Dermatologic Emergencies CME Part I: Inflammatory disorders, angioedema, and anaphylaxis
Department of Dermatology, Basildon University Hospital NHS Foundation Trust, UK
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Early detection of erythroderma is essential in clinical practice since the related systemic implications might be fatal. it is characterized by widespread redness with a highly variable degree of scaling. Most of cases have an underlying skin problem or a systemic disorder that is known to be related to erythroderma. idiopathic erythroderma occurs in about 30% of cases. Generalized pustular psoriasis is a rare and life-threatening variant of psoriasis manifests with successive crops of pustules on a background of red and tender skin that covers most of the body. Erythroderma and generalized pustular psoriasis are typically managed with hospitalization, supportive measures, and specialist care.
Key words: Erythroderma; Anaphylaxis; Angioedema; Skin failure; Generalized pustular psoriasis
WHAT IS ALREADY KNOWN ABOUT THIS TOPIC?
Various primary dermatologic conditions can present in a life-threatening manner, with high mortality and morbidity rates without adequate early intervention.
WHAT DOES THIS REVIEW ADD?
Participants should be able to identify the distinguishing features of the main dermatologic emergencies, the common pitfalls in making their diagnosis, and the appropriate early intervention strategies.
Variety of primary dermatological conditions can present as an emergency, in some cases due to primary skin failure, and in others, dermatological disease may imply secondary life-threatening internal organ complications. Acute skin failure (ASF) is defined as the loss of normal temperature control with an inability to maintain the core body temperature and failure to prevent percutaneous loss of fluid, electrolytes, and protein, resulting in the imbalance and failure of the mechanical barrier to prevent penetration of foreign materials . It represents a dermatological emergency requiring urgent care through a multidisciplinary approach comparable to other organ failures such as heart, respiratory and renal failure. To avoid adverse outcome, prompt identification and appropriate management of dermatologic emergencies are crucial.
The first section of this CME series reviews cutaneous inflammatory disorders, angioedema, and anaphylaxis
INFLAMMATORY CUTANEOUS DISORDERS
- Erythroderma (Exfoliative dermatitis)
Erythroderma is a potentially life-threatening condition characterized by diffuse erythema and scaling that involves at least 90% of the body surface area. Clinical diagnosis of erythroderma can be problematic, as it constitutes the final clinical endpoint of a broad spectrum of skin and systemic diseases (Table 1). Other manifestations of erythroderma include severe pruritus, diffuse alopecia, palmoplantar keratoderma, nail dystrophy, ectropion, lymphadenopathy, tachycardia, and peripheral edema [2,3]. Major systemic complications include loss of fluid and electrolytes, hypoalbuminemia, thermoregulatory disturbance, capillary leak syndrome, heart failure, infections, and even death .
Defining the underlying cause of erythroderma represents a challenge and must include a thorough history, complete clinical examination, and profound clinicopathological correlation with or without immunohistochemistry. Despite a comprehensive evaluation, the cause may remain unrecognized (idiopathic erythroderma) in approximately 25%-30% of cases [5,6].
The treatment approach should include discontinuing all non-essential medications and adequate workup to address the underlying causes combined with supportive measures (Fig. 1). Sedating antihistamines may be used to ease severe pruritus, and systemic corticosteroids may be necessary for severe idiopathic and drug-related erythroderma . Cyclosporine, acitretin, methotrexate, and biologics are among the most commonly used drugs for the treatment of erythroderma [8,9].
|Figure 1: Erythroderma. Approach to patients with erythroderma includes identifying and removing the possible underlying triggers and supportive care [14,15].|
- Acute generalized pustular psoriasis (AGPP)
While psoriasis is rarely seen as a life-threatening condition, emergency medical care is required in patients with erythrodermic and acute generalized pustular psoriasis .
AGPP (also known as von Zumbusch psoriasis) is characterized by abrupt onset, widespread, painful erythema studded with extensive sterile pinpoint-sized pustules, particularly flexures. The pustules often coalesce and expand to form lakes of pus, and subungual pus collection may lead to nail avulsion. AGGP is often accompanied by prominent systemic symptoms such as fever, malaise, and polyarthralgia. Potential life-threatening complications may occur during the course of AGPP, including neutrophilic cholangitis, interstitial pneumonitis, acute renal failure, sepsis and death [17,18].
Diagnostic criteria have been proposed including recurrent episodes of typical cutaneous eruption accompanied by systemic manifestations, laboratory abnormalities (leukocytosis, elevated erythrocyte sedimentation rate or C-reactive protein, hypoalbuminemia, etc.), and findings of Kogoj spongiform pustules on histopathological examination .
Hospital admission and supportive treatment are necessary for severe cases. The first-line treatments include acitretin, cyclosporin A, methotrexate, or infliximab. Other therapeutic options include other biologics such as etanercept or adalimumab, PUVA, and combination therapy (Fig. 2) [23,24]. A monoclonal antibody against the interleukin-36 receptor shows promising results in phase 2 trials irrespective of the IL36RN mutation .
ANGIOEDEMA, ANAPHYLAXIS AND RELATED DISORDERS
Angioedema is a common presentation in the ED, and it could be fatal when involving the airway; hence prompt diagnosis and proper management are vital. Angioedema is characterized by the abrupt onset of non-pitting and non-pruritic swelling that involves the reticular dermis and subcutaneous and submucosal layers . Lesions are typically well-defined, asymmetric in distribution, and are located in non-dependent areas. Common locations include the face, especially the lips and periorbital area; the extremities; genitalia; and abdominal viscera. In contrast to urticaria, angioedema typically lasts from 24 to 96 h .
Histamine mediates the most common pathophysiologic mechanism of angioedema in ED (allergic or histaminergic angioedema); however, physicians should be alert to the less common forms mediated by bradykinin (non-allergic or non-histaminergic angioedema) that do not respond to the usual treatment of histaminergic angioedema. Therefore, identifying a particular type of angioedema is crucial for proper treatment  (Table 2).
Angioedema is a clinical diagnosis based on suggestive history and physical examination. There is no laboratory test to differentiate histamine from bradykinin-mediated angioedema. Laboratory tests may help identify the underlying triggering allergens, infections, or systemic diseases. Measurement of C1 inhibitor, C4, and C1q levels may help in the diagnosis of hereditary and acquired angioedema associated with C1 inhibitor abnormalities [29,30] (Fig. 3).
Assessment and securing airway patency are essential for patients presenting with angioedema in or near the airway. Patients with angioedema associated with anaphylaxis should be treated with intramuscular adrenaline, fluids, oxygen, and other supportive measures .
Antihistamines and corticosteroids are the main therapeutic agents for less severe mast cell-mediated angioedema . The treatment options for acute attacks of hereditary angioedema include C1 inhibitor concentrate, ecallantide, icatibant, and fresh frozen plasma if the aforementioned treatments are not available .
Anaphylaxis is a severe, life-threatening generalized or systemic hypersensitivity reaction caused by the sudden release of mast cells and basophils mediators into the circulation. Anaphylaxis is characterized by gradual onset, life-threatening bronchospasm, circulatory collapse, and gastrointestinal symptoms and is usually associated with skin and mucosal urticarial/angioedema lesions .
Common triggers include food, drugs, contact allergens, and insect bites; however, sometimes, no trigger can be identified despite careful evaluation (idiopathic anaphylaxis). Anaphylaxis rarely complicates dermatologic conditions and/or therapeutic modalities (Table 3). The World Allergy Organization clinical criteria for the diagnosis of anaphylaxis are summarized in Table 4.
|Table 3: Dermatologic causes of anaphylaxis.|
|Table 4: 2011 World Allergy Organization clinical criteria for anaphylaxis .|
Anaphylaxis is a serious and potentially life-threatening event that must be immediately treated with epinephrine, regardless of the underlying pathophysiological mechanism. Intramuscular adrenaline is considered the first-line intervention that can be repeated every 5-10 min Figure 4. Adjunct measures include removal of the trigger and call for help, oxygen, fluid resuscitation, antihistamines, glucocorticosteroids, and inhaled short-acting beta-2-agonists [41–43]. Anaphylaxis related to the use of biological agents is discussed in Table 5.
|Figure 4: Anaphylaxis. Flow chart for the management approach of anaphylaxis. # NICE guidelines recommend measuring tryptase on two occasions, immediately after the emergency treatment of anaphylaxis and a second sample ideally within 1-2 hours of the onset of symptoms .|
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