a Case.— A 23-year-old white woman was referred to our dermatology service in August 1987 for evaluation of a bullous eruption of 3 years' duration. The patient was in good health until 1984 when, approximately 1 week after a dental procedure, she developed a generalized eruption and oral ulcerations. The initial eruption cleared after an intramuscular dose of penicillin. Four

Two cases of Stevens-Johnson syndrome

Stevens-Johnson syndrome is a severe erythema multiforme exudative with simultaneous lesions of the mucous membranes of natural orifices. Along with changes in the skin and mucous membranes, internal organs are also affected. The etiology of the disease is not fully elucidated. In recent years, there is more and more data for hyperergic form of drug dermatitis. In this regard, two cases of Stevens-Johnson syndrome are of interest.

A Case of Toxic Epidermal Necrolysis

Toxic epidermal necrolysis is a reactive erythema of nonstaphylococcal origin characterized by a scalded appearance of the skin. The TEN is widely regarded as a variant of severe erythema multiforme because of its acute course, its freguent common cause, its freguent overlap with Stevens-Johnson disease, and its histologic identity. I present a case of TEN with severe mucosal involvement resembled Stevens-Johnson disease.

Clinical and immunologic responses to levamisole in 13 patients with erythema multiforme

Thirteen patients with severe oral erythema multiforme (EM) were treated with levamisole. Apparent clinical benefit consisting of a marked decrease in the severity and frequency of attacks was observed in 8 of these patients. Immunologic parameters, including skin test reactivity, lymphocyte stimulation by antigen and PHA, and rosette forming cells did not change in any consistent fashion following therapy. The immunologic responses did not correlate with clinical results. From this open trial with levamisole we concluded: levamisole may offer substantial clinical benefit to some patients with EM; levamisole may be associated with less serious side effects than prednisone; and further studies of the efficacy of levamisole in this disease are warranted.

Severe erythema multiforme complicated by fatal gastrointestinal involvement following co-trimoxazole therapy

A patient is described with erythema multiforme who developed features of an acute abdominal condition necessitating laparotomy in which post-operative complications led to death. Gastric and intestinal ecchymoses were found at autopsy. The precipitating drug was considered to be co-trimoxazole.