Erythema multiforme

Erythema Multiforme Recurrence

Figure 12-2. Vascular dermatoses. Erythema multiforme-like eruption on arm during pregnancy. (Dermik Laboratories, Inc.)

The term erythema multiforme introduces a flurry of confusion in the mind of any student of medicine. It is our purpose in this section to attempt to dispel that confusion. Erythema multiforme, as originally described by Hebra, is an uncommon, distinct disease of unknown cause characterized by red iris-shaped or bull's eye-like macules, papules, or bullae confined mainly to the extremities, the face, and the lips. It is accompanied by mild fever, malaise, and arthralgia. It occurs usually in children and young adults in the spring and the fall, has a duration of 2 to 4 weeks, and frequently is recurrent for several years.

The only relation between Hebra's erythema multiforme and the following diseases or syndromes is the clinical appearance of the eruption.

Stevens-Johnson syndrome is a severe and rarely fatal variant of erythema multiforme. It is characterized by high fever, extensive purpura, bullae, ulcers of the mucous membranes, and, after 2 to 3 days, ulcers of the skin. Eye involvement can result in blindness. It can be related to drugs and it's severest form is considered by some to be the same as toxic epidermal necrolysis (see Chapter.,22).

Erythema multiforme bullosum is a severe, chronic, bullous disease of adults (see Chap..22). There is an opinion that this syndrome is completely separate from erythema multiforme. More macular, truncal lesions and more epidermal necrosis and less infiltrate may be seen in Stevens-Johnson syndrome (toxic epidermal necrolysis). Sulfonamides, anticonvulsant agents, allopurinol, chlormezanone and nonsteroidal antiinflammatory drugs.

Erythema multiforme-like drug eruption is frequently due to phenacetin, quinine, penicillin, mercury, arsenic, phenylbutazone, barbiturates, trimethadione, phenytoin, sulfonamides, and antitoxins (see Chap..9).

Erythema multiforme-like eruption is seen rather commonly as part of a herpes simplex outbreak and also in conjunction with rheumatic fever, pneumonia, meningitis, measles, Coxsackievirus infection, pregnancy, and cancer, as well as after deep x-ray therapy, and as an allergic reaction to foods.

The erythema perstans group or figurate erythemas of diseases includes over a dozen clinical entities with impossible-to-remember names. (See D,ictjMoina^[yzIn,d.e.x, under erythema perstans.) All have various sized erythematous patches, papules, or plaques with a definite red border and a less active center, forming circles, half circles, groups of circles, and linear bands. Multiple causes have been ascribed, including tick bites; allergic reactions; fungal, bacterial, viral, and spirochetal infections; and internal cancer. The duration of and the response to therapy varies with each individual case.

Erythema chronicum migrans is the distinctive cutaneous eruption of the multisystem tick-borne spirochetosis Lyme disease. The deer tick, Ixodes dammini, is the vector for the spirochete. Early therapy with doxycycline or ampicillin may prevent late manifestations of the disease (see Chap 16).

Reiter's syndrome is a triad of conjunctivitis, urethritis, and, most important, arthritis, that occurs predominantly in males and lasts about 6 months. The skin manifestations consist of psoriasiform dermatitis that is called balanitis circinata on the penis and keratoderma blenorrhagica on the palms and soles.

Behget's syndrome consists of a triad of genital, oral, and ophthalmic ulcerations seen most commonly in males; it can last for years, with recurrences. Other manifestations include cutaneous pustular vasculitis, synovitis, and meningoencephalitis. It is more prevalent in eastern Mediterranean countries and Japan.

Differential Diagnosis

Urticaria: Clinically, urticaria may resemble erythema multiforme, but hives are associated with only mild systemic symptoms; it can occur in any age group; iris lesions are unusual; usually, it can be attributed to penicillin or other drug therapy; and it responds rapidly but often not completely to antihistamine therapy (see first part of this chapter). It is evanescent and dissipates or moves to a new area in less than 24 hours.

Treatment

A 12-year-old boy presents with bull's eye-like lesions on his hands, arms, and feet; erosions of the lips and mucous membranes of the mouth; malaise; and a temperature of 101- F (38.3- C) orally. He had a similar eruption last spring.

1. Order bed rest and increased oral fluid intake.

2. Acetaminophen (Tylenol), 325 mg OTC Sig: Take 1 to 2 tablets q.i.d, or

Prednisone, 10 mg #16

Sig: Take 2 tablets stat and then 2 tablets every morning for 7 days.

3. For severe cases, such as the Stevens-Johnson form, hospitalization is indicated, where intravenous corticosteroid therapy (debatable), intravenous infusions, immune globulin, and other supportive measures can be administered.

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