Seita Fukumaru

SUCCESSFUL TREATMENT OF SCLERODERMA WITH PUVA THERAPY

PUVA therapy was carried out on four patients with scleroderma; three of them had cutaneous manifestations of progressive systemic sclerosis and one other exhibited generalized morphea. PUVA therapy was given with daily doses of 0.25 J/cm2 or 0.4 J/cm2 for 3–8 weeks, resulting in total doses between 3.5 J/cm2 and 9.6 J/cm2. All four patients responded well to this treatment; improvements of hand closure, skin sclerosis index, and flexion of fingers or knee joints were obtained. Thus, PUVA appeared to be beneficial for treating scleroderma.

SLE with Death from Acute Massive Pulmonary Hemorrhage Caused by Disseminated Strongyloidiasis

We describe a case of disseminated strongyloidiasis involving a female patient with active systemic erythematosus (SLE). The cause of death was massive pulmonary hemorrhage induced by a filariform larvae infection. This was initially diagnosed during examination of the bronchoalveolar lavage fluid just 2 days before her death. The case indicated that immunosuppressed individuals would be paid attention to possible parasitic infection prior to starting therapy even in non-endemic areas as well as other microorganisms.

A Case of Adult T‐cell Leukemia/Lymphoma (ATLL) with Angiocentric and Angiodestructive Features

This report describes a case of adult T‐cell leukemia/lymphoma (ATLL) with angiocentric and angiodestructive features. The patient was a 66‐year‐old Japanese woman who began developing widespread skin lesions ten months prior to admission. The diagnosis of ATLL was made on the basis of her having an antibody to human T‐cell lymphotropic virus type ‐1 (HTLV‐1) and typical flower cells (ATLL cells) in peripheral blood smears. Once hospitalized, the course of her disease was very acute and severe, as is seen with angiocentric lymphoma. Based on histological features, this case was judged not to be angiocentric lymphoma; however, it may lie within the spectrum of angiocentric immunoproliferative lesions (AIL). The findings in this case strongly suggest that HTLV‐1 can be a pathogenic factor in the expression of angiocentric and angiodestructive features in ATLL, as is Epstein‐Barr virus (EBV) (1–4). To our knowledge, the present case is the sixth reported in the literature of lymphoma in which these features are associated with HTLV‐1 infection (5–7).

Multiple giant pilomatricoma

Dear Editor, Pilomatricoma (PM) is a benign adnexal tumor with differentiation toward hair matrix cells. It usually manifests as a firm, solitary lesion measuring 0.5–3.0 cm in diameter on the face and upper extremities. We report a 16-year-old girl with multiple giant PM. The patient was referred to Saiseikai Sendai Hospital for the evaluation of three large tumors on her back and right shoulder. She had first noticed the lesions 1 year earlier and they gradually enlarged. On physical examination, we noted two domeshaped hard tumors, 5 cm × 5 cm and 4 cm × 3 cm, on her back and a similar 7 cm × 6 cm tumor on her right shoulder. They were movable and slightly tender. The color of the overlying skin was moderately violaceous (Fig. 1). All the lesions were surgically excised. On histopathological examination, the tumors showed demarcation from the dermis and subcutaneous tissue; they were composed of eosinophilic shadow cells with basaloid cells and calcium deposits in some areas (Fig. 2). Based on these findings, a diagnosis of PM was made. She returned to our clinic 2 years after excision of the three tumors because seven new PM had appeared on her back and neck. Considering the association of multiple PM with systemic disorders such as myotonic dystrophy, Rubinstein–Taybi, Gardner, and Turner syndromes, we performed extensive neurological, mycological, gastrointestinal, hormonal and radiological examinations. No abnormalities were discovered. There was no family history of PM. Julian and Bowers, who reviewed 209 PM, reported that the size of these tumors ranged from 0.5–6.0 cm; most of them were between 1.0 and 1.5 cm. Our case is remarkable in that the lesions were much larger (7 cm × 6 cm, 5 cm × 5 cm and 4 × 3 cm) and that new lesions appeared after their resection. In their published work search, Jang et al., who presented two patients with multiple giant PM, found only one previously reported case. Our patient developed a total of 10 PM. An association of multiple PM with some systemic disorders has been reported. According to Chan et al., who

Novel mRNA molecules are induced in hypertrophied ventricles of carnitine-deficient mice and belong to a family of up-regulated gene in cells overexpressing c-erbB-2

To clarify the pathogenesis of cardiac hypertrophy in carnitine-deficient juvenile visceral steatosis (JVS) mice, we performed differential mRNA display analysis with the ventricles of control and JVS mice. We found a novel up-regulated gene, designated as carnitine deficiency-associated gene expressed in ventricle (CDV)-3. Northern blot analysis with a cDNA probe derived from the novel gene revealed two substantial mRNA species of prominent 4.1- and faint 3.5-kb in examined tissues of control and JVS mice. In spite of their widely expressed features, up-regulation of the gene was found predominantly in the ventricles and slightly in the auricles and skeletal muscles of JVS mice. The up-regulation of CDV-3 gene in the ventricles of JVS mice was significantly relieved by carnitine administration within 6 h. The entire cDNA nucleotide sequences showed that two kinds of cDNA, long and short versions (CDV-3A and -3B), corresponding to the detected mRNAs, are different in a 711 base fragment. Analysis of genomic DNA revealed that the two mRNAs were derived from a single CDV-3 gene with five exons by alternative splicing. The deduced amino acid sequences indicated that the isoforms consist of 236 and 281 residues, differing at regions near the carboxy-terminus but sharing 231 residues of the amino-terminal regions. A BLAST search revealed that they show a high similarity to a human predicted nuclear protein (H41), which has been reported to be up-regulated in breast cancer cells overexpressing cellular-erythroblastosis B-2 (c-erbB-2, a kind of tyrosine kinase).We report the identification and characterization of novel transcripts that may be involved in the development of cardiac hypertrophy caused by carnitine deficiency.

Case of dermatofibroma with monster cells: a review and an immunohistochemical study.

We report a dermatofibroma with monster cells. The patient was a 79-year-old woman who had a dark-brown nodule of her left leg for approximately 3 years. The lesion was composed of spindle-shaped fibroblastic cells, histiocytic cells, and multinucleated giant cells. Most of the histiocytic cells had foamy cytoplasm with numerous hemosiderin deposits. In addition to these cells, bizarre multinucleated cells with markedly hyperchromatic nuclei and xanthomatous cells with very large nuclei (monster cells) were also noted. No mitotic figures of the cellular components were present. This lesion has been shown to be completely benign despite the presence of pleomorphic or bizarre cells. From a clinical standpoint, recognition of a benign lesion of this type is very important since an incorrect histologic interpretation could result in inappropriate treatment.

A Case of Leiomyoma of the Nipple

76歳，男性。2001年2月頃より左乳頭部に時々，自発痛を感じていた。同年5月に近医外科及び皮膚科を受診し，左乳頭の肥大を指摘された。当科受診時には，左乳頭が右側に比較して明らかに肥大していた。腫瘍性病変は触知されなかったが，組織学的には，真皮内にエオジン好性の腫瘍巣を認めた。腫瘍細胞は紡錘形で，楕円形の核を有していた。アザン·マロリー染色，マッソン·トリクローム染色で赤染，エラスチカ·ワンギーソン染色で黄染し，α-SMAが陽性であり，平滑筋腫と診断した。乳頭の平滑筋より発生したと考えられた。乳頭·乳暈部に生じる平滑筋腫について，若干の文献的考察を加えて報告する。