EMERGENCY DERMATOLOGY
Dermatology and the acute patient
Fifth disease: erythema producing a slapped cheek appearance, with an associated reticulate erythema on the limbs, due to erythrovirus (formerly parvovirus) B19 infection.
Elisabeth M Higgins
Acute eczema Patients may present with an acute exacerbation of previous atopic dermatitis3 (often triggered by infection e bacterial or viral (see eczema herpeticum below) e or as a new presentation).3 The latter may be an acute exposure to a contact allergen (e.g. hair dye). Acute eczematous reactions on the palms and soles may be associated with extensive formation of vesicles, which can coalesce into large multi-loculated lesions (pompholyx) and cause significant functional impairment. However, in contrast to acute exacerbations of atopic eczema, the patient is usually systemically well and afebrile.
Abstract Dermatological conditions may present acutely in primary care or via the emergency department. In some cases the problem is an acute exacerbation of a pre-existing dermatosis, but in many cases it is a de novo presentation and therefore poses a greater diagnostic challenge for the clinicians involved. While life-threatening dermatoses are relatively rare, the morbidity associated with acute cutaneous disease is high. In both instances, early recognition and intervention is important in achieving disease control. This review aims to outline the most frequent and the most important of these presentations.
Scabies should always be excluded in any patient with extensive pruritus and minimal rash.
Keywords acute dermatosis; bullous disorders; drug eruptions; toxic epidermal necrolysis; toxic erythema; urticaria; vasculitis
Psoriasis Patients with psoriasis may also present with acute exacerbations. Distinct patterns are recognized4:
Acute inflammatory dermatoses
Guttate psoriasis: the sudden eruption of multiple widespread very small plaques associated with a recent streptococcal infection. It is often the first presentation of psoriasis, although there may be a family history. It may be self-limiting, but responds well to ultraviolet (UV) B phototherapy.
Urticaria/angioedema Urticaria is one of the most common acute dermatology presentations. It is a type 1 hypersensitivity reaction. Although there may be a specific trigger (e.g. food, drug), in many cases the reaction is non-specific and may be associated with a low-grade infection, usually viral.1 Blotchy, erythematous and oedematous patches and plaques develop on the body and are intensely itchy (hives) (Figure 1).
Acute generalized pustular psoriasis is a medical emergency. The patient is acutely unwell with friable erythematous and oedematous skin, studded with pinpoint sterile pustules, which are fragile and easily eroded to leave areas of denuded skin that are vulnerable to secondary infection. Treatment is with systemic agents and careful supportive nursing care. Complications include pulmonary oedema and the capillary leak syndrome, and the condition carries a significant mortality. Generalized pustular psoriasis can be precipitated by the injudicious use of potent topical corticosteroids, or the sudden withdrawal of systemic corticosteroids, both of which are always best avoided in psoriasis.
Angioedema: in more severe reactions, associated oedema may close the eyes or cause dramatic lip swelling, which is alarming for the patient. Usually associated with food or drug hypersensitivity, but C1-esterase deficiency should also be considered. Treatment of most urticarial reactions is with simple antihistamines, or occasionally, if the reaction is severe or prolonged, a short course of oral prednisolone. The condition is not lifethreatening, unless there are associated systemic symptoms of wheeze or laryngeal oedema, or signs of cardiovascular collapse.
Erythroderma Generalized erythema of the skin may be caused by acute exacerbations or pre-existing skin disease (e.g. eczema, psoriasis, pityriasis rubra pilaris) or as a new phenomenon associated with a drug reaction, infection, cutaneous lymphoma or as a paraneoplastic phenomenon. Treatment depends on the cause, but the condition is associated with significant physiological disturbance and attentive supportive care is required.
Toxic erythema This is a widespread acute, maculopapular erythema, which may be associated with a drug eruption2 (see later) or infection. The history is key in determining aetiology and associated fever is common. Many toxic erythemas are associated with non-specific low-grade viral infections but some specific patterns of viral exanthems are recognized: Measles: it is becoming more frequent again due to the low uptake of immunization over the past decade.
Erythema multiforme Targetoid erythematous lesions occur especially on distal sites, palms and soles. Infection, especially with herpes simplex virus (HSV), is the commonest cause. It is usually self-limiting (Figure 2).
Elisabeth M Higgins MA FRCP is a Consultant Dermatologist at King’s College Hospital, London, UK. Conflicts of interest: none declared.
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Drug reactions Drug reactions are common and may take many forms from localized (fixed drug eruption) to generalized. The most common patterns are: Toxic erythema2 (see above) Drug reaction with systemic symptom (DRESS) This pattern of drug reaction is characterized by erythema, significant oedema, particularly facial, peripheral eosinophilia, lymphadenopathy and transaminitis.5 Fever is common. The spectrum of causal agents is wide, but antibiotics and anticonvulsants are the most frequent causes. Withdrawal of the culprit drug and treatment with systemic corticosteroids is required, but the reaction may be quite prolonged. Acute generalized exanthematous pustulosis (AGEP) Sheets of superficial, pinpoint pustules appear on a background erythema. The condition may be localized or generalized and is most commonly associated with an antibiotic reaction. The eruption should be distinguished from generalized pustular psoriasis. Figure 1 Blotchy, intensely itchy, erythematous and oedematous patches and plaques in urticaria.
StevenseJohnson syndrome/toxic epidermal necrolysis (TEN)
Erythema nodosum In this condition, acute, firm, painful areas of subcutaneous swelling develop on the limbs, most usually the shins. A reactive immune complex-mediated condition, it may be associated with a wide variety of disorders, including sarcoidosis, infection, drugs and inflammatory bowel disease.
Erythema multiforme-like cutaneous lesions and erosions occur, associated with involvement of mucous membranes. Nomenclature depends on the extent of involvement/epidermal detachment. In TEN, widespread areas of epidermal detachment and necrosis occur (Figure 3). Mortality is high and specialist care with intensive care unit support is required. Drug reactions are most commonly implicated.
Pyoderma gangrenosum A rapidly developing, acutely painful area of ulceration with a characteristic purplish over-hanging edge, associated with inflammatory bowel disease, connective tissue disorders and haematological malignancies. The condition may occasionally be triggered by trauma, but must be distinguished from necrotizing fasciitis, as debridement is contra-indicated and causes the condition to extend. Treatment is with high-dose immunosuppression and treatment of the underlying condition.
Vasculitis Inflammation of cutaneous blood vessels is manifest as widespread palpable, purpuric lesions, most commonly on the legs (Figure 4). Infection is a common cause and specific patterns on €nlein purpura) may be the legs and buttocks (HenocheScho associated with a streptococcal infection and characterized by immunoglobulin A deposition on immunofluorescence. Associated abdominal pain and arthralgia are common.6 Vasculitis may also be associated with systemic disease6 (e.g. ChurgeStrauss syndrome, connective tissue disease and malignancy). Renal involvement is common, with proteinuria and haematuria. More widespread areas of cutaneous necrosis suggest vascular occlusion (e.g. anti-phospholipid syndrome) or disseminated intravascular coagulation. Infections Cellulitis: acute painful swelling and erythema, most commonly of the leg, due to a streptococcal infection.7 The oedema may be severe enough to produce localized blistering of the skin. The patient feels unwell, with a fever, and may experience rigors. When the process occurs on the face it is known as erysipelas and begins unilaterally. Necrotizing fasciitis is associated with a compartment syndrome characterized by intense pain, dusky areas on the skin and hypotension. It is a surgical emergency, requiring prompt debridement of the necrotic tissue.
Figure 2 Targetoid lesions of erythema multiforme.
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EMERGENCY DERMATOLOGY
Figure 3 Extensive denuded areas in a patient with toxic epidermal necrolysis.
Meningococcal septicaemia: the appearance of purpuric lesions in an unwell patient with signs of meningitis indicates meningococcal septicaemia and is a medical emergency, necessitating immediate treatment.8 A Gram stain of a smear from a skin lesion can help confirm the diagnosis.
Figure 4 Palpable purpura on the legs in vasculitis.
eczema in an acutely unwell patient, with fever and discomfort. The condition is often mistaken for bacterial infection, but responds promptly to aciclovir.
Varicellaezoster: chickenpox is usually a self-limiting infection in childhood, but can be more severe in adults, and complicated by pneumonitis and encephalitis.
Photosensitivity Acute photosensitive eruptions may be a manifestation of a connective tissue disease (e.g. systemic lupus erythematosus or dermatomyositis) or may occur as a primary photosensitive dermatosis, or as a drug- induced phenomenon (e.g. tetracyclines).9 They are distinct from simple sunburn, due to overexposure of skin to direct sunlight.
Herpes zoster: the reactivation of varicella is characterized by painful blisters, which may become haemorrhagic and eroded, occurring in a dermatomal distribution. There may be acute (e.g. Ramsay Hunt syndrome: bladder dysfunction in sacral cases) or late neurological sequelae (post-herpetic neuralgia). Disseminated infection indicates underlying immunosuppression.
Polymorphic light eruption: an acute and intensely itchy, papulo-vesicular eruption, most commonly seen in young adult females. It typically occurs 24e48 hours after sun exposure and may take up to 1 week to settle. It is more common in early summer, or during more intense exposure to sunshine abroad.
Staphylococcal scalded skin syndrome: acute erythema and superficial desquamation, usually in young children, due to a specific toxin-producing staphylococcus. The skin is exquisitely painful and individuals need treatment with appropriate intravenous antibiotics and supportive care. The condition should be distinguished from TEN.
Phototoxic reactions: some drugs, such as NSAIDs and quinolones, may induce a phototoxic reaction. Phytophotodermatitis A specific phototoxic reaction may occur if plant sap comes into contact with skin on a sunny day. Lesions are typically streaky in a rather haphazard fashion and may
Eczema herpeticum: inoculation of HSV onto eczematous skin spreads rapidly and is associated with deterioration of the
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2 Stern RS. Exanthematous drug eruption. N Eng J Med 2012; 366: 2492e501. 3 Creamer D, Barker J, Kerdel FA. Eczema, Acute adult dermatology diagnosis and management. London: Manson publishing, 2011; 7e24. 4 Creamer D, Barker J, Kerdel FA. Psoriasis, Acute adult dermatology diagnosis and management. London: Manson publishing, 2011; 25e34. 5 Cacoub P, Musette P, Descamps V , et al. The DRESS syndrome: a literature review. Am J Med 2011; 124: 588e97. 6 Marzano AV, Vezzoli P, Berti E. Skin involvement in cutaneous and systemic vasculitis. Auto-immune Rev 2013; 12: 467e76. 7 Cox NH. Streptococcal cellulitis/erysipelas of the lower leg. In: Williams HC, ed. Evidence-based dermatology. 2nd edn. Oxford: Blackwell Science, 2008. 8 Sabatini C, Bosis S, Semino M, et al. Clinical presentation of meningococcal disease in childhood. J Prev Med Hyg 2012; 53: 116e9. 9 Photosensitivity. In: Bolognia JL, Jorizzo JL, Schaffer JV, eds. Dermatology. 3rd edn. Oxford: Elsevier, 2012. 10 Creamer D, Barker J, Kerdel FA. Pregnancy dermatoses, Acute adult dermatology diagnosis and management. London: Manson publishing, 2011; 211e216. 11 Graham RM, Cox NH. Systemic disease and the skin. In: Burns DA, Breatnach S, Cox N, Griffiths CEM, eds. Rook’s textbook of dermatology. 8th edn. Oxford: Wiley-Blackwell, 2010.
blister. Intense post-inflammatory pigmentation is seen as the eruption fades. A very dramatic variant can be seen in individuals who have cleared overgrown areas using a strimmer (strimmer’s dermatitis). Pregnancy eruptions Although any rash can occur during pregnancy, some specific cutaneous eruptions are associated with pregnancy.10 Pruritic urticarial papules and plaques of pregnancy (PUPP): intensely itchy, small erythematous papules develop on the abdomen, typically within the striae, and may become confluent. Sparing of the umbilicus is characteristic. The eruption typically occurs towards the end of the last trimester in primagravidas, but may occasionally occur post-partum. The condition should be distinguished form the more serious immunobullous entity, pemphigoid gestationis, which is more typically seen in multigravidas and may be associated with fetal complications.
Auto-immune blistering disorders Immunobullous disorders, such as pemphigus vulgaris and bullous pemphigoid, need to be considered in the presentation of any blistering condition, but usually have a more insidious onset, although they can become very extensive and associated with significant morbidity. Control is achieved with immunosuppression (see Dermatological pharmacology: systemic drugs on pages 330e333 of this issue).
Practice points Connective tissue diseases C
These are predominantly covered in other chapters, but acute cutaneous presentations of lupus and dermatomyositis may occur, especially following sun exposure. Although dermatomyositis may be auto-immune, association with malignancy as a para-neoplastic phenomenon should be considered, especially in older patients.11 Detailed assessment of the extent of muscle involvement is required, especially in relation to respiratory function and bulbar involvement. A
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REFERENCES 1 Grattan C. The urticarias: pathophysiology and management. Clin Med 2012; 12: 164e7.
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Consider eczema herpeticum as a cause of acute deterioration in a patient with atopic dermatitis Remember to request an ophthalmic assessment in cases of herpes zoster involving the first branch of the trigeminal nerve, to exclude a dendritic ulcer Always consider infection in cases of vasculitis with fever (blood cultures should be set up) Drug eruptions may persist for several days/weeks after withdrawal of the culprit drug Rapid deterioration of vital signs (especially the development of hypotension) in a patient with lower leg cellulitis should raise the possibility of necrotizing fasciitis and the need for urgent surgical intervention
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