視神経乳頭腫脹と乳頭周囲の線状出血を認めたWeber-Christian病の1例

Papilledema and peripapillary splinter hemorrhage in a case of Weber-Christian disease

要約　目的：視神経乳頭腫脹と乳頭周囲の線状出血を認めたWeber-Christian病の1例を報告する。

症例：38歳，女性。発熱および両下肢の熱感と圧痛を伴う結節性紅斑で皮膚科を受診した。生検の結果Weber-Christian病と診断された。眼科的な自覚症状はなかったが，過去の文献より眼病変の合併も懸念され当科を受診となった。

所見と経過：矯正視力は両眼とも1.2であった。両眼に視神経乳頭腫脹，乳頭周囲の線状出血を認めた。光干渉断層計では視神経乳頭は腫脹し，硝子体側へと突出していた。眼科的自覚症状はなく，主科での抗菌薬治療にて皮膚所見も改善傾向にあったため経過観察としたが，本症は再燃を繰り返すこともあり，皮膚所見だけでなく眼所見も残存していることからインフリキシマブが投与された。皮膚所見は著明に改善，眼所見も徐々に改善を認め，以降寛解に至った。

結論：Weber-Christian病は稀であるが，本症例のように眼病変を合併することがあり，自覚症状がなくても眼科的検査を行うことは重要である。

Abstract　Purpose：To report a case of Weber-Christian disease with papilledema and peripapillary splinter hemorrhage.

Case：A 38-year-old female visited a dermatologist for fever and erythema nodosum in both lower limbs. Skin biopsy led to the diagnosis of Weber-Christian disease. Although she had no subjective ophthalmic symptoms, she was referred to our department because some Weber-Christian disease cases have ocular complications.

Findings and Clinical Course：Corrected visual acuity was 1.2 for both eyes. Papilledema and linear hemorrhage around the papillary were observed in both eyes. On optical coherence tomography, the optic disc was swollen and protruded toward the vitreous body. There were no subjective ophthalmic symptoms, and the skin findings were improved with antibiotic, thus we followed the patient up at first. However, this disease can sometimes recur, and both skin and ocular findings persisted. Therefore, infliximab was administered. The skin findings improved markedly, and the ocular findings gradually improved, leading to remission.

Conclusion：Weber-Christian disease is very rare, but eye lesions may occur, as in this case. It is important to perform ophthalmologic examination even in cases without subjective symptoms.