PMC9950124_01
Clinical skin manifestation of the patient. (A) Multiple flaccid vesicles, pustules, and erosions on the left inframammary fold. Arrowhead: multiple flaccid vesicles and bullae. Arrow: a few pustules with the appearance of "half-half" blisters. (B) Erosion with erythema over the left supraclavicular area. (C) Multiple vesicles and erosions on the right inframammary fold. (D) Purpura and a hemorrhagic bulla on the right hand.
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Some bullae resembled "half-half" blisters ( Figures 1A-C ). Nikolsky signs, petechiae, and purpura were observed on her hands and lower legs ( Figure 1D ). No mucous membranes were involved.
Histopathology, DIF, and immunohistopathology of the skin biopsy sample. (A) Hematoxylin-eosin staining showed a subepidermal blister, with perivascular infiltration of inflammatory cells in the dermis (x40). (B) Leukocytoclastic vasculitis affecting small-to-medium-sized vessels were observed in the dermis (x100). (C) Leukocytoclasis forming nuclear dust and fibrinoid necrosis of the vessels (x200). (D) DIF revealed deposits of IgA in the intercellular space of the epidermis and BMZ (x400). DIF, direct immunofluorescence.
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The skin biopsy of the hemorrhagic bulla revealed a subepidermal blister and distinct leukocytoclastic vasculitis, primarily affecting small to medium-sized vessels in the dermis ( Figures 2A-C ). DIF revealed deposits of IgA autoantibodies in the BMZ and the intercellular space of the epidermis ( Figure 2D ).
Immunohistopathology of the skin biopsy specimen. (A) The monoclonal kappa-light chain was positive (x200). (B) The monoclonal lambda chain was negative (x200).
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Further laboratory investigations revealed monoclonal kappa-light chains in the dermis, as detected by immunohistopathology ( Figures 3A, B ); amyloids were not detected with Congo red staining (data not shown).