Tatsuyoshi Arao

Triggering of the vascular permeability reaction by activation of the Hageman factor-prekallikrein system by house dust mite proteinase

A 30-kilodalton (kDa) proteinase from the house dust mite Dermatophagoides farinae (Df-proteinase) was recently purified (Takahashi et al. (1990) Int. Arch. Allergy Appl. Immunol. 91, 80-85). In this paper we detailed the biological activities of the Df-proteinase. The activation of the kinin cascade by Df-proteinase was examined in vitro by using purified guinea pig Hageman factor (HF), prekallikrein (PK) and high-molecular-weight kininogen (HMWK) and the effect of this proteinase on endogenous human plasma proteinase inhibitors (serpins) and alpha 2-macroglobulin was tested. In addition, enhancement of the vascular permeability reaction in guinea pig skin by Df-proteinase was examined in vivo. These experiments showed that Df-proteinase could activate all the steps of the kinin-generating cascade, i.e., HF, PK and HMWK, and that Df-proteinase retained proteolytic activity even in the presence of an excess amount of endogenous proteinase inhibitors in plasma. We also found that the marked enhancement of the vascular permeability reaction was induced by Df-proteinase via the activation of the kinin-generating cascade without the release of histamine. From these results, we conclude that the proteinase of the house dust mite, Df-proteinase, has the potential to generate bradykinin and that the presence of this proteinase in biological systems would exacerbate inflammatory reactions in some pathological conditions.

Structure of the mouse C-reactive protein gene.

A genomic DNA clone corresponding to the mouse C-reactive protein (CRP) has been isolated and characterized. The mouse CRP gene is 1.9-kilobase pairs in length and contains a single intron of 213-base pairs which interrupts the codon for the 2nd amino acid residue of the mature CRP protein. We compared nucleotide sequences of the mouse and human CRP genes and discussed structures of possible regulatory sequences. With this characterization, the isolation and sequence analyses of a set of mouse and human pentraxin genes, i.e. CRP and serum amyloid P component genes is not complete.

Malignant schwannoma associated with xeroderma pigmentosum in a patient belonging to complementation group D

A 43-year-old man with xeroderma pigmentosum, XP97TO, was allocated to complementation group D. He had had moderate photosensitivity at age 1 year and freckles by age 6 but no neurologic abnormalities. Nevertheless, his fibroblasts in culture had the XP-D phenotype. They showed a sevenfold hypersensitivity to killing by 254 nm ultraviolet radiation and a diminished level (29%) of unscheduled DNA synthesis. Phototesting revealed delayed maximum erythema at 72 hours after UVB exposure and a lowered minimal erythema dose. Lentigo maligna developed on the patients face, and a rapidly growing malignant schwannoma was found on the left trigeminal nerve. This may be the first case of a peripheral nervous tissue neoplasm in xeroderma pigmentosum.

Complicating systemic amyloidosis in dystrophic epidermolysis bullosa, recessive type

&NA; An autopsy case of dystrophic epidermolysis bullosa, recessive type, complicated by systemic secondary amyloidosis is described. The patient had developed multiple bullous lesions and erosions from birth, followed by repeated infection. At autopsy, chronic persistent inflammation was observed in the skin and in various visceral organs, accompanied by systemic amyloidosis. By the peroxidase‐antiperoxidase (PAP) method, amyloid deposits stained positively for anti‐AA‐ protein antiserum. In the present case, we concluded that the systemic amyloidosis was of the AA type, and developed secondarily to the chronic persistent inflammation in the prolonged course of dystrophic epidermolysis bullosa, recessive type.

A Case of Lymphocutaneous Nocardiosis with a Review of Lymphocutaneous Nocardiosis Reported in Japan

An 82‐year‐old Japanese male developed nodules and ulcers along the lymphatics after a fall in the garden of his house resulting in injuries to the dorsum of his left hand which lasted for 3 months. Nocardia brasiliensis was isolated from a nodule, supporting a diagnosis of the lymphocutaneous type of nocardiosis. He had previously developed generalized bone metastasis from prostatic cancer, and his resulting depressed immunity might have played a part in the nocardiosis genesis.

SPONTANEOUS GANGRENE OF THE SCROTUM AND PENIS (FOURNIER'S GANGRENE)

Two Japanese males developed spontaneous gangrene of the scrotum and penis. Antibiotics were given and surgical treatments were performed successfully. The latter consisted of debridement of the wound, local flaps and free skin grafts. We failed to obtain significant bacterial cultures. Histology revealed thrombosed and thickened small vessels in the reticular dermis near the border of the gangrenous scrotal tissue.

Pigmented Basal Cell Carcinoma Developing on the Lower Extremities: Three Cases Masquerading as Malignant Melanoma

Three cases of pigmented basal cell carcinoma were reported which needed to be differentiated from malignant melanoma based on their location and clinical features. Clinicopathologically, they were characterized by a large number of melanophages in the stroma, blockade melanocytes in the parenchyma, and phagocytosis of melanosomes in tumor cells as well as a large number of intraparenchymal melanophages. Two cases were characterized by the aggregation of melanophages in cystic spaces caused by stellate atrophy.

Immunohistological detection of human malignant melanoma using monoclonal antibody to a melanoma-associated antigen

In 1975, Kohler and Milstein first succeeded in producing monoclonal antibodies [7]. Since then, many monoclonal antibodies, have been developed and used not only in basic research but also in clinical medicine. In the field of oncology, monoclonal-antibody technology was introduced primarily in order to detect tumor-specific or tumor-associated antigens. In particular, the melanoma-associated antigen has received considerable attention with regard to the early detection of malignant change. Imai et al. have prepared several monoclonal antibodies to human malignant melanoma and found that the monoclonal antibody, 225.28 S, reacts with cultured human melanoma but does not react with other cultured carcinoma or lymphoid cells [4-6]. In the present study, we examined the tissue distribution of the antigen detected by monoclonal antibody 225.28S in various pigmented and nonpigmented minors as well as normal skin using immunofluorescence methods. Twenty-three specimens of primary and metastatic malignant melanoma were used. As controls, normal skin, pigmented nevi, and tumors of the skin and central nervous system were used (Table 1). All tissues were obtained at biopsy or operation and cut in half; one half was fixed in 10%-formalin solution for routine histopathological diagnosis, and the other half was snap frozen in normal hexane at -80~ and stored in a deep freezer until use. Hybridomas were made using the murine-myeloma cell line, SP 2/0-Ag-14, and splenocytes from mice

ERYTHEMA ELEVATUM DIUTINUM DEVELOPING SQUAMOUS CELL CARCINOMA

After a 25‐year history of erythema elevatum diutinum involving the extremities and later the face and the buttocks in a 70‐year‐old Japanese man, squamous cell carcinoma developed from one of the lesions on the right foot. Histological examination of erythema elevatum diutinum revealed leukocytoclastic angiitis of the upper dermal vessels and perivascular fibrosis. DDS was not effective for erythema elevatum diutinum.

A CASE OF ANGIOKERATOMA CORPORIS CIRCUMSCRIPTUM NEVIFORME

A 21‐year‐old man with angiokeratoma corporis circumscriptum neviforme (ACCN) involving his left buttock, thigh, leg, and foot was treated by resection of the lesions followed by skingrafts. The lesions had gradually spread and proliferated for 10 years following previous surgical therapy in our department.