Pemphigoid, Bullous Disease – The Healing Space Clinic

Pemphigoid Bullous Disease

DESCRIPTION

Chronic benign bullous eruption considered to be an autoimmune disease. Most frequently affects people over 60.

System(s) affected: Skin/Exocrine, Hemic/Lymphatic/Immunologic
Genetics: HLA typing does not reveal any typical pattern
Incidence/Prevalence in USA: Uncommon
Predominant age: Greater than 60 years
Predominant sex: Female > Male

SIGNS AND SYMPTOMS

Large bullae 2 to 5 cm in diameter; occasional tiny peripheral vesicles
Bullae that arise from normal-appearing skin (sometimes) or erythematous skin (usually)
Bullae stay intact for many days
Located on extremities at first, trunk later
Occasionally located on the scalp, palms, and soles; mucous membranes (infrequently)
Intact blisters outnumber erosions (reverse is true with pemphigus)
Clear fluid fills bullae (usually)
Blood-tinged fluid in bullae (sometimes)
Itching (sometimes severe)
Some patients are asymptomatic
10–20% of skin surface is continuously involved
Pruritus may antedate onset of blisters (weeks to months)

CAUSES

Autoimmune disorder

RISK FACTORS

Female, age over 60
Drug associated: furosemide, phenacetin, various penicillins

LABORATORY

Circulating autoantibodies in 70% directed at the basement membrane (by immunofluorescence). These can be demonstrated in serum or skin.
Demonstration of antibodies directed toward bullous pemphigoid antigens BP230 (BPAG1) and BP180 (BPAG2)

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

PATHOLOGICAL FINDINGS

Bullae located in a subepidermal location
Light microscopy reveals subepidermal blister with perilesional inflammation containing many eosinophils and mononuclear cells
Immunofluorescent studies – deposition of C3 (100%) and IgG (65–90%) in the lamina lucida

SPECIAL TESTS

N/A

IMAGING

N/A

DIAGNOSTIC PROCEDURES

History and physical
Biopsy and immunofluorescence studies – essential for precise diagnosis

APPROPRIATE HEALTH CARE

Outpatient, unless significant complications

GENERAL MEASURES

Soak active lesions to debride and remove crusts
Analgesic mouth washes (see Medications)

SURGICAL MEASURES

N/A

ACTIVITY

Depends on severity of disease and/or complications

DIET

Liquid. Regular diet when tolerated.

PATIENT EDUCATION

Use of oral analgesics
Teach side effects and adverse reactions of steroids

PREVENTION/AVOIDANCE

N/A

POSSIBLE COMPLICATIONS

Superimposed infection (may result in death in elderly debilitated patient)
Complications of steroid therapy
Associated malignancy
Untreated severe disease can be fatal

EXPECTED COURSE AND PROGNOSIS

A chronic disease that lasts indefinitely
Old lesions heal rapidly as new lesions appear
Accompanying debilitation not as great as with pemphigus

ASSOCIATED CONDITIONS

May have an associated malignancy

AGE-RELATED FACTORS

Pediatric: Not a problem in pediatric age group
Geriatric: Older people with pemphigoid may have higher than expected rate of malignancy
Others: N/A

PREGNANCY

N/A

OTHER NOTES

N/A

ABBREVIATIONS

N/A

Clinical Investigations

ROLE OF HOMOEOPATHY

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