case_images: PMC11719246_01
case_images: A-D, Clinical features at the first visit. A 65-year-old woman presented with numerous pustule formations and annular erythematous lesions that had appeared from her trunk and spread to the whole body. Numerous well-defined red annular plaques ranging from 5 to 30 mm in diameter are on her head, face, neck, and forearms. Pustules were fragile and clusters were detected in the neck and head.
case_images: gr1.jpg
case_images: PMC11719246_01_gr1.jpg
case_images: fig1
case_images: Fragile pustules and clusters were detected in the neck and head (Fig 1, A-D).
case_images: A skin biopsy from erythema including pustules on upper portion of the arms showed subcorneal pustule formation, spongiotic changes, and vacuolar degeneration were present in the epidermis. In the dermis, a mild to moderate perivascular inflammatory cell infiltrate with mixed lymphocytes and neutrophils was observed in the predominant perivascular zone in the papillary dermis. (A, B, Hematoxylin-eosin stain.)
case_images: gr2.jpg
case_images: PMC11719246_01_gr2.jpg
case_images: fig2
case_images: Clinical features a week after starting intravenous spesolimab. A-D, Systemic symptoms, pustule, and erythema showed marked improvement within 10 days.
case_images: gr3.jpg
case_images: PMC11719246_01_gr3.jpg
case_images: fig3
case_images: Low-grade fever and general fatigue, pustule, and erythema markedly improved (day 10, Fig 3, A-D).
case_images: Direct immunofluorescence of the skin demonstrated deposits of IgG and C3 in the upper layer of the epidermis but not of IgM or IgA in intercellular spaces of the epithelia (A). Western blotting analysis was performed using epidermal extracts as substrates. Patient serum was diluted 20-fold, and a 100-fold dilution of antipatient serum was used as a secondary antibody. A 160 kb band corresponding to desmoglein 1 was observed. Lane 1 was negative control, lane 2 was serum from a patient with bullous pemphigoid, and lane 3 was serum from a patient with pemphigus vulgaris (B).
case_images: gr4.jpg
case_images: PMC11719246_01_gr4.jpg
case_images: fig4
case_images: IgG and C3 deposition, but not of IgM or IgA in the upper layers of the epidermis (Fig 4, A). Indirect fluorescence using normal human skin detected total IgG (titer 1/160) autoantibodies against intracellular spaces. Western blotting analysis using epidermal extract as substrate showed the upbeat band at 160 kb for the patient's plasma (Fig 4, B).