PEMPHIGUS

PEMPHIGUS
• Pemphigus is a serious autoimmune disease of the skin and of the

mucous membranes, characterized by the appearance of flaccid
blisters (bullae) of various sizes on apparently normal skin and
mucous membranes (mouth, esophagus, conjunctiva, vagina)
• Familial benign chronic pemphigus (Hailey-Hailey disease) is a familial
type of pemphigus that appears in adults, affecting particularly the
axillae and groin.
• It is usually due to IgG1 and IgG4 auto antibodies, directed against
desmogleins-1 and 3 and causing intra-epidermal blistering, but it can
also be drug-induced (e.g. by penicillamine or captopril) and is rarely
associated with an underlying malignancy (‘para neoplastic
pemphigus’).
Pathophysiology and Etiology
• The etiologic agent causing pemphigus is unknown.

• Certain drugs, other autoimmune diseases, and genetics may play a
role in its development.
• Many variants of pemphigus exist.
Clinical features
•Initial lesions may appear in oral cavity; flaccid blisters (bullae) may arise on
normal or erythematous skin.
• The bullae enlarge and rupture, forming painful, raw, and denuded
areas that eventually become crusted.
• The eroded skin heals slowly; eventually, widespread areas of the body
may become involved.
• In the mouth, the blisters are usually multiple, of varying size and
irregular shape, painful, and persistent. Oral lesions may appear
initially, with lesions of the mucous membranes of the pharynx and
esophagus; the conjunctivae, larynx, urethra, cervix, and rectum may
become affected as well.
• An offensive odor may emanate from the bullae due to infection
• Flaccid blisters occur on the skin, usually on the upper trunk and
back.
• As the blisters are superficial, they are fragile and may look like
erosions.
• The Nikolsky sign is positive. (separation of epidermis when minimal
pressure is applied to the skin. Downward pressure on a bulla will
cause it to expand laterally.)
• Mucosal involvement can be severe, increasing the morbidity and
risk of mortality.
• Pemphigus can be associated with other autoimmune disorders and
full assessment for these is mandatory
Diagnostic Evaluation
• Skin biopsies of blisters and surrounding skinâ€”demonstrate

acantholysis (separation of epidermal cells from each other).
• Immunofluorescence of skin cells shows antibodies that bind to the
epidermis in a lacy pattern network. (pemphigus antibodies).
• In the acute situation, histology from a Tzank smear (taken from the
base of the blister with a scalpel) reveals acantholysis, i.e. separation
of the epidermal cells.
• Disease activity can be monitored by measur ing the level of
circulating autoantibody in serum by indirect immunofluorescence.
• Investigations should also include full blood count, ESR, urea and elec
trolytes, liver function tests, chest X-ray and other imaging,
particularly if paraneoplastic pemphigus is suspected
Management
• Bullous pemphigus can be managed with very potent topical steroids
in frail elderly patients, but in most patients a high-dose systemic
corticosteroid or azathioprine is required.
• With appropriate management, the disease tends to remit within
about 5 years.
• Anti-type VII collagen IgG, IgA, IgM at BMZ Systemic treatment with
high-dose oral steroids is required, but the disease is more difficult to
control than BP and life-long treatment is generally required.
• Azathioprine and cyclophosphamide are alternatives. Intravenous IgG
may be used to gain rapid control in severe cases.
• Corticosteroids in large doses to control the disease and keep skin
free from blisters.
• Immunosuppressive agents, such as cyclophosphamide (Cytoxan) and
azathioprine (Imuran), are used alone or in combination with steroids,
for immunosuppressive and steroid-sparing effect.
• Plasmapheresis reinfusion of specially treated plasma cells;
temporarily decreases serum level of antibodies.
• Treatment of denuded skin.
Complications
• Infections (skin, pneumonia, septicemia).

• Adverse effects from acute and chronic corticosteroids; GI bleeding,
secondary infection, psychosis, hyperglycemia, and others.
Nursing Assessment
• Assess for odor or drainage from lesions, which may indicate

infection.
• Assess for fever and signs of systemic infection.
• Assess for adverse effects of corticosteroids, such as abdominal pain,
white patches in mouth that indicate Candida infection, and
emotional changes.
Nursing Diagnoses
• Impaired Oral Mucous Membrane related to ruptured bullae

• Impaired Skin Integrity related to ruptured bullae
• Risk for Deficient Fluid Volume related to transudation of fluid into
bullae
• Disturbed Body Image related to widespread or chronic skin lesions
Nursing Interventions
• Restoring Oral Mucous Membrane Integrity

• Inspect oral cavity daily; note and report any changesâ€”oral lesions
heal slowly.
• Keep oral mucosa clean and allow regeneration of epithelium.
• Give topical oral therapy as directed.
• Offer prescribed mouthwashes through a straw, to rinse mouth of
debris and to soothe ulcerative areas.
• Teach patient to apply petrolatum to lips frequently.
• Use cool-mist therapy to humidify environmental air.
Restoring Skin Integrity
• Keep skin clean and eliminate debris and dead skin the bullae will clear if
epithelium at the base is clean and not infected.
• Obtain swab of bullous fluid for cultures”most common organism is S. aureus.
• Administer cool, wet dressings or baths or teach patient to administer, to
soothe and cleanse skin. Large areas of blistering have a characteristic odor
that is lessened when secondary infection is under control.
• After the bath, dry and cover with talcum powder as directed; this enables
the patient to move more freely in bed. Large amounts are necessary to keep
clothes and sheets from sticking.
• The nursing management of patients with blistering or with bullous skin
conditions is similar to that of the patient with a burn
Restoring Fluid Balance
• Evaluate for fluid and electrolyte imbalance extensive denudation of the skin
leads to fluid and electrolyte imbalance.
• Monitor serum albumin and protein levels.
• Monitor vital signs for hypotension or tachycardia.
• Weigh patient daily.
• Monitor intake and output.
• Administer I.V. saline solutions as directed.
• Encourage the patient to maintain hydration; suggest cool nonirritating fluids.
• Suggest soft, high-protein, high-calorie diet, or liquid supplements (Ensure,
Sustacal, eggnogs, milkshakes) that will not be irritating to oral mucosa but will
replace lost protein.
Promoting Positive Body Image
• Develop a trusting relationship with the patient.

• Educate patient and family about the disease and its treatment; this
reduces uncertainty and clears up misconceptions.
• Encourage expression of anxieties, embarrassment, discouragement.
• Encourage patient to maintain social contacts and activities among
support network.
Patient Education and Health Maintenance
Instruct the patient as follows:

• The disease may be characterized by relapses that require continuing
therapy to maintain control.
• Long-term administration of immunosuppressive drugs is associated
with numerous adverse effects and risks hyperglycemia, osteoporosis,
psychosis, adrenal suppression, and increased risk of cancer. Report
for health care follow-up visits regularly.
• Monitor skin/mouth for recurrence of pemphigus activity.
Evaluation: Expected Outcomes
• Oral mucous membranes pink with healing lesions and no signs of

infection
• Skin with intact bullae, healing lesions, and no signs of infection
• Vital signs stable; urine output adequate
• Patient expresses concerns and plans activities

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PEMPHIGUS

• Pemphigus is a serious autoimmune disease of the skin and of the

• The etiologic agent causing pemphigus is unknown.

• Skin biopsies of blisters and surrounding skinâ€”demonstrate

• Infections (skin, pneumonia, septicemia).

• Assess for odor or drainage from lesions, which may indicate

• Impaired Oral Mucous Membrane related to ruptured bullae

• Restoring Oral Mucous Membrane Integrity

• Develop a trusting relationship with the patient.

Instruct the patient as follows:

• Oral mucous membranes pink with healing lesions and no signs of

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