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[Erythema multiforme. A heterogeneous pathologic phenotype]
M Carrozzo1, M Togliatto, S Gandolfo
1Dipartimento di Fisiopatologia Clinica, Università degli Studi, Torino. Mcarro@tin.it
Abstract:
The term Erythema Multiforme (EM) include actually a wide range of clinical expressions, from exclusive oral erosions (Oral EM) to mucocutaneous lesions (EM Minor), sometimes with severe involvement of multiple mucosal membrane (EM major, Stevens-Johnson syndrome [SJS]) or with involvement of a large area of the total body surface (toxic epidermal necrolysis [TEN]). However, this terminology is not worldwide accepted and often the various clinical categories show some overlapping features. Among the great number of suspected etiological factors, herpes simplex virus is involved in many cases of EM minor whereas SJS and TEN are caused in 80% of cases by systemic drugs, mainly by anticonvulsivants, sulfonamides, nonsteroidal anti-inflammatory drugs and antibiotics. Several oral EM seem idiopathic, but data on this topic are very few. There is no specific or consistent microscopic and immunopathologic pattern of EM and the diagnosis should be done by excluding other similar diseases. The treatment include the use of antivirals for EM minor, mainly if recurrent, and of immunosuppressants (especially systemic corticosteroids) for SJS. TEN patients require adequate supportive care and often they have to be treated in emergency departments. Finally, patients with exclusive oral lesions may be treated with both topical and systemical corticosteroids.
Insights
Erythema Multiforme (EM) encompasses a spectrum of conditions, from oral lesions to severe mucocutaneous reactions like Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). Diagnosis relies on exclusion, with varied treatments based on the specific presentation and cause.
Area of Science:
- Dermatology and Immunology
- Clinical Medicine
Context:
- Erythema Multiforme (EM) presents a spectrum of clinical manifestations, from isolated oral erosions (Oral EM) to widespread mucocutaneous lesions (EM Minor, EM Major, Stevens-Johnson syndrome [SJS], and toxic epidermal necrolysis [TEN]).
- Current terminology is not universally standardized, leading to overlapping features among different clinical categories.
- Etiological factors vary, with herpes simplex virus implicated in EM Minor and systemic drugs (anticonvulsants, sulfonamides, NSAIDs, antibiotics) causing most SJS/TEN cases; some oral EM cases appear idiopathic.
Purpose:
- To review the clinical spectrum, etiological factors, diagnostic challenges, and treatment strategies for Erythema Multiforme and its severe variants.
- To highlight the diagnostic difficulties due to the lack of specific histopathological and immunopathological markers.
- To differentiate treatment approaches based on the severity and presumed cause of the EM spectrum.
Summary:
- EM encompasses a range of conditions from mild oral lesions to severe, life-threatening mucocutaneous reactions (SJS/TEN).
- Herpes simplex virus is a common trigger for EM Minor, while systemic drugs are the primary cause of SJS/TEN.
- Diagnosis is often made by excluding other conditions, and treatment varies from antivirals for EM Minor to immunosuppressants for SJS and supportive care for TEN.
Impact:
- Clarifies the complex classification and varying etiologies within the Erythema Multiforme spectrum.
- Emphasizes the importance of differential diagnosis in managing EM and its severe forms.
- Provides a concise overview of current treatment modalities tailored to specific EM presentations, guiding clinical management.