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Biomedical subjects

H Tagami

Publications and source records attributed to H Tagami.

At least 235 records · Page 13Linked to original sources
PubMed1991

Tyrosinemia II: report of an incomplete case and studies on the hyperkeratotic stratum corneum.

We report a case of tyrosinemia II in a 20-year-old man who had painful palmoplantar hyperkeratotic lesions and corneal erosion. His serum tyrosine level was more than 10 times above normal. Though dietary control failed to eliminate the skin lesions, etretinate cleared them temporarily and grafted skin from the buttock did not become hyperkeratotic during the observation period of 1 1/2 years. Amino acid analysis in the hyperkeratotic stratum corneum disclosed a moderate increase not only in soluble tyrosine but also in other amino acid contents. Turnover time of the hyperkeratotic stratum corneum was greatly elongated as compared with that of the grafted site.

Adult↗
PubMed1991

Complement fragment C4d and Bb levels in inflammatory skin diseases (e.g. SLE, atopic dermatitis, erythroderma and pustulosis palmaris et plantaris) for assessment of complement activation.

The complement is one of the major effector system in the process of inflammation. Complement activation has been shown to occur in inflammatory dermatoses such as systemic lupus erythematosus, atopic dermatitis, erythroderma of unknown origin, and pustulosis palmaris et plantaris by the elevated blood levels of complement fragments. To clarify the complement activation, especially the alternative pathway involvement, we have measured the concentrations of classical pathway-specific C4d and alternative pathway-derived Bb in the plasma of patients with these inflammatory disorders at a mild to exacerbated stage. Only the SLE plasma showed significantly elevated Bb levels. These results suggest that assessments of plasma C4d and Bb levels may be of value in monitoring the involvement of the complement system in patients with inflammatory dermatoses with significant complement activation.

Adult↗
PubMed1991

Nucleotide sequence of the putative human tyrosinase pseudogene.

We have cloned and sequenced the putative human tyrosinase pseudogene, which shares more than 98% nucleotide homology with exon 4 and exon 5 of the human tyrosinase gene including their flanking introns. Because of such a high homology, both the tyrosinase gene and its pseudogene could be amplified from genomic DNA by polymerase chain reaction. The nucleotide sequences presented thus enable us to discriminate the tyrosinase gene from its related sequences and are invaluable for a gene diagnosis of oculocutaneous albinism.

Base Sequence↗
PubMed1991

Exanthematic type of pustular psoriasis consisting of two types of pustular lesion.

A 35-year-old female developed generalized pustules within a short period of time. Clinically, two distinct types of pustules were observed, viz. erythematous patches studded with crops of small pustules, and isolated large pustules with a red halo. Histologically, the former were subcorneal spongiform pustules, whereas the latter were unilocular pustules involving the hair follicular infundibulum or a subcorneal unilocular pustule on the palmo-plantar skin. On the basis of the sudden appearance of the pustules without any pre-existing lesions of psoriasis and the histological findings of the spongiform pustules, we made a diagnosis of the exanthematic type of pustular psoriasis. The lesions responded in a dose-dependent fashion to oral cyclosporin.

Administration, Oral↗
PubMed1991

Development of Ki-1 lymphoma in a child suffering from multicentric reticulohistiocytosis.

We report a case of Ki-1 lymphoma that developed in a 16-year-old youth who had suffered from multicentric reticulohistiocytosis for 10 years. Over the past 3 years he had had a peculiar sclerosing lesion of the leg for which oral prednisone 5 mg daily was tried for one year, with a moderate effect. He developed a marked swelling of the inguinal lymphadenopathy on the same side as the affected leg lesion, which also developed a prominent swelling of the skin surrounding the sclerosed area. Immunohistochemical analysis of the lymph node biopsy revealed the features of Ki-1 lymphoma. This is the first case of association of multicentric reticulohistiocytosis with Ki-1 lymphoma.

Adolescent↗
PubMed1991

Acquired zinc deficiency in breast-fed infants.

Zinc deficiency in breast-fed infants is a rare disease caused by a low level of zinc in their mother's milk. Premature infants are more vulnerable to develop zinc deficiency than full-term infants because, despite their high zinc requirements, they have insufficient body stores of zinc and a poor capability to absorb zinc from the gut. The clinical aspect of zinc deficiency is acrodermatitis enteropathica, in which the severity is proportional to the zinc level. The patients respond well to oral zinc supplements.

Breast Feeding↗
PubMed1991

Functional analyses of the superficial stratum corneum in atopic xerosis.

Dermatologists universally recognize that the unaffected skin of patients with atopic dermatitis tends to be dry and slightly scaly. To characterize the functional properties of the superficial stratum corneum in atopic xerosis, we studied the forearms of 28 patients with atopic dermatitis, aged 14 to 30 years, and 18 age-matched controls, with the use of mainly noninvasive methods. Patients with atopic xerosis showed markedly higher transepidermal water loss and markedly lower skin surface hydration levels than did the controls. The corneocytes in atopic xerosis tended to desquamate in clumps of cell aggregates instead of as individual cells. They contained a substantially lower amount of water-soluble amino acids, which play a role in the water-retaining capacity of stratum corneum, than did those of controls. Although the number of stratum corneum cell layers in atopic xerosis (21 +/- 4) was substantially larger than that in controls (15 +/- 1), its turnover time (7 +/- 2 days) was appreciably shorter than that for controls (14 +/- 2 days). Like those noted in the skin with increased epidermal proliferation, the size of superficial corneocytes in patients with atopic xerosis was substantially smaller than in controls. Histopathologic examination revealed acanthotic epidermis, mild perivascular mononuclear cell infiltrate, and pigment incontinence. Atopic xerosis, the dry skin of patients with atopic dermatitis, shows various stratum corneum functional impairments, probably reflecting increased epidermal proliferation due to a low-level ongoing dermatitis.

Adolescent↗
PubMed1990

Molecular basis of tyrosinase-negative oculocutaneous albinism. A single base mutation in the tyrosinase gene causing arginine to glutamine substitution at position 59.

Tyrosinase-negative oculocutaneous albinism (OCA) is one of classical inborn errors of metabolism, characterized by a complete lack of melanin pigments in the eyes and skin. We have isolated and characterized the tyrosinase gene of one child (F. S.) affected with tyrosinase-negative OCA. Sequence analysis reveals a single-base mutation in the exon 1 (a G to A transition at nucleotide residue 312), causing the Arg (CGG) to Gln (CAG) substitution at position 59. This base change eliminates one MspI site and creates a new BstNI site in the patient's exon 1, which is invaluable for screening other OCA patients and heterozygote carriers for this mutation. We are thus able to confirm that the patient F. S. is homozygous for this OCA allele. The family members of the patient F. S. are phenotypically normal, but are shown to be heterozygote carriers. Transfection of the mutant gene fails to give rise to detectable tyrosinase activity in transient expression assays, suggesting that the mutation affects the stability or the catalytic activity of the enzyme. We therefore propose that the albino phenotype of the patient F. S. is a consequence of the Arg to Gln substitution at position 59 caused by a point mutation in the tyrosinase gene.

Albinism, Oculocutaneous↗
PubMed1990

Interleukin 2, soluble interleukin 2 receptor, and interferon-gamma in the suction blister fluids from psoriatic skin.

Psoriasis represents inflammatory skin disorders characterized by significant changes in cellular immunity, particularly exhibiting alterations in T lymphocyte-related functions. Early psoriatic lesions have been reported to show an infiltration of activated helper T cells. Elevated levels of interleukin 2 (IL-2), IL-2 receptor (IL-2R), and interferon-gamma (INF-gamma) are associated with an early activation of T cells. To examine local activation of T cells in psoriatic skin, the amounts of activated T cell products, IL-2, secretory form of IL-2R (sIL-2R) and INF-gamma were measured in the fluids of suction blisters raised on psoriatic skin. sIL-2R levels were significantly elevated in the suction blister fluids raised on psoriatic involved skin compared with those on normal and psoriatic uninvolved skin. On the other hand, neither IL-2 or IFN-gamma was detected in the suction blister fluids either from normal, psoriatic uninvolved, or involved skin. However, we could detect IFN-gamma and IL-2 in the psoriatic scale extracts. Although we failed to detect IL-2 and IFN-gamma in the suction blister fluids, the increased levels of sIL-2R in the suction blister fluids from the psoriatic lesional skin indicate local activation of T cells in psoriatic lesional skin.

Adolescent↗
PubMed1990

Therapeutic success of epidermal grafting in generalized vitiligo is limited by the Koebner phenomenon.

Eighteen patients with vitiligo (two with localized type, five with segmental type, and 11 with generalized type) were treated with an epidermal graft using the roof of a suction blister. In all patients with localized and segmental lesions and in seven with generalized lesions, successful repigmentation without scarring was observed at the grafted sites approximately 2 to 3 weeks after grafting, and a subsequent two- to threefold expansion of the pigmented area occurred within 3 to 4 months. On the other hand, no repigmentation could be achieved in four patients with generalized vitiligo in whom depigmentation (i.e., the Koebner phenomenon) occurred at the donor sites. Our results suggest that the Koebner phenomenon occurs in an all-or-none pattern only in patients with generalized vitiligo. Thus, although epidermal grafting is a simple and useful method, successful repigmentation at the recipient sites depends on the selection of patients with localized lesions in whom the Koebner phenomenon does not develop.

Adolescent↗
PubMed1990

Erythema induratum of Bazin as a type of tuberculid.

We report three cases of patients with erythema induratum of Bazin who showed extreme tuberculin hypersensitivity reactions and a good therapeutic response to isoniazid. In the first patient satellite lesions with epithelioid granuloma formation developed 3 weeks after injection of purified protein derivative. In the second patient a high fever developed 1 day after injection of purified protein derivative, and the third patient blistered at the injection site; the histopathologic pattern was similar to erythema multiforme. Immunohistologic study of the epithelioid granulomas demonstrated a predominance of Leu-1--bearing, Leu-3a--bearing, and human lymphocyte antigen-DR-expressing cells. We conclude that erythema induratum of Bazin represents a tuberculid caused by heightened delayed-type hypersensitivity to components of tubercle bacilli released from latent foci of infection.

Adult↗
PubMed1990

Autologous mixed lymphocyte reaction is reduced in patients with psoriasis.

The autologous mixed lymphocyte reaction (auto-MLR) was studied to test the interactions between immunocompetent cells in patients with psoriasis. The auto-MLR in 20 patients with psoriasis was significantly lower than in 16 normal controls. Lower values were found in untreated psoriatic patients than in those in remission following treatment. The values in the latter group were significantly lower than in controls and in six patients with atopic dermatitis in remission. The tendency for an increase in the auto-MLR with a decrease in disease activity was further confirmed in five patients studied before and after treatment. In contrast, the allogeneic lymphocyte reaction (allo-MLR) in psoriatics was similar to that in normal controls.

Culture Media↗
PubMed1990

Activation of the alternative pathway of complement in psoriatic lesional skin.

The complement system may play an important role in the inflammatory reaction of psoriasis. While the classical pathway of the complement has been shown to be activated in psoriasis, there have been few reports on the involvement of the alternative pathway in the inflammatory reactions in psoriasis. Complement fragments, C4d and Bb, are released at the time of the classical and alternative pathway activation, respectively. The presence of the C4d or Bb fragment, therefore, denotes a preceding complement activation through the classical and/or alternative pathway. In the present study, we have measured the levels of C4d and Bb in psoriatic scale extracts using enzyme immunoassay techniques. The scales of these dermatoses contained higher levels of C4d and Bb detectable by enzyme immunoassay than those in the stratum corneum of noninflammatory skin. These results suggest that the alternative as well as the classical pathway of complement are activated in psoriatic lesional skin.

Complement Activation↗
PubMed1990

Granulocyte-macrophage colony-stimulating factor in psoriasis.

Granulocyte-macrophage colony-stimulating factor (GM-CSF), a product of activated T lymphocytes, macrophages, endothelial cells, fibroblasts, and keratinocytes, is thought to play an important role in inflammatory reactions by 'priming' or enhancing the functions of neutrophils and macrophages. The aim of this study was to determine whether GM-CSF is detectable in psoriatic lesions. No GM-CSF was detected by ELISA in the suction blister fluids raised on normal, psoriatic uninvolved or involved skin. In contrast, although we could not detect GM-CSF in most of the extracts from noninflammatory stratum corneum, it was readily detected in most of the scale extracts from psoriasis and sterile pustular dermatoses, and its level was significantly higher than that from the controls. These results suggest that in psoriasis GM-CSF may amplify and modulate inflammatory reactions and activated T cells.

Adolescent↗
PubMed1990

Hereditary benign telangiectasia: a congenital type.

Hereditary benign telangiectasia (HBT) is inherited in an autosomal dominant fashion, and in all the cases reported in the past it developed after birth. We describe 3 congenital cases of HBT in the same Japanese family: a 29-year-old father and his 5-month- and 2-year-old daughters have had scattered erythematous patches of various sizes in diameter since birth.

Adult↗
PubMed1990

Pigmented nail streak associated with Bowen's disease of the nail matrix.

We described a 59-year-old male physician with Bowen's disease occurring on the nail matrix of his right 5th finger. The rapid growth of the pigmented nail streak accompanied by nail deformity led us to consider the possibility of subungual melanoma clinically. Histologic features, however, were compatible with those of Bowen's disease accompanied by melanocytes with melanin-rich long dendrites in the nail matrix. We speculate that his occupational exposure to X-rays for 25 years played an important role in the pathomechanism of the present case.

Bowen's Disease↗