Akira Mamada

Xeroderma pigmentosum group D patient bearing lentigo maligna without neurological symptoms.

A 35-year-old Japanese female patient with xeroderma pigmentosum (XP), registered as XP114TO, was assigned to complementation group D by the cell fusion complementation test. The patient had manifested moderate solar sensitivity and freckles by the age of 6 years. The skin phototest using 290- and 300-nm monochromatic ultraviolet (UV) light revealed slightly lowered minimal erythema doses at 24 h after irradiation. The XP114TO skin fibroblasts exhibited about the 6-fold higher sensitivity to the lethal effect of 254-nm UV as did normal cells. Unscheduled DNA synthesis (UDS) induced in XP114TO cells by 254-nm UV (10 J/m2) was 33% of normal, falling into the group D range of 25-50% UDS. The patient developed lentigo maligna on the right side of the nose. Unlike the typical XP group D cases in the West, she showed no neurological abnormalities.

Beneficial clinical effects of cyclosporin A on severe psoriasis and its dissociation from serum concentration of soluble interleukin-2 receptor, soluble interleukin-6 receptor, soluble CD14 antigen, soluble intercellular adhesion molecule-1, and soluble vascular cell adhesion molecule-1

Psoriasis is a disease characterized by hyperproliferation of epidermal keratinocytes and infiltrates of mononuclear cells in the dermal epidermal junction. Several studies have suggested autoimmune mechanisms in the pathogenesis of psoriasis, and this view is confirmed by the fact that immunosuppressive therapies are beneficial in psoriasis. Activated T lymphocytes and keratinocytes produce several cytokines such as interleukin-1 (IL-1), IL-6, or tumor necrosis factor-α (TNF-α), which have been found to be elevated in the serum of psoriatic patients [1]. In psoriasis, trafficking of T cells into the skin is one of the main pathological events and precedes histological changes in epidermal cells [2]. The augmented expression of cell adhesion molecules, including intercellular adhesion molecule-1 (ICAM-1), E-selectin, and vascular cell adhesion molecule-1 (VCAM-1), has been reported in psoriatic lesions [1]. Soluble IL-2 receptor (sIL-2R) is shed from the cell membrane during T-cell activation [3]. CD14 is a newly discovered cell surface marker on monocytes that is shed after cell activation [4]. In inflamed skin, the expression of ICAM-1 on keratinocytes or peripheral blood monocytes has been found, and this adhesion molecule is also released [5, 6]. Soluble ICAM-1 (sICAM-1) plays a role in mediating cell to cell adhesion during inflammatory responses. Raised sIL-2R, soluble CD14 (sCD14), and sICAM-1 levels have been found in psoriatic patients [7–9]. In this study, the concentrations of soluble forms of several cytokine receptors and adhesion molecules were measured using an enzymelinked immunosorbent assay (ELISA) technique before cyclosporin A (CsA) therapy and after remission, and the association of the extent of psoriasis, as assessed by the psoriasis area and severity index (PASI), with the levels of these cytokines and adhesion molecules was also examined. A group of 16 patients with severe psoriasis vulgaris (all men, aged 26–71 years) were enrolled in this study. Psoriasis was clinically assessed using the PASI score. The PASI scores ranged from 13.2 to 53.8 (median 25.7). Before starting oral CsA (Sandimmune; Sandoz) therapy the patients provided consent, and they had not been treated with systemic prednisolone, retinoid or methotrexate. CsA was started at a dose of 2 mg/kg per day and gradually increased to 5 mg/kg per day until remission was induced, and then the CsA was gradually tapered. Blood samples were collected from the 16 patients before starting CsA and after remission, and the serum was stored at –20°C until use. The concentrations of sIL-2R (Cellfree IL-2R, Yamanouchi, Tokyo, Japan; normal 246–702 U/ml), sIL-6R (Biotrack IL-6SR, Amersham, UK; normal 12.5– 40.5 ng/ml), sICAM-1 (ICAM-1 ELISA kit, Bender Medical Systems, Vienna; normal 82.5–276 ng/ml), sVCAM-1 Toshiyuki Yamamoto · Kyoko Kimura · Ichiro Katayama · Taeko Koyano · Akira Mamada · Kazuhiro Miyazaki · Toshikatsu Irimajiri · Yoshiro Furuse · Kiyoshi Nishioka

Defective DNA Repair in Cultured Melanocytes from Xeroderma Pigmentosum Patients

The DNA repair of ultraviolet (UV)‐induced damages in primary cultured melanocytes from xeroderma pigmentosum (XP) patients and normal subjects were studied by measuring unscheduled DNA synthesis (UDS) on autoradiographs. Melanocytes were cultured in α‐minimum essential medium (α‐MEM) supplemented with 10% fetal calf serum (FCS), 12‐O‐tetradecanoyl‐phorbol‐13 acetate (TPA), and geneticin. The levels of UDS in XP melanocytes were compared with those in normal melanocytes.

Syringocystadenoma papilliferum arising on the thigh without connection to the overlying epidermis.

Syringocystadenoma papilliferum (SCAP) is a benign adnexal tumor originating from sweat glands. SCAP occurs most often in the head and neck region de novo or within the nevus sebaceous. Histologically, cystic invaginations lined by squamous, keratinizing cells extend downward from papillomatous epidermis. We present here a case of SCAP on the thigh, without connection to the overlying epidermis. A 46-year-old woman visited our hospital complaining of an asymptomatic intradermal nodule on her thigh. The duration from onset was unknown. A physical examination showed a firm nodule, sized nearly 1 cm, on the flexor aspect of her left thigh. It was fixed to the skin, and the surface was skin colored and smooth. The tumor was surgically removed. Histological examination revealed a cystic nodule localized in the middermis (Fig. 1a). The overlying epidermis was intact without epidermal acanthosis and was not connected to the tumor. Numerous papillary folds projected into the cystic spaces. The lumina were lined by a double layer of cells consisting of a luminal layer of tall, columnar cells that showed decapitation secretion and of an outer layer of small cuboidal cells (Fig. 1b). A number of plasma cell infiltrates were found in the connective tissue cores of the papillations (Fig. 1c). Furthermore, keratinous cyst walls lined by stratified epithelium were partially observed in connection with the apocrine cells that comprised the majority of the cystic structures (Fig. 1d). Unfortunately, serial sections were not obtained. This tumor was diagnosed to be an instance of SCAP. SCAP is known to usually occur in the head and neck and is rarely found in the lower limbs. In a series reported by Mammino and Vidmar, 75% of 145 occurrences were in the head and neck, 20% were in the trunk, and only 5% were in the extremities. Of occurrences in the lower limb, 70% were found

High Level of Serum Squamous Cell Carcinoma-Related Antigen in a Case of Bowen’s Disease

A case of Bowens disease on the lower abdomen is described. It was characterized by the unusually large size of the lesion and the high level of serum squamous cell carcinoma-related antigen which, however, decreased rapidly after surgical removal of the tumor.

Leukaemia cutis in chronic CD8+ T lymphocytic leukaemia

A 66-year-old woman with CD8+ chronic lymphocytic leukaemia developed multiple indurated erythema or plaques. A skin biopsy specimen showed dense infiltration of leukaemia cells in the dermis and subcutaneous tissue. Most of the infiltrating cells were CD8 + cells. To our best knowledge, leukaemia cutis such as this case showing plaques with diffuse infiltration of CD8 + cells has not been reported previously.