We report a case of Sjögren syndrome that developed pernio-like acral erythema and acrocyanosis on the soles induced by PD-1 immune checkpoint inhibitor. A 76-year-old male received pembrolizumab therapy for his throat cancer. Six months later, he developed pernio-like acral erythema, swollen fingers, acrocyanosis on the soles, and plantar numbness. Biopsy taken from the finger revealed dermal sclerosis and an increased number of blood vessels in the upper dermis, as well as moderate mononuclear cell infiltration around the appendages in the mid-dermis. Another biopsy taken from the forearm also revealed dermal sclerosis. No systemic sclerosis-specific autoantibodies were detected in the serum. After treatment with pembrolizumab, he noticed oral sicca, and serum anti-SS-A and anti-SS-B antibodies were positive. Biopsy of the minor salivary gland revealed mononuclear cell infiltration around the duct in the interstitium. Although the patient did not have dry eye, we concluded that Sjögren syndrome was also induced by PD-1 inhibitor.

We here report a 58-year-old man who presented at our department with a one-month history of sore throat, palpable purpura on both lower limbs and lower limb weakness. Anterior rhinoscopy performed in the otorhinolaryngology department revealed mucosal redness and granuloma-like structures. In addition, CT showed shadows in both maxillary and ethmoid sinuses, as well as multiple pulmonary nodules. Blood tests were positive for PR3-ANCA. The purpura appeared on both lower limbs 3 days before the patient's first visit to our department. Skin biopsy revealed leukocytoclastic vasculitis. Biopsy of the nasal mucosa revealed granulomatous inflammation containing multinucleated giant cells. The case was diagnosed as granulomatosis with polyangiitis. This is one representative case included in our summary report of all the cases of granulomatosis with polyangiitis in Japan, which examines the relationship between skin eruptions, histopathology, and ANCA.

We conducted a survey of Japanese dermatologists and patients with prurigo nodularis (PN) to identify potential needs in the treatment of PN. When many dermatologists and patients were asked about the impact of PN on patients' lives and daily activities, they pointed to physical symptoms. When asked specific questions rather than abstract questions about mental symptoms and limitation of daily activities, patients were more likely to answer that their lives and daily activities were affected by PN; physicians showed the opposite tendency. Questioning patients with PN about more specific situations may help to uncover their potential treatment needs, leading dermatologists to set up more treatment goals for each patient.