A 40 year old male developed persistant oral ulcers followed by multiple flaccid bullae on trunk and extremities. Direct examination of a skin biopsy immunoflurescence showed intercellular IgG deposits in the epidermis. The most probable diagnosis is:
Correct Answer: Pemphigus vulgaris
Description: A i.e. Pemphigus Vulgaris- Pemphigus vulgaris is the commonest and pemphigus vegetans is the rarest variety.In pemphigus vulgaris (an auto immune disease), autoantibodies (IgG) are directed against intercellular substance desmogleins (Dsgs)Q, a transmembrane desmosomal glycoprotein that belong to cadherin supergene family of calcium dependent adhesion molecules causing loss of cohesion (acantholysis)Q between epidermal cells of deep stratum basal layer Q. That is why, histology (tzanck smear) show (intra) epidermal acantholytic bullae (blister) in deep supra basal layer of epidermisQ along with single layer of intact stratum basale cells attached to basement membrane (row of tomb stone appearance) that form the base of blister. Indirect immuno fluorescence microscopy demonstrate circulating autoantibodies against keratinocyte cell surface antigen whereas diagnostic (gold standard) direct immunofluorescence microscopy (DIM) show deposits of IgG on the surface keratinocyte in fishnet like patternQ and in intercellular space (causing it to widen).Pemphigus vulgaris presents as muco-cutaneous lesions (blister or erosions) predominantly in middle aged patients (> 40 year age). It typically begins on mucosal surface and nearly all patients have mucosal involvement (oral in 70% Q >> ocular, nasal, pharyngeal, larynx, oesophagus, urethra, vulva, cervix). PVoften progresses to widespread skin involvement with a predilection for scalp, face, neck, axillae, groin, trunk Q and pressure points.PV is characterized by painful, mostly non pruritic, fragile flaccid blistersQ filled with clear fluid on a normal or erythematous base that ruptures to produce extensive denudation of mucous membranes andskin resulting in ill defined irregular shaped erosions. Healing occurs without scarring but with temporary hyperpigmentation.
Category:
Skin
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