Butterfly rash
Butterfly rash: Excerpt from Handbook of Signs & Symptoms (Third Edition)
The presence of a butterfly rash is typically a sign of systemic lupus erythematosus (SLE), but it can also signal dermatologic disorders. Typically, butterfly rash appears in a malar distribution across the nose and cheeks. (See Recognizing butterfly rash, page 122.) Similar rashes may appear on the neck, scalp, and other areas. Butterfly rash is sometimes mistaken for sunburn because it can be provoked or aggravated by ultraviolet rays, but it has more substance, is more sharply demarcated, and has a thicker feel in relation to surrounding skin.
History and physical examination
Ask the patient when he first noticed the butterfly rash and if he has recently been exposed to the sun. Has he noticed a rash elsewhere on his body? Also, ask about recent weight or hair loss. Does he have a family history of lupus? Is he taking hydralazine or procainamide (common causes of drug-induced lupus erythematosus [LE])?
Inspect the rash, noting any macules, papules, pustules, or scaling. Is the rash edematous? Are areas of hypopigmentation or hyperpigmentation present? Look for blisters or ulcers in the mouth, and note any inflamed lesions. Check for rashes elsewhere on the body.
Medical causes
❑ Discoid lupus erythematosus. With discoid lupus erythematosus, a localized form of LE, the patient may come into your facility with a unilateral or butterfly rash that consists of erythematous, raised, sharply demarcated plaques with follicular plugging and central atrophy. The rash may also involve the scalp, ears, chest, or any part of the body exposed to the sun. Telangiectasia, scarring alopecia, and hypopigmentation or hyperpigmentation may occur later. Other accompanying signs include conjunctival redness, dilated capillaries of the nail fold, bilateral parotid gland enlargement, oral lesions, and mottled, reddish blue skin on the legs.
❑ Erysipelas. Erysipelas causes rosy or crimson swollen lesions, mainly on the neck and head and commonly along the nasolabial fold. It may cause hemorrhagic pus-filled blisters. Other signs and symptoms include fever, chills, cervical lymphadenopathy, and malaise.
❑ Polymorphous light eruption. Butterfly rash appears as erythema, vesicles, plaques, and multiple small papules that may later become eczematized, lichenified, and excoriated. Provoked by ultraviolet rays, the rash appears on the cheeks and bridge of the nose, the hands and arms, and other areas, beginning a few hours to several days after exposure. It may be accompanied by pruritus.
❑ Rosacea. Initially, butterfly rash may appear as a prominent, nonscaling, intermittent erythema limited to the lower half of the nose or including the chin, cheeks, and central forehead. As rosacea develops, the duration of the rash increases; instead of disappearing after each episode, the rash varies in intensity and is commonly accompanied by telangiectasia. With advanced rosacea, the skin is oily, with papules, pustules, nodules, and telangiectasis restricted to the central oval of the face. In men with severe rosacea, butterfly rash may be accompanied by rhinophyma — a thickened, lobulated overgrowth of sebaceous glands and epithelial connective tissue on the lower half of the nose and, possibly, the adjacent cheeks. This is more common in elderly patients.
❑ Seborrheic dermatitis. Butterfly rash appears as greasy, scaling, slightly yellow macules and papules of varying size on the cheeks and the bridge of the nose, in a “butterfly” pattern. The scalp, beard, eyebrows, portions of the forehead above the bridge of the nose, nasolabial fold, or trunk may also be involved. Associated signs and symptoms include crusts and fissures (particularly when the external ear and scalp are involved), pruritus, redness, blepharitis, styes, severe acne, and oily skin. Severe seborrheic dermatitis of the face occurs in acquired immunodeficiency syndrome.
❑ Systemic lupus erythematosus. Occurring in about 40% of patients with this connective tissue disorder, butterfly rash appears as a red, usually scaly, sharply demarcated macular eruption. The rash may be transient in patients with acute SLE or may progress slowly to include the forehead, the chin, the area around the ears, and other exposed areas. Common associated skin findings include scaling, patchy alopecia, mucous membrane lesions, mottled erythema of the palms and fingers, periungual erythema with edema, reddish purple macular lesions on the volar surfaces of the fingers, telangiectasia of the base of the nails or eyelids, purpura, petechiae, and ecchymoses.
Butterfly rash may also be accompanied by joint pain, stiffness, and deformities, particularly ulnar deviation of the fingers and subluxation of the proximal interphalangeal joints. Related findings include periorbital and facial edema, dyspnea, a low-grade fever, malaise, weakness, fatigue, weight loss, anorexia, nausea, vomiting, lymphadenopathy, photosensitivity, and hepatosplenomegaly.
Other causes
❑ Drugs. Hydralazine and procainamide can cause a lupuslike syndrome.
Special considerations
Prepare the patient for immunologic studies, complete blood count and, possibly, liver studies. Obtain a urine specimen, if needed. Withhold photosensitizing drugs, such as phenothiazines, sulfonamides, sulfonylureas, and thiazide diuretics. Instruct the patient to avoid exposure to the sun or to use sunscreen. Suggest that he use hypoallergenic makeup to help conceal facial lesions.
Pediatric pointers
Rare in pediatric patients, a butterfly rash may occur as part of an infectious disease such as erythema infectiosum, or “slapped cheek syndrome.”
Pictures
Book Source Details
- Book Title: Handbook of Signs & Symptoms (Third Edition)
- Author(s): Springhouse
- Year of Publication: 2006
- Copyright Details: Handbook of Signs & Symptoms (Third Edition), Copyright © 2006 Lippincott Williams & Wilkins.
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