Lupus Erythematosus Disease

BASICS

DESCRIPTION

Discoid lupus erythematosus (DLE) is the most common form of chronic cutaneous lupus. It is a chronic skin disease characterized by sharply marginated dull, red macules with adherent scales extending into areas of atrophy, telangiectasias, or follicular plugging

Localized DLE - more common form with lesions occurring on the face especially the malar areas, bridge of nose, lower lip, lower eyelids and ears
Widespread DLE - lesions seen on upper extremities and thorax
System(s) affected: Skin/Exocrine
Genetics: N/A
Incidence/Prevalence in USA:
- 3/100,000 Caucasian females; 8/100,000 black females
- 100/100,000
Predominant age: 25 to 45
Predominant sex:
- Localized DLE - Female > Male (3:1)
- Widespread DLE - Female > Male (9:1)

SIGNS AND SYMPTOMS

Red plaque-like lesions on face, thorax, or extensor aspect of upper extremities; rare below waist
Older lesions atrophy and appear as smooth white or hyperpigmented scars with telangiectasias
Scarring alopecia with scalp lesions
"Carpet tack" appearance of skin when scale removed
Lesions occasionally slightly pruritic or stinging
Oral ulceration in 15 percent of patients
Photosensitivity
Koebner response (precipitation by cutaneous trauma)

CAUSES

Unknown

RISK FACTORS

Systemic lupus erythematosus (SLE)

DIAGNOSIS

LABORATORY

Localized DLE: positive ANA in low titer (30%)
Widespread DLE, may occasionally find, increased sedimentation rate, positive ANA (30-40%), positive dsDNA (< 5%), leukopenia, hematuria and albuminuria if concomitant SLE

Drugs that may alter lab results: N/A
Disorders that may alter lab results: Concomitant SLE

PATHOLOGICAL FINDINGS

Hyperkeratosis
Epidermal atrophy
Liquefactive degeneration of basal cell layer
Edema, mucin, and inflammation of dermis
Follicular plugging
Basement zone thickened with strong periodic acid–Schiff reaction staining

SPECIAL TESTS

Immunofluorescent staining of skin biopsies (lupus band test)

IMAGING

Chest x-ray: consolidation with radiolucency
Air-fluid level
Pleural effusion
CT: define location and extent

DIAGNOSTIC PROCEDURES

Bronchoscopy if obstruction suspected
Bronchoscopic protected brushing
Bronchoalveolar lavage
Transthoracic needle aspiration

TREATMENT

APPROPRIATE HEALTH CARE

Outpatient

GENERAL MEASURES

Avoid sun exposure, avoid excessive heat, cold, or trauma

SURGICAL MEASURES

N/A

ACTIVITY

Full activity

DIET

Regular

PATIENT EDUCATION

Teach patients proper use of sunscreens and other measures to prevent sun exposure (e.g., wide-brimmed hats, long sleeves, etc.)
Advise patient about symptoms of systemic lupus erythematosus (SLE) that should be watched for
Griffiths: Instructions for Patients, Philadelphia, W.B. Saunders Co.

FOLLOW UP

PREVENTION/AVOIDANCE

Avoid sun exposure or excessive heat, cold, or skin trauma

POSSIBLE COMPLICATIONS

Hypertrophic scarring, hypopigmentation (especially in blacks)

EXPECTED COURSE AND PROGNOSIS

40% remit completely; 1–5% may develop systemic lupus (these patients usually have widespread DLE)
Not life-threatening unless it turns into systemic type

MISCELLANEOUS

ASSOCIATED CONDITIONS

Systemic lupus erythematosus
Mixed connective tissue disease (MCTD)
Antiphospholipid syndrome

AGE-RELATED FACTORS

Pediatric: Neonatal lupus erythematosus is a syndrome of cutaneous lupus and/or congenital heart block. It is caused by transplacental passage of one of several maternal antibodies.
Geriatric: N/A
Others: N/A

PREGNANCY

N/A

OTHER NOTES

N/A

ABBREVIATIONS

N/A

Clinical Investigations

ROLE OF HOMOEOPATHY

Holistic healing that treats the person, not just the disease. Experience gentle, lasting wellness with expert constitutional care.