山田 朋子

A 49‐year‐old‐Japanese woman visited us with a 1‐year history of verrucous lesions on the oral mucosa and fingers. Her past medical history was unremarkable except for chilblain in winter. She had no family history of collagen disease. Physical examination showed a well‐defined, hyperkeratotic erythematous plaque with ulceration on the palate and verrucous nodules on the fingers (Fig. 1a,b). She had no butterfly rash or discoid lesions on any other sites. Histology from both oral mucosa and finger revealed similar features; marked hyperkeratosis and acanthosis with perivascular lymphocytic infiltrates in the dermis. Liquefaction degeneration with a few necrotic keratinocytes was also found (Fig. 1c,d). We regarded the patient as verrucous lupus erythematosus (LE). Then, leukopenia (2,820/μl) and thrombocytopenia (67,000/μl) were observed as well as positive antinuclear antibody (ANA). However, the diagnosis of systemic lupus erythematosus (SLE) was not confirmed by the negative finding of anti‐DNA antibody, and the lack of requirements in 1997 updated ACR criteria for SLE validated back at that time. She developed skin ulcers on the left lower leg in the next two months (Fig. 2a). Four months later, laboratory examination revealed leukopenia (2,850/μl), thrombocytopenia (89,000/μl), low C3/C4 level (27 mg/dl, 3 mg/dl, respectively), positivity for ANA (1 : 640, speckled type), and anti‐Sm antibody (1 : 2). Histology of the leg ulcer exhibited no vasculitis but the occlusion of the vessels (Fig. 2b). Direct immunofluorescence revealed IgM, C1q, and C3 depositions on vessel walls as well as linear IgM deposition at basement membrane zone (Fig. 2c). Seven months later, she had seizure attacks. Then, SLE was diagnosed based upon ACR (1997) and SLICC criteria. Even under 2019 EULAR/ACR classification criteria,1 she was classified as SLE from total 24 points scored by the findings of fever (38.9 °C), leukopenia, thrombocytopenia, seizure, low C3/C4, and anti‐Sm antibody. Then, the patient was transported to us for dyspnea and high fever. Methylprednisolone pulse therapy was performed. Chest roentgenogram revealed extensive lung infiltrates. Aspergillus fumigatus was isolated from the sputum. Invasive aspergillosis was diagnosed. She died of multiple organ failure 8 months after the first visit.