‘Suprabasal acantholytic’ blisters are seen in:
Correct Answer: Pemphigus vulgaris
Description: Ans. A. Pemphigus vulgaris. (Ref. Robbin's Pathologic Basis of Disease 8th/ Fig. 2530B).In a subcorneal blister, the stratum corneum forms the roof of the bulla (as in pemphigus foliaceus). In a suprabasal blister a portion of the epidermis, including the stratum corneum, forms the roof (as in pemphigus vulgaris). The suprabasal acantholytic blister that forms is characteristic of pemphigus vulgaris.The key microscopic features of pemphigus vulgaris:1. Suprabasilar blister with acantholysis which extends into the follicular epithelium2. Basal layer appeared ("tombstone")3. Rarely, eosinophilic spongiosis observed.4. DIF: IgG and possibly C3 deposited in the intercellular regions of the epidermis.Pemphigus vulgaris:Potentially fatal autoimmune skin disorder with IgG antibody against desmoglein (a component of desmosomes).Flaccid intraepidermal bullae caused by acantholysis (keratinocytes in stratum spinosum are connected by desmosomes);oral mucosa also involved.Immunofluorescence reveals antibodies around epidermal cells in a reticular (net-like) pattern.Nikolsky sign + (separation of epidermis upon manual stroking of skin).Pemphigus vulgaris. Note multiple crusty and weepy erythematous erosions where blisters have broken.Bullous pemphigoid: Less severe than pemphigus vulgaris. Involves IgG antibody against hemidesmosomes (epidermal basement membrane; antibodies are "bullow" the epidermis).Tense blisters containing eosinophils affect skin but spare oral mucosa.Immunofluorescence reveals linear pattern at epidermal-dermal junction.Nikolsky sign negative.Bullous pemphigoid. Note multiple intact, tense bullae.Potentially fatal autoimmune skin disorder with IgG antibody against desmoglein (a component of desmosomes).Flaccid intraepidermal bullae caused by acantholysis (keratinocytes in stratum spinosum are connected by desmosomes); oral mucosa also involved.Immunofluorescence reveals antibodies around epidermal cells in a reticular (net-like) pattern.Nikolsky sign? (separation of epidermis upon manual stroking of skin).Less severe than pemphigus vulgaris. Involves IgG antibody against hemidesmosomes (epidermal basement membrane; antibodies are "bullow" the epidermis).Tense blisters containing eosinophils affect skin but spare oral mucosa.Immunofluorescence reveals linear pattern at epidermal-dermal junction.Nikolsky sign?# In pemphigus vulgaris and pemphigus veggetans. acantholysis selectively involves the cells immediately above the basal cell layer, (suprabasal acantholytic blister).# The separation of bullous pemphigoid from pemphigus is based on the identification of subepidermal, nonacantholytic blisters, (associated basal cell Iyer vacuolization). Bullous pemphigoid feature linear deposition of immunoglobulin and complement in the basement membrane zone.#In pemphigus foliaceus. blister form in selectively involves the superficial epidermis at the level of stratum granulosum.# Paraneoplastic pemphigus occurs is an association with various malignancies, most commonly non-Hodgkin Lymphoma.Educational points:# Bullous phemphigoid, autoantibodies bind BPAG2, a component of the hemidesomes, leading to blister formation at the level of the lamina lucida of the basement membrane.# In pemphigus vulgaris, autoantibodies against Dsgl and Dsg3 cause blisters in the deep suprabasal epidermis,# In pemphigus foliaceus, the autoantibodies are against Dsgl alone, leading to superficial, subcorneal blisters.
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