PMC9773387_01
Clinical and immunological findings of bullous pemphigoid patient. (A) Tense bullae in the upper limbs and the right hand; (B) linear deposition of IgG along the dermo-epidermal junction by direct immunofluorescence; (C) IgG staining of the epidermal side (white arrows) of the artificial blister by indirect immunofluorescence on human salt-split skin; and (D) patient with BP in complete remission on minimal therapy.
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F1
On physical examination, the patient showed excoriations and tense bullae on an inflammatory basis in the upper limbs, lower limbs, trunk, and abdomen and a lesion in the nasal mucosa, with a bullous pemphigoid disease area index (BPDAI) score of 60 (Figure 1A). DIF was positive with linear deposition of IgG and C3 along the dermo-epidermal junction (Figure 1B). BP180 and BP230 ELISAs. Both the immunological assays resulted in positive with IgG staining of the epidermal side of the artificial blister by IIF (Figure 1C) and IgG titers of 36.4 and 44.1 U/ml against BP180 and BP230, respectively (negative <9 U/ml for both BP180 and BP230 ELISA) (MBL, Nagoya, Japan). After 2 months, she reached remission on minimal therapy of 10 mg/day of prednisone, and a BPDAI of 10 was only obtained with damage evaluation (Figure 1D).
Timeline with relevant data about the onset, diagnosis, and therapy of bullous pemphigoid patient.
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F2
The patient reports that bullous lesions started in October 2021 in a milder form, and she initially went to a private practice dermatologist who set therapy with betamethasone 5 mg/day (Figure 2). Medical history has revealed that the patient is also affected by MS, diagnosed in 1988 following 14 episodes of optic neuritis: magnetic resonance imaging and lumbar puncture showing oligoclonal bands confirmed the diagnosis (Figure 2). Capillaroscopy revealed vascular anomalies, such as mega capillaries and avascular areas (Figure 2).