Toshikazu Usuda

Clinical and immunologic predictors of scleroderma renal crisis in Japanese systemic sclerosis patients with anti-RNA polymerase III autoantibodies.

To identify predictive factors for scleroderma renal crisis (SRC) in patients with anti–RNA polymerase III (anti–RNAP III) antibodies.

Five cases of necrotizing fasciitis: Lack of skin inflammatory signs as a clinical clue for the fulminant type

Herein, we describe five patients with necrotizing fasciitis (NF) who had variable outcomes and clinical manifestations. At the onset, all patients exhibited purpura with or without blister and ulceration accompanied by severe pain and tenderness in the affected skin. Out of five patients, three lacked inflammatory signs such as redness and heat, and two of the three patients showed fulminant progression and died despite intensive treatments including surgical debridement, antimicrobial therapy, close monitoring and physiological support. Tissue specimens from the patients without skin inflammatory signs showed mild neutrophil infiltration in addition to necrosis from the epidermis to subcutaneous fat, and variable amounts of thrombi. Furthermore, numerous bacteria were detected by Gram stain. By contrast, the remaining two patients with skin inflammatory signs revealed slower progression, and tissue specimens from both patients showed heavy neutrophil infiltration, but bacteria were hardly detected. Therefore, these cases suggest the possibility that the paucity of skin inflammatory signs, such as redness and heat, in NF may be a clinical clue to predict the fulminant type.

Surgical treatment of speckled skin caused by dyschromatosis symmetrica hereditaria--case report.

Thin split skin autografts taken from the abdomen were used to replace speckled skin caused by dyschromatosis symmetrica hereditaria in one patient. Normal skin colour appeared in the recipient areas and no recurrence was observed in the treated areas.

Immunogenetic Study of Three Japanese Families with Neonatal Lupus Erythematosus

We encountered three Japanese families with neonatal lupus erythematosus. None of the three fathers showed any signs of collagen disease. The three mothers were found to suffer from Sjögrens syndrome; they all tested positive for anti‐SSA and SSB antibodies and had lymphocyte infiltration into the small salivary gland. In two families, one child had neonatal lupus erythematosus while a sibling was normal; in the third family, both children had neonatal lupus erythematosus. Thus, a mother with positive anti‐SSA and SSB antibodies can give birth to one infant with and one infant without or have two infants with neonatal lupus erythematosus. We conducted HLA typing of all 12 members of the three families in order to clarify the immunologic factors involved. We found no increased frequency of any HLA phenotype in the three mothers and their four children with neonatal lupus erythematosus; however, HLA‐DR4 was present in three of the children with neonatal lupus erythematosus.

Case of congenital dermatofibrosarcoma protuberans: Change of clinical features and pathological findings over 10 years

infiltrations of atypical large lymphocytes with round nuclei and a few nucleoli throughout the dermis (Fig. 1b–d). Slight infiltration of histiocytes and mild lymphocytic vasculitis of small vessels were also seen. Atypical large lymphocytes expressed CD20 (Fig. 1e) and histiocytes expressed CD68 (Fig. 1f). No cells were positive for latent membrane protein 1 or EBNA2. In situ hybridization (ISH) for EBV disclosed EBER expression (Fig. 1g). Computed tomography revealed multiple faint macular shadows in the lungs (Fig. 1h), together with multiple subcutaneous and intramuscular lesions on the whole body, and bilateral axillary lymph node swelling. The diagnosis of grade III LYG was made. We retrospectively studied a biopsy taken from the skin lesion 12 years prior and found band-like lymphocytic infiltration with a few large CD20-positive atypical cells in the upper dermis, which were consonant with grade I LYG (Fig. 1i, j), although ISH for EBV did not disclose EBER expression. The same monoclonality of the IgVH gene was demonstrated in DNA samples prepared from both biopsy specimens (Fig. 1k). Oral prednisolone at 0.5 mg/kg per day remarkably improved both skin and pulmonary lesions over 1 month. Dry cough also disappeared. We ceased oral prednisolone 2 months later, without exacerbations of any symptoms. In this study, we described an elderly patient of LYG with long clinical course. The results of the first skin biopsy, which was consonant with grade I LYG, and subsequent continuous skin symptoms suggested that LYG gradually progressed over 12 years. Although grade III LYG usually requires aggressive chemotherapy, monotherapy of low-dose oral prednisolone was selected in our case, because of the long clinical course and old age. The favorable outcome in our patient may signify that low-dose prednisolone monotherapy can be another option for grade III old LYG patients with indolent course. ACKNOWLEDGMENTS: We gratefully appreciate the secretarial work of Ms Tomoko Tashima and Ms Mami Nishida. We thank the patient for his participation.

A case of giant pilomatrix carcinoma

症例は58歳男性。既往歴に慢性B型肝炎あり。1996年頃から右臀部に小結節が出現し2003年頃から急速に増大して出血, 疼痛を伴うようになったため, 2005年3月15日当院を受診した。初診時, 右臀部にドーム状に隆起する14×13×6cm大の骨様硬の巨大腫瘤を認め, 中央は自潰していた。病理組織所見では, 腫瘍はbasaloid cell, shadow cell, およびsquamoid cellで構成されており, 大小不同で不整形な腫瘍巣の集合が不規則に間質内に浸潤していた。basaloid cellには核異型があり, 核分裂像も多く認められた。以上の臨床, 病理組織所見よりpilomatrix carcinomaと診断した。現在術後約2年3ヵ月経過し, 再発転移は認められていない。

Squamous Cell Carcinoma Arising within Seborrheic Keratosis.

Seborrheic keratosis (SK) is common benign skin tumor. But some authors reported malignant transformation of SK.The patient is a 72-year-old man who had noticed a small black nodule on right lower abdomen for 30 years. The nodule was getting larger slowly, but it becomes ulcerative and elevates recently.Histopathologically, this tumor was revieled a deeply invasive squamous cell carcinoma that had developed from the base of a SK. [Skin Cancer (Japan) 2000; 15: 152-154]