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.

Bullous pemphigoid (BP) is a common autoimmune blistering disorder with unknown etiology. Recently, increasing numbers of BP cases which developed under the medication with dipeptidyl peptidase-4 inhibitors (DPP4i), widely used antihyperglycemic drugs, have been reported in published works. Here, we report a case of DPP4i (teneligliptin)-associated BP that developed in a 70-year-old Japanese man. Interestingly, the patient had acquired reactive perforating collagenosis (ARPC), which is also known to be associated with the onset of BP. In the present case, clinical, histopathological and immunological findings suggested that DPP4i rather than ARPC was associated with the onset of BP.

BACKGROUND: Rosacea is a common skin disease and predominantly affects on the face of middle-aged women. It exceptionally occurs on the extrafacial areas such as ear, neck, axilla, and upper extremities, and has been reported as disseminated rosacea. MAIN OBSERVATION: A 40-year-old Japanese female presented with one-month history of erythematous skin eruption with burning sensation on the face, neck, and upper limbs. Physical examination showed rosacea-like eruption on the face as well as multiple papules disseminated on the neck, forearms, and hands. These extrafacial lesions demonstrated papulonecrotic appearance. Bilateral conjunctiva showed marked hyperemic which was consistent with ocular rosacea. Corneal opacity was also seen. Histology of the umbilicated papule on the neck revealed necrobiotic granulomas around the hair follicle with transepidermal elimination. Another tiny solid papule on the forearm suggesting early lesion also demonstrated necrobiosis with palisading granuloma but no transepidermal elimination. Systemic administration of minocycline and topical tacrolimus therapy promptly improved the skin lesions. Topical application of fluorometholone in temporary addition with levofloxacin improved ocular involvement 12 weeks after her 1st visit. The clinical course of the skin lesion and ocular symptoms mostly correlated. Then, the skin lesion and ocular symptoms often relapsed. Rosacea uncommonly associates with the extrafacial involvement as disseminated rosacea. The present case is characterized by the disseminated papulonecrotic lesions of the extrafacial areas histologically showing transepidermal elimination of necrobiotic granulomas. CONCLUSIONS: Dermatologists should recognize that papulonecrotic lesions of the neck and upper extremities might be extrafacial rosacea when the patient has rosacea on the face.

69歳, 女性。10歳頃, いろりに転落し, 頭部に熱傷受傷し広範な脱毛瘢痕形成したが, 鬘を使用し家族にさえ隠していた。7年前から, 潰瘍が出現し軽快しなかったが, 突然の意識障害および痙攣発作で当院に緊急入院するまで放置。入院後, 頭部の腫瘤を発見された。頭頂部を覆う, 径約13cmの巨大な噴火口状の腫瘍で, 悪臭, 壊死組織を伴い易出血性で, 中央が拍動性に上下に動いていた。生検で有棘細胞癌と診断。リンパ節転移や遠隔臓器への転移は認められなかったが, 頭部X線やMRI検査により, 骨融解, 硬膜浸潤がみられ, 上矢状静脈洞圧排狭窄があったことから, 根治的な切除不能と診断し, 髄膜炎や痙攣発作の軽減を目的に放射線療法を行った。腫瘤の縮小, 臨床症状の軽快がみられ退院可能となったが, 初診から1年4ヵ月後, 死亡した。

Spontaneous regression of lentigo maligna melanoma (LMM) is well known to occur occasionally. We experienced a possible cass of LMM located from internal canthus to lower eyelid and a red-bean sized black-brown nodule at adjacent bulbar conjunctiva was also present. The apparent continuity was not found between two lesions. In addition, histological findings suggestive of spontaneous regression were not recognized. This discontinuity of two lesions is most possibly explained by spontaneous regression of LMM, although we did not prove clinical and histological continuity. The other possible explanations are that two lesions occurred separately, or that malignant melanoma of bulbar conjunctiva was due to intransit metastasis.