Dermatology

DR. ABDULQADIR M. MOALIM

DR. ABDULQADIR M. MOALIM
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BULLOUS DISEASES
Blistering can occur at any level in the skin and, as
such, there are a variety of different presentations,
depending on the underlying defect and level of
involvement.

DR. ABDULQADIR M. MOALIM

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Toxic epidermal necrolysis

Toxic epidermal necrolysis (TEN) is a medical
emergency, as the extensive mucocutaneous blistering
is associated with a high mortality rate. It is usually
drug-induced, particularly by anticonvulsants,
sulphonamides, sulphonylureas, NSAIDs, allopurinol
and antiretroviral therapy.

DR. ABDULQADIR M. MOALIM

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DR. ABDULQADIR M. MOALIM

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TEN

Usually 1–4 weeks after drug commencement, the

patient becomes systemically unwell and often
pyrexial, and erythema and blistering develop, initially
on the trunk but rapidly involving all skin. Sheets of
blisters coalesce and denude, and the underlying skin
is painful and erythematous

DR. ABDULQADIR M. MOALIM

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TEN
Identification and discontinuation of the causative drug are
essential. Intensive care in a dedicated dermatology ward
or intensive care or burns unit is of paramount importance.
Treatment is supportive, with regular sterile dressings and
emollients, careful attention to fluid balance and monitoring
for infection. Urethral and ocular involvement is common
and must be looked for and treated symptomatically, as
ocular and urethral scarring in survivors can be problematic.

DR. ABDULQADIR M. MOALIM

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Immunobullous diseases

The age of the patient may be informative in suspected

immunobullous disease. The key investigation is an
elliptical biopsy taken from the edge of a recent blister.

DR. ABDULQADIR M. MOALIM

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Bullous pemphigoid

Bullous pemphigoid (BP) is the most common

immunobullous disease and occurs worldwide. It is a
disease of the elderly, with an average age of onset of
65 years; males and females are equally affected.
A range of autoantibodies develop. Antibody–antigen
binding initiates complement activation and
inflammation, hemi-desmosomal damage and
consequent subepidermal blistering.

DR. ABDULQADIR M. MOALIM

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There is often a lengthy prodrome of an itchy,

urticated, erythematous rash prior to the
development of tense bullae. Milia may develop due
to basement membrane disruption. Mucosal
involvement is uncommon. Histology shows
subepidermal blistering with an eosinophil-rich
inflammatory infiltrate. Direct immunofluorescence
demonstrates the presence of IgG and C3 at the
basement membrane,

DR. ABDULQADIR M. MOALIM

Bullous pemphigoid.
A Large, tense, unilocular blisters.
B Immunofluorescence on salt-split skin, showing a
subepidermal blister and linear IgG and C3 deposition at
the basement membrane zone.

DR. ABDULQADIR M. MOALIM

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DR. ABDULQADIR M. MOALIM

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DR. ABDULQADIR M. MOALIM

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Management
Very potent topical steroids to all sites may be
sufficient in frail elderly patients. Tetracyclines, such as
doxycycline, have an important role and may limit the
use of systemic corticosteroids. However, most require
systemic corticosteroids, often combined with
azathioprine.

DR. ABDULQADIR M. MOALIM

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Pemphigus
Pemphigus is less common than BP and patients are usually
younger. Typically, IgG1 and IgG4 autoantibodies, directed
against desmogleins-1 and 3, develop, resulting in intra-
epidermal blistering. Secondary causes are drugs (e.g.
penicillamine or captopril) and underlying malignancy
(‘paraneoplastic pemphigus’). Pemphigus foliaceus is a very
superficial form, in which antibodies are directed against
desmoglein-1 only and affect just the most superficial
epidermis.

DR. ABDULQADIR M. MOALIM

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Clinical features and diagnosis

Skin and mucosae are usually involved, although disease may
be restricted to mucosae only, which may be severely affected.
Due to the higher level of split within the epidermis, the blisters
are flaccid, easily ruptured and often not seen intact. Erosions
are common and the Nikolsky sign is positive. The trunk is
usually affected. The condition is associated with significant
morbidity and mortality.

DR. ABDULQADIR M. MOALIM

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DR. ABDULQADIR M. MOALIM

DR. ABDULQADIR M. MOALIM
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DR. ABDULQADIR M. MOALIM

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DR. ABDULQADIR M. MOALIM

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DR. ABDULQADIR M. MOALIM

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Management

Pemphigus is more difficult to control than BP and

high-dose systemic corticosteroids are usually required.
Azathioprine, cyclophosphamide and intravenous
immunoglobulins may be used as steroid-sparing
agents. Often, long-term treatment is required to
prevent relapse.

DR. ABDULQADIR M. MOALIM