30 year old male had severely itchy papula vesicular lesions on extremities, knees, elbows and buttocks for one year. Direct immunofluorescence staining of the lesions showed IgA deposition at dermoepidermal junction. The most probable diagnosis is
A Pemphigus vulgaris
B. Bullous pemphigoid
C. Dermatitis herpetiformis
D. Nummular eczema
Ans.
C. Dermatitis herpetiformis.
Dermatitis herpetiformis - manifested
by pruritic papules, vesicles, and papulovesicles mainly on the elbows, knees
buttocks, posterior neck, and scalp
Associated with HLA-B8 -DR 3 and DQ-2
Diagnosis
1. Neutrophils at the dermal papillary
tips
2. Granular deposition of IgA along the
dermal papillae
3. Circulating anti endomysium
antibodies and antibodies to tissue transglutaminase are present in 70% cases
Pemphigus vulgaris- Deposition of IgG
and C3 on the surface of keratinocytes against desmoglein. intercellular
Bullous pemphigoid - Linear band of IgG
and/or C3 in epidermal basement cone.
Patients have gluten sensitive
enteropathy
Patients with dermatitis herpetiformis
are at increased risk of development of GI-lymphoma
Treatment - Gluten free diet, Dapsone*
DIRECT IMMUNOFLUORESCENCE FINDINGS IN AUTOIMMUNE BLISTERING DISEASE.
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|
Disease
|
Pattern and nature of immunoreactants
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Pemphigus
|
Epidermal cell-surface
deposits of IgG
and C3
|
Bullous
pemphigoid
|
Linear, homogeneous
deposits of IgG
epidermal side on salt-split
skin. and
C3 at the dermal-epidermal junction.
|
Linear IgA dermatosis
|
Linear homozeneousdepositsof IgA at
the dermal-epidermal iunction
|
Herpes gestationis
|
Linear
homogeneous.depositsof
C3 at the dermal-epidermal
junction
|
Epidermolysis bullosaacquisita
|
Linear
homogeneous deposits of IgG
dermal side on salt-split skin. and C3 at
the dermal- epidermal
iunction.
|
Cicatricial
pemphigoid
|
Linear
homogeneous deposits of IgG
and C3 at the dermal-epidermal
junction
|
Dermatitis
herpetiformis
|
Focal granular
deposits of IgA at the
papillary tips
|
Bullous eruption
of lupus erythematosus
|
Linear homogeneous,
or non-homogeneous, deposits
of multiple immunoglobulins,
C3 and fibrin at the dermal-epidermal
junction.
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