Key point P.3 Flashcards
Erythema Multiforme:
Definition
Erythema multiforme (EM) is an acute, immune-mediated condition characterized by the appearance of distinctive target-like lesions on the skin
Erythema Multiforme:
Characteristics
Types
These lesions are often accompanied by erosions or bullae involving the oral, genital, and/or ocular mucosa
- Erythema multiforme major is the term used to describe EM with severe mucosal involvement (and may have associated systemic symptoms, such as fever and arthralgias)
- Erythema multiforme minor refers to EM without (or with only mild) mucosal disease (and without associated systemic symptoms)
Erythema Multiforme:
Etiology
Infections, medications, malignancy, autoimmune disease, immunizations, radiation, sarcoidosis, and menstruation, have been linked to the development of EM
- Infections (viral, bacterial, or fungal) account for approximately 90%, with HSV as the most commonly identified precipitant
- Drugs induce EM in less than 10% of cases
- The most common precipitators appear to be NSAIDs**, **sulfonamides**, **antiepileptics**, and **antibiotics
Erythema Multiforme:
Clinical Features
Cutaneous
- Target lesions are the hallmark of the disorder
- Initial lesions may begin as round, erythematous papules that evolve into classic target lesions
- Typical target lesions consist of three components: a dusky, central area or blister, a dark red inflammatory zone surrounded by a pale ring of edema, and an erythematous halo on the extreme periphery of the lesion
- Are generally less than 3 cm in diameter
- Atypical target lesions may also occur
- These manifest as raised, edematous, palpable lesions with only two zones of color change and/or a poorly defined border
Erythema Multiforme
Clinical Features
Mucosal
- Lesions can involve the oral, ocular, and/or genital mucosa and commonly manifest as diffuse areas of mucosal erythema, painful erosions, and/or bullae
- Lesions tend to affect the vermilion lip** and **mucosal surfaces, including the buccal mucosa, labial mucosa, non attached gingiva, and tongue. Rarely, involvement can extend to the pharynx and upper respiratory tract
Erythema Multiforme
Differential Diagnosis
- Includes: pemphigus vulgaris, paraneoplastic pemphigus, mucous membrane pemphigoid, oral lichen planus-> present with oral erosions, Complex aphthosis
- In contrast to these chronic conditions, the course of EM is typically self- limited
- However, the possibility of these other disorders should be considered in patients with frequently recurring disease
- Biopsies** with **direct immunofluorescence studies are useful for establishing the diagnosis
Erythema Multiforme
Decision to hospitalize:
- Most patients with EM can be managed in the outpatient setting. However, severe mucous membrane involvement that prevents sufficient oral intake may require hospitalization for nutrition and pain control
- The suggested approach to an acute episode of EM associated with infection consists of appropriate treatment of the infection. For herpes simplex virus (HSV)-associated EM, this typically does not involve antiviral treatment because EM typically develops several days or more after the onset of clinical signs of HSV infection, when treatment for HSV infection is no longer indicated
- If the onset of EM is associated with drug exposure, it is generally accepted that the causative drug should be promptly discontinued, if feasible
Treatment of specific sites—Interventions to improve symptoms of EM are based upon the clinical presentation
Erythema Multiforme
Cutaneous involvement
Topical corticosteroids** can be helpful for alleviating pruritus and skin discomfort. **Oral antihistamines (eg, hydroxyzine) may also be helpful for pruritus
Erythema Multiforme
Nondisabling oral involvement
- For patients with limited oral mucosal involvement that is not disabling, management is focused on symptomatic relief. Painful oral erosions can be treated with a high-potency (eg, groups 1 or 2) topical corticosteroid gel and mouthwashes that contain a mixture of lidocaine, diphenhydramine, and antacids
- We typically use fluocinonide 0.05% gel applied two to three times per day and a mouthwash containing equal parts of viscous lidocaine 2%, diphenhydramine (12.5 mg/5 mL), and an aluminum hydroxide and magnesium hydroxide mixture (eg, Maalox) as a swish-and-spit, as needed, up to four times per day
Erythema Multiforme:
Disabling oral involvement
Extensive oral mucosal involvement may result in severe pain, leading to an inability to ingest foods or liquids. In addition to the comfort measures used for nondisabling oral involvement, systemic glucocorticoids are often prescribed in an attempt to decrease the severity of symptoms and to shorten the course of the disease
- 40 to 60 mg per day of prednisone or its equivalent tapered over two to four weeks
Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN):
Definition
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe mucocutaneous reactions, most commonly triggered by medications, characterized by extensive necrosis and detachment of the epidermis
Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN):
Signs and symptoms that should alert the clinician to the possibility of SJS/TEN include:
- fever >38°C
- mucositis
- skin tenderness
- blistering
Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN):
Mucosal lesion
Mucosal involvement occurs in approximately 90% of cases of SJS/TEN and can precede or follow the skin eruption. Painful crusts and erosions may occur on any mucosal surface
Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN):
Management:
The main principles of supportive care are the same as for major burns and include wound care, fluid and electrolyte management, nutritional support, temperature management, pain control, and monitoring or treatment of superinfections
- Systemic corticosteroids — The use of systemic corticosteroids in patients remains controversial
- Intravenous immune globulin** — Data are limited and conflicting **Intravenous immune globulin plus systemic corticosteroids — the data are too limited to draw any firm conclusions
- Cyclosporine — cyclosporine given at the dose of 3 to 5 mg/kg per day may slow the progression of SJS/TEN, in the absence of significant toxicity
- Tumor necrosis factor inhibitors — A single infusion of 5 mg/kg of the TNF- alpha inhibitor infliximab halted the progression of skin detachment and induced a rapid re-epithelization of the denuded skin
Anemia
Classification of anemias by morphology:
Anemia
Anemia, regardless of etiology, can result in clinically significant oral manifestations including:
- opportunistic infection
- glossodynia
- delayed wound healing
- angular cheilitis
- oral mucosal pallor
- atrophic glossitis
- ulcers
- decreased mandibular trabeculation
Aplastic anemia
Definition
Is a hematologic disorder that is characterized by failure of the hematopoietic precursor cells in the bone marrow to produce adequate numbers of all types of blood cells (pancytopenia)
- Represent an immune-mediated disease caused by cytotoxic T lymphocytes that target differentiating hematopoietic cells in the marrow
Aplastic anemia
Oral Findings
- Risk for clinical appearance of oral bleeding due to decreased baseline circulating platelet counts are <25,000/mm3
- Gingival hemorrhage, oral mucosal petechiae, purpura, ecchymoses, and spontaneous oozing of blood from the gingiva and periodontium
- Oral mucosa may appear pale
- Oral ulcerations associated. Minimal erythema is usually associated with the periphery of the ulcers.
- Gingival hyperplasia
