Noriyuki Hirashima

A case of herpes zoster in a child with congenital insensitivity to pain with anhidrosis

HHV-6 reactivation, regardless of whether this is a causal factor or a consequence of disease. Unfortunately, however, Peyrière et al. did not specify this point in their patients, raising the possibility that they may have studied a very heterogeneous group of patients, presenting as a continuum from mild papulomacular rashes to full-blown DIHS. This syndrome has several unique features that cannot be explained solely by a drug-based aetiology: they include delayed onset in relation to introduction of the causative drug and paradoxical worsening of clinical symptoms after discontinuation of the causative drug. A major difficulty in establishing a correlation between causative drugs and the onset of this syndrome is such a long lag period before onset of clinical symptoms. However, large series of patients from Japan revealed that the drugs responsible for the development of DIHS are limited to eight drugs in the vast majority of patients: they include carbamazepine, phenytoin, phenobarbital, zonisamide, mexiletine, dapsone, sulfasalazine and allopurinol. Atypical cases caused by other drugs, although reported, are much less common. The lack of a longitudinal study including viral load evaluation in the authors’ study may have made the unique clinical entity of DIHS uncertain. Once the suspicion of DIHS arises on the basis of initial history-taking and clinical presentations, a thorough investigation of viral reactivations should follow. As the recognition of this syndrome as a distinct clinical entity with highly reproducible clinical and laboratory features increases, it becomes clear that DIHS has potential long-term complications, such as type 1 diabetes mellitus, even after disease-free intervals of months or years. The diagnosis is unlikely to be missed if the possibility of this syndrome is considered in the differential diagnosis of any patients with fever, rash, lymphadenopathy and hepatitis, and if HHV-6 IgG titres are routinely examined at the right time. HHV-6 reactivation would be the diagnostic marker for DIHS that is reliable and easy to determine on a routine basis. The incidence of this syndrome is much greater than previously thought. If this unique disease is viewed only as a reaction pattern and a search for viral reactivations is not made, the disease may remain idiopathic as it was in the past.

Atrophic dermatofibrosarcoma protuberans with diffuse eosinophilic infiltrate

Atrophic dermatofibrosarcoma protuberans (atrophic DFSP) is a variant of dermatofibrosarcoma protuberans (DFSP), and is clinically characterized by depressed lesions. We report a patient with a typical atrophic DFSP lesion with marked eosinophilic infiltration. The patient was a 55‐year‐old woman with a dark‐red, depressed lesion in the epigastric region. Histopathological examination of the lesion showed proliferation of fibroblast‐like cells in a storiform pattern in the dermis and subcutaneous tissue. Immunohistochemical staining of tumor cells was positive for CD34. The lesion was histopathologically typical of DFSP, but no elevated lesion was clinically observed. Thus, a diagnosis of atrophic DFSP was made. Moreover, this tumor tissue exhibited marked eosinophilic infiltration. To our knowledge, they are no reports of eosinophilic infiltration in DFSP tissue. Therefore, this seems to be an extremely rare case of DFSP.

Extramedullary plasmacytoma of the testis which formed cutaneous tumors

78歳男性。1992年右陰嚢腫大が出現し, 9年後に腹部, 左側胸部に腫瘤が出現した。さらに翌年, 左眼瞼下垂が出現し, CT上頭蓋内に病変を認めたため入院となった。腹部と左側胸部に紫紅色, 弾性硬の腫瘤を, 眼所見では両側眼瞼下垂と左動眼神経麻痺を認めた。血液検査では総蛋白の増加, 蛋自分画でM-蛋自陽性だった。骨髄穿刺所見で異常はなかった。病理組織所見では真皮上層から皮下組織にかけてびまん性に細胞浸潤を認めた。腫瘍細胞は軽度異型性を示し, 車軸様の核を有するなど形質細胞の特徴を有していた。免疫組織学的に腫瘍細胞は形質細胞マーカーに陽性を示した。遺伝子再構成は免疫グロブリンJHで認めた。精巣腫瘤も同様の所見を示したことから精巣原発の髄外性形質細胞腫と診断した。治療は放射線療法と化学療法を併用し, 一時縮小したが, 9ヵ月後に腫瘤が再び増大, 背部や膝窩に多発してきたため, 外科的切除と化学療法を併用した。