Approach to the Adult Rash

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191 Approach to the Adult Rash

Pathophysiology

A rash is a skin eruption that has a defined morphology and location. The morphology of the rash is usually distinct and related to the pathophysiologic dysfunction of the skin. Most severe rashes begin as exanthems (from the Greek exanthema, which means “breaking out,” and anthos, which means “flowering”). These rashes subsequently take on a particular morphology, characteristic lesions, or distribution. Table 191.1 delineates the most common terms used for skin lesions. Understanding these terms helps one better document progression of disease and facilitates communication with consulting physicians.

Table 191.1 Common Terms for Skin Lesions

TERM DESCRIPTION
Lesion Single small diseased area
Macule Circumscribed area of change without elevation
Rash Skin eruption that is more extensive than a single lesion
Papule Solid raised lesion < 1 cm
Nodule Solid raised lesion > 1 cm
Plaque Raised confluence of papules > 1 cm
Pustule Fluid-filled area containing purulence
Vesicle Fluid-filled area < 1 cm containing clear fluid
Bullae Fluid-filled area > 1 cm
Petechiae Pinpoint flat round spots < 3 mm caused by intradermal bleeding that do not blanch
Purpura Hemorrhagic area > 3 mm that does not blanch
Exanthem Rash outside the body (skin)
Enanthem Rash inside the body (mucous membranes)

Presenting Signs and Symptoms

Eliciting the initial distribution and progression of a rash is essential. Additionally, the involvement of palms, soles, and mucous membrane is of key importance. It should be kept in mind that dysphasia, as well as eye or genital irritation, may be a manifestation of as mucosal involvement and is often the initial symptom of several life-threatening conditions. The rash’s rapidity of progression is also an essential in diagnosis. Box 191.1 categorizes these important findings.

Specific Signs

Two signs are important in the evaluation of these rashes: the Nikolsky sign and the Asboe-Hansen sign. A positive Nikolsky sign (Fig. 191.1) is noted when slight rubbing of the skin results in exfoliation of the outermost layer with lateral extension of the erosion into the intact skin. The area of denuded skin is pink and tender. The Asboe-Hansen sign (indirect Nikolsky sign or Nikolsky II sign) is extension of a blister into normal skin with the application of light pressure on the top of the blister. All patients with tender, blistering, or sloughing skin should be evaluated serially for these important signs.

Differential Diagnoses and Medical Decision Making

History taking is an essential component in formulating appropriate differential diagnoses and guiding medical decision making. Inquiry regarding the patient’s travel, medical, occupational, recreational, and medicinal history is required. Once the history and physical examination are complete, in-depth evaluation of the rash is in order. The differential diagnoses can be narrowed by categorizing the rash as erythematous, maculopapular, petechial/purpuric, or vesiculobullous.

The Algorithmic Approach

Erythematous Rashes

These rashes are characterized by diffuse redness of the skin as a result of capillary congestion. Erythematous rashes are differentiated by the presence or absence of fever and the Nikolsky sign (Fig. 191.2).1 If a Nikolsky sign is present, the diagnosis is narrowed substantially, usually to TEN in adults and to staphylococcal scalded skin syndrome (SSSS), generally in infants and young children. If fever is present without a Nikolsky sign, the differential diagnosis includes Kawasaki disease, scarlet fever, erythroderma, and toxic shock syndrome (TSS). Patients with an erythematous rash but without a fever or Nikolsky sign may be having an anaphylactic reaction or a reaction to vancomycin, scombroid, or alcohol exposure. Please refer to Chapter 18 for review of SSSS, Kawasaki disease, and scarlet fever. Refer to Chapter 192 for review of TEN, TSS, and erythroderma.

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Fig. 191.2 Algorithm for erythematous rashes.

SSS, Scalded skin syndrome; TEN, toxic epidermal necrolysis

(From Murphy-Lavoie H, LeGros TL. Emergent diagnosis of the unknown rash: an algorithmic approach. Emerg Med 2010;42[3]:6-17.)

Maculopapular Rashes

The term maculopapule is a portmanteau of macule and papule. Maculopapular rashes are differentiated by the distribution of the rash and systemic toxicity (Fig. 191.3). Patients with centrally distributed rashes who appear toxic and febrile have a wide differential diagnosis; however, it is paramount that patients living in endemic areas be assessed for Lyme disease. Those with centrally distributed rashes but no signs of toxicity usually have either a drug reaction or pityriasis rosea. Patients with peripherally distributed rashes have a broader differential diagnosis that is dependent on systemic toxicity, the presence or absence of target lesions, and whether the rash is located on the flexor or extensor surfaces. Target lesions (Fig. 191.4) are pathognomonic for SJS or erythema multiforme (EM). The target lesion of Lyme disease is usually a single large bull’s eye that measures at least 5 cm in diameter (erythema migrans). Patients with peripheral lesions and systemic toxicity but without target lesions require emergency evaluation for meningococcemia, RMSF, and syphilis. Nontoxic patients with a peripherally distributed rash and no target lesions require further assessment for flexor involvement (scabies or eczema) or extensor involvement (psoriasis). Please refer to Table 191.2 for review of EM minor and major. See Chapter 18 for review of viral exanthems. Please refer to Chapter 192 for review of Lyme disease, meningococcemia, RMSF, SJS, and syphilis.

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Fig. 191.3 Algorithm for maculopapular rashes.

(From Murphy-Lavoie H, LeGros TL. Emergent diagnosis of the unknown rash: an algorithmic approach. Emerg Med 2010;42[3]:6-17.)

Table 191.2 Erythema Multiforme

Causes Possibly autoimmune
Unknown in 50%
Exposures Infections: herpes simplex, Mycoplasma, fungi
Drug exposures: sulfa and other antibiotics, anticonvulsants
Classification Erythema multiforme minor Erythema multiforme major
Description Mild, self-limited rash Severe, life-threatening disease with significant mucous membrane involvement2
Prodromal symptoms No prodromal symptoms Mild upper respiratory infection with moderate fever
Cough, sore throat, chest pain
Vomiting and diarrhea
Rash appears in 1-2 wk
Rash characteristics Symmetric extremity lesions that develop into target lesions Begins as a maculopapular rash that evolves into target lesions; rapidly progressive with centripetal spread
Distribution Lower extremities Palms, soles, dorsa of hands, face, and extensor surfaces
Mucous membrane involvement None Significant mucous membrane involvement
Eye involvement (10%); often bilateral, purulent conjunctivitis
Pruritic Yes No
Diagnosis Confirmed with biopsy Confirmed with biopsy
Treatment

Physical examination pearl In contrast to Stevens-Johnson syndrome or toxic epidermal necrolysis, the target lesions of erythema multiforme are discrete rather than confluent, and the skin is not usually tender. The skin is very tender with toxic epidermal necrolysis

Petechial/Purpuric Rashes

These rashes can be especially challenging and are associated with devastating differential diagnoses; however, an algorithmic approach can help the physician narrow the diagnosis with confidence (Fig. 191.5). By definition, petechiae do not blanche with pressure; additionally, remembering the cause of palpable versus nonpalpable lesions is paramount. Palpable (raised) purpura occurs in vasculitic diseases secondary to inflammation or infection. Nonpalpable purpura (flat, subcutaneous hemorrhages) are seen with thrombocytopenic conditions. Patients with petechial/purpuric rashes and fever or toxicity require emergency evaluation. If the lesions are palpable, the differential diagnosis includes meningococcemia, disseminated gonococcal disease, endocarditis, RMSF, and Henoch-Schönlein purpura (HSP). Those with petechial/purpuric rashes and fever or toxicity but without palpable lesions may have purpura fulminans, disseminated intravascular coagulopathy (DIC), or TTP. If the patient is afebrile and has a petechial or purpuric rash, the diagnosis may be far simpler and less ominous. Nontoxic patients with palpable lesions may have a vasculitis; those with nonpalpable lesions may have idiopathic thrombocytopenic purpura (ITP). A detailed history may elucidate the cause of a vasculitis by identifying a key drug use history, recent illness, or an underlying symptom characteristic of the triggering disease.5 In patients with ITP, it is important to exclude other causes of thrombocytopenia, including HIV infection, hepatitis, autoimmune disease, liver or renal disease, cancer, infection, pregnancy, alcohol use, and exposure to heparin or other inciting drugs or agents. Patient with ITP also require a full assessment of their risk for complications because of their thrombocytopenic condition, including age older than 60 years, engagement in athletic sport activities, peptic ulcer disease, menorrhagia, and intracranial hemorrhage.4 Please refer to Table 191.3 for review of disseminated gonococcemia, secondary vasculitis, and ITP. Box 191.2 lists a vasculitis classification scheme, and these conditions are discussed in more detail in Chapter 110. Please refer to Chapter 18 for review of HSP and Chapter 192 for review of meningococcemia, endocarditis, RMSF, TTP, and purpura fulminans/DIC.

Vesiculobullous Rashes

Vesiculobullous rashes provoke significant angst in many physicians (Fig. 191.6). However, the differential diagnosis can be greatly simplified by categorizing patients with these rashes as febrile or afebrile and noting whether the distribution of the rash is diffuse or localized. Patients with a diffuse vesiculobullous rash and a fever may have varicella or a more devastating illness such as smallpox, disseminated gonococcal disease, or purpura fulminans/DIC. Necrotizing fasciitis and hand-foot-and-mouth disease are characterized by localized lesions and fever. In afebrile patients with a diffuse vesiculobullous rash, the differential diagnosis includes bullous pemphigus (BP) and pemphigus vulgaris (PV). One of the distinguishing features is based on the Nikolsky sign. A positive Nikolsky sign is seen with PV but is absent in BP. These entities are regularly confused, and it is essential to differentiate them urgently. It is important to remember that both BP and PV have strong associations and known triggers. For those with BP, the list of drug triggers includes furosemide, ibuprofen, captopril (or other thiol-containing compounds), penicillamine, and antibiotics. Patients with PV have similar drug triggers (with the addition of rifampin). Please refer to Table 191.4 for additional associations for PV and BP. The differential diagnosis is simpler and less of an emergency in a patient who is afebrile with a localized vesiculobullous rash; contact dermatitis, herpes zoster, dyshidrotic eczema, and burns are included in the differential diagnosis. Please refer to Table 191.4 for a discussion of PV and BP. Refer to Chapters 18 and 192 for review of varicella and Chapter 192 for review of smallpox, purpura fulminans/DIC, and necrotizing fasciitis.

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Fig. 191.6 Algorithm for vesiculobullous rashes.

DIC, Disseminated intravascular coagulopathy; GC, gonococcal disease.

(From Murphy-Lavoie H, LeGros TL. Emergent diagnosis of the unknown rash: an algorithmic approach. Emerg Med 2010;42[3]:6-17.)

Table 191.4 Pemphigus Vulgaris and Bullous Pemphigus

  PEMPHIGUS VULGARIS BULLOUS PEMPHIGUS
Causes

Characteristics

Associations Clinical sign Diagnosis Treatment Prognosis

Treatment

The type of rash, the overall health and physical reserves of the patient, and the rapidity of diagnosis determine treatment. Many of these diseases are associated with significant morbidity and mortality, and a high index of suspicion is required to prevent a delay in diagnosis.

Erythematous Rashes

Febrile Patients with a Positive Nikolsky Sign

These patients are systemically ill and require intensive treatment. Patients with SSSS usually require hospitalization for fluid and electrolyte management and supportive wound and skin care. Young, well-appearing patients with SSSS and minimal skin sloughing may be managed as outpatients. However, adult patients with SSSS have a 60% mortality rate and require much closer monitoring. Antibiotics are usually recommended, but is it unclear whether they measurably alter the course of the disease. Please refer to Chapters 18 and 192 for further review. Patients with TEN require immediate discontinuation of the offending agent, wound care, eye care, fluid and electrolyte resuscitation, and admission to either an intensive care unit (ICU) or burn unit. Intravenous immune globulin (IVIG) may be helpful, but it has yet to be approved by the Food and Drug Administration for this use. Most physicians recommend against steroid use. It is important to avoid sulfadiazine on these wounds because sulfa is a common offending agent.

Maculopapular Rashes

Peripheral Maculopapular Rashes in Febrile or Ill-Appearing Patients with Target Lesions

These patients may have EM major (refer to Table 191.2 for review). SJS and Lyme disease are also in the differential diagnosis. Treatment of SJS involves discontinuation of the offending agent, optimization of fluid and electrolyte balance, meticulous wound care, and ICU admission. For patients with Lyme disease, doxycycline is first-line treatment in nonpregnant adults. Children are treated with amoxicillin. Refer to Chapter 192 for further review of SJS and Lyme disease.

Petechial/Purpuric Rashes

Palpable Petechial/Purpuric Rashes in Febrile Patients

These patients have wide differential diagnoses with high morbidity and mortality. Meningococcemia and RMSF were discussed in the section related to the treatment of maculopapular rashes. Treatment of disseminated gonococcemia is reviewed in Table 191.3. Patients with endocarditis require early recognition, intensive therapy, broad-spectrum antibiotics to cover methicillin-resistant Staphylococcus (with subsequent guidance by blood cultures), and cardiology consultation. See Chapter 192 for further information on endocarditis. HSP is a small vessel vasculitis that is usually self-limited, and treatment is supportive. NSAIDs are used to reduce joint and soft tissue pain. Patients with significant bleeding, intussusception, and renal failure require admission for HSP. Please refer to Chapter 192 for review of endocarditis and Chapter 18 for review of HSP.

Vesiculobullous Rashes

Follow-Up and Patient Education