Scleredema of Buschke associated with malignant insulinoma.
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Female↗
Biomedical subjects
H Tagami
Publications and source records attributed to H Tagami.
Functional assessment of the stratum corneum under the influence of oral aromatic retinoid (etretinate) in guinea-pigs and humans. Comparison with topical retinoic acid treatment.
Clinically we have noted that the skin of patients treated with long-term oral etretinate becomes uniformly soft and smooth to touch, like facial skin that becomes smoother and less wrinkled following treatment with topical tretinoin. This suggests that retinoids, whether used systemically or topically, alter the physical properties of the skin, particularly of the stratum corneum (SC). To study the influence of retinoids on the SC, we serially assessed the functional properties of the SC non-invasively in retinoid-treated humans and experimental animals. SC hydration and barrier function were assessed by measurement of high-frequency conductance and transepidermal water loss (TEWL), respectively. Daily application of topical retinoic acid creams was found to rapidly induce a time- and dose-dependent, linear increase in SC hydration of the forearm skin of healthy adults over a 2-week period and to compromise its water barrier function in a similar fashion. Systemic administration of high-dosage etretinate, 4 or 8 mg/kg/day, to guinea-pigs also induced dose-dependent increases in both SC hydration and TEWL measured on the plantar skin after 1 month. Moreover, in the animals given etretinate 4 mg/kg/day we confirmed a slight but significant decrease in the number of cell layers of the plantar SC. Likewise, patients with various dermatoses began to show similar functional changes of the SC in the uninvolved skin of the flexor surface of the forearms 3 weeks after the start of oral etretinate treatment, consisting of 50 mg daily for 2 weeks, followed by gradual dose tapering.(ABSTRACT TRUNCATED AT 250 WORDS)
Administration, Oral↗
Coexistence of numerous superficial basal cell epithelioma of the back and non-specific ulcers of the small intestine: occurrence of two rare diseases in the same patient.
A 53-year-old female developed multiple disseminated brownish macules that were confirmed histologically as superficial basal cell epitheliomas on her lower back. She also suffered from short bowel syndrome after repeated resection of a segment of the small intestine because of non-specific ulcers of the small intestine, a rare ulcerative disorder of unknown aetiology. The association of these two rare conditions in the same patient is of interest because it may be more than a simple coincidence.
Basal Cell Carcinoma↗
Malignant melanoma complicated by schwannoma.
We report two cases of malignant melanoma associated with a schwannoma. Case 1 is a 32-year-old Japanese man who had a nodular melanoma in his epigastrium, associated with one subcutaneous nodule on the neck and another on the right thigh, both of which were histologically-proven schwannomas. Case 2 is a 48-year-old Japanese woman with an acral lentiginous melanoma on her right sole who later developed a schwannoma of the cerebello-pontine angle. Of all 146 malignant melanoma cases found in our clinic during the past 20 years, 1.4% were associated with a neurogenic tumour, a significant association.
Adult↗
Skin metastasis in laryngeal carcinoma.
Cutaneous metastases from carcinoma of the larynx are very rare. We observed a 64-year-old Japanese patient with squamous cell carcinoma of the larynx who developed a subcutaneous nodule on the abdomen. It was found to be a metastatic tumour from the laryngeal cancer, histopathologically as well as histochemically.
Carcinoma, Squamous Cell↗
Identification of mutations in the pigment cell-specific gene located at the brown locus in mouse.
The pigment cell-specific gene, located at the brown (b)-locus in mouse, encodes the protein that determines the type of melanin synthesized. This protein is known as tyrosinase-related protein, but here we tentatively term it b-locus protein to avoid confusions with the related sequence cross-hybridizing to the tyrosinase gene. In order to identify the mutation at the b-locus, we have cloned and characterized the b-locus protein gene of BALB/c mouse (b/b, c/c). The gene is about 18 kb long and organized into 8 exons and 7 introns. Sequence analysis of the b-locus protein gene reveals four base changes within the protein-coding regions: two missense mutations and two silent mutations. Two missense mutations result in the Cys to Tyr substitution at position 86 (codon 110) and the Arg to His substitution at position 302 (codon 326) of a b-locus protein molecule. Using allele-specific amplification, we confirmed that these missense mutations are actually present in the genomic DNA of two b-mutant strains examined, BALB/c and DBA/2 (b/b, C/C) mice, suggesting that these mutations are specific for the mutant mice at the b-locus. Moreover, we are able to show that the b-locus protein containing Tyr 86 is not reactive with the anti-b-locus protein monoclonal antibody, TMH-1, in transient expression assays.
Amino Acid Sequence↗
Molecular bases of tyrosinase-negative oculocutaneous albinism: a single base insertion or a missense point mutation in the tyrosinase gene.
We have identified two different mutations in the tyrosinase genes of Japanese patients with tyrosinase-negative oculocutaneous albinism (OCA). One is a single base insertion in the exon 2 of the tyrosinase gene that shifts the reading frame and introduces a premature termination codon (TGA) after the amino acid residue 298 (codon 316). The other is a G to A transition at residue 312, leading to a single amino acid substitution, arginine at position 59 (codon 77) to glutamine. The promoter activity of the patients' tyrosinase genes was evaluated in the cell-free transcription system prepared from pigmented melanoma cells, indicating that the patients' genes were accurately transcribed in vitro. It is therefore conceivable that the tyrosinase gene is expressed in their melanocytes. Furthermore, transient expression of the mutated genes indicates that the truncated tyrosinase or the tyrosinase containing glutamine 59 is unable to form melanin in melanocytes. We therefore propose that these mutations in the tyrosinase genes lead to a phenotype of tyrosinase-negative OCA.
Albinism, Oculocutaneous↗
Melanocyte-stimulating properties of arachidonic acid metabolites: possible role in postinflammatory pigmentation.
Normal human epidermal melanocytes became swollen and more dendritic with an increase in the amount of tyrosinase and immunoreactive b-locus protein when they were cultured for 2 days with the following arachidonic acid metabolites: prostaglandin (PG) D2, leukotriene (LT) B4, LTC4, LTD4, LTE4, thromboxane (TX) B2 and 12-hydroxy eicosatetraenoic acid (12-HETE). The effect of LTC4 was particularly strong compared to that of PGE2, about which we have previously reported. On the other hand, PGE1, PGF2 alpha and 6-ketoPGF1 alpha did not show any significant stimulatory effect. These data suggest that arachidonate-derived chemical mediators, especially LTC4, may be responsible for the induction of post-inflammatory hyperpigmentation of the skin.
12-Hydroxy-5,8,10,14-eicosatetraenoic Acid↗
Menkes' disease: report of a case and determination of eumelanin and pheomelanin in hypopigmented hair.
We report a male infant with Menkes' disease who showed, at the age of 3 months, slow growth, hair abnormalities such as pili torti and white hair, and low levels of serum copper and ceruloplasmin. The exceptionally bright portions of his hair contained eumelanin and pheomelanin at levels only half those of normal Japanese controls. After subcutaneous administration of copper-histidinate for 2 months, his scalp hair changed to dark brown.
Ceruloplasmin↗
Annular psoriasiform eruption with lymphocytic infiltration of the epidermis: a variant of acute psoriasis?
Disseminated annular psoriasiform lesions developed over a period of 2 months in a 48-year-old man with no preceding psoriatic history of drug intake, being accompanied by general dullness and arthralgia. Etretinate was effective for both skin eruption and arthralgia; only the latter recurred on its cessation 5 months later. However, histologic features examined by serial sections totally lacked those of pustular psoriasis; there were no neutrophils in the epidermis where massive T lymphocyte infiltration existed instead, in a fashion similar to that of early psoriatic lesions. We differentiated this peculiar annular psoriasiform eruption from the annular erythematous lesions noted in pityriasis rosea, erythema annulare centrifugum, subacute cutaneous lupus erythematosus, annular erythema associated with Sjögren's syndrome and erythema chronicum migrans. It is our speculation that this dermatosis represents a variant of acute psoriasis, rather than annular pustular psoriasis. The histopathologic and immunohistologic findings suggest ongoing cellular immune responses in these lesions where some unknown inhibitory mechanisms might be operative against further production of neutrophil chemotactic factors that usually takes place in psoriatic lesions.
Acute Disease↗
Generation of terminal complement complexes in psoriatic lesional skin.
The complement system is thought to play an important role in the recruitment of neutrophils within the epidermis. In the present study we examined whether or not complement activation in psoriatic lesional skin results in the deposition of terminal complement complexes within the epidermis by measuring levels of SC5b-9 in the plasma and horny tissues of psoriatic patients. The levels of SC5b-9 in psoriatic plasma were significantly higher than those of controls or those of patients with atopic dermatitis. However, when the levels of SC5b-9 in the psoriatic plasma were compared before and after successful treatment of psoriasis, a significant reduction was observed after treatment. Studies of total protein extracts from lesional skin showed that, while no SC5b-9 was detected in the noninflammatory horny tissues, there were high levels of SC5b-9 in lesional horny tissues of psoriasis. By immunofluorescence using a monoclonal antibody to the C5b-9 neoantigen, deposition of C5b-9 was observed only in the stratum corneum of psoriatic skin. Thus the results of the present study suggests that in psoriatic lesional skin, the complement system is activated and that this complement activation proceeds all the way to the terminal step, generating membrane attack complex.
Adolescent↗
Eosinophilic pustular folliculitis starting initially only with palmoplantar pustular lesions. Report of a case and review of the literature.
We report a 23-year-old Japanese male with eosinophilic pustular folliculitis (EPF) that had started with palmoplantar rash. Only when follicular pustules appeared on the bilateral cheek 31 months later, we revised our initial diagnosis of pustulosis palmaris et plantaris (PPP) to EPF, and all the skin eruptions cleared mostly with indomethacin. A review of the Japanese literature for the past 20 years disclosed that in 207 cases of EPF so far reported, palmoplantar pustular lesion was noted in 38 (18%). Among them, in 16 cases (8%) the skin lesions started first from the palmoplantar region with the average period of 26 months until the appearance of other eruptions of EPF. None of them was diagnosed as EPF when skin lesions were localized only to the palmoplantar region. When we find pustules on the palmoplantar region, we should suspect the possibility of early lesions of EPF as well as PPP. Histopathologic demonstration of multilocular pustules located in the upper epidermis containing numerous eosinophils in the palmoplantar pustular lesions, together with the dramatic therapeutic response to indomethacin greatly favor the diagnosis of EPF.
Adult↗
Preferential activation of the alternative pathway of complement in psoriatic lesional skin.
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Complement C3 Convertase, Alternative Pathway↗
Juvenile generalized pustular psoriasis in a pair of monozygotic twins presenting strikingly similar clinical courses.
We describe an exceptionally rare case of juvenile generalized pustular psoriasis noted in monozygotic twins who, after developing the disease on the same day (the 48th day after birth) continued to show strikingly similar clinical features of generalized pustular psoriasis for 7 years. Not even therapeutic intervention by tonsillectomy performed at age 4 years on one of the twins, which was expected to have some beneficial effect, could decrease the number of attacks or pustulation compared with the counterpart.
Child, Preschool↗
Structural organization of the pigment cell-specific gene located at the brown locus in mouse. Its promoter activity and alternatively spliced transcript.
The pigment cell-specific gene, located at the brown (b) locus in mouse, has been cloned and characterized. Its gene product is required for the formation of black melanin rather than brown, although its exact function remains to be elucidated. We thus tentatively named it b-locus protein in this report. The b-locus protein gene is about 18 kilobase pairs long and organized into 8 exons and 7 introns. Functional analysis of its promoter region suggests that the nucleotide residues -38/154 is sufficient to direct the pigment cell-specific transcription in melanoma whole cell extracts. On the other hand, we were unable to detect its transcripts in HeLa whole cell extracts. Sequence comparison with the promoter region of the tyrosinase gene, another pigment cell-specific gene, reveals that two elements of the b-locus protein gene (-33/-24 and 18/28) are also conserved in the tyrosinase gene at equivalent positions, suggesting that these two elements may be involved in their pigment cell-specific transcription. Furthermore, we have cloned a cDNA, pMT3, coding for an isoform of b-locus protein from a cDNA library of mouse B16 melanoma cells. Sequence analysis of pMT3 reveals a deletion of 103 base pairs, which corresponds to the 5'-end of the exon 8 of the b-locus protein gene, indicating that pMT3 represents a mRNA species generated by alternative splicing. Since this deletion changes the reading frame and eliminates the transmembrane domain of b-locus protein, the pMT3-type mRNA may code for a soluble isoform. Such an isoform, consisting of 553 amino acids, differs only in its carboxyl terminus and is larger than b-locus protein by 16 amino acids. Using transient expression assays, we confirmed that such an isoform is able to react with anti-b-locus protein monoclonal antibody, TMH-1, suggesting that a b-locus protein isoform may have some function in pigmentation.
Amino Acid Sequence↗
Determination of tumor necrosis factor in blister fluids of bullous pemphigoid.
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Aged↗
In vitro comparison of water-holding capacity of the superficial and deeper layers of the stratum corneum.
We measured the electrical conductance at 3.5 MHz of a sheet of normal stratum corneum (SC) mounted with either the upper surface or the lower surface downward in simulated in vivo conditions. In this way, we assessed the water-binding capacity of the upper and lower portions of the horny layer. Measurements were made with the SC model in environments of various relative humidities. Between 30% and 90% relative humidity, the conductance of the upper surface was always significantly higher than that of the lower surface. In contrast no significant difference was observed in transepidermal water loss (TEWL) with the SC sheet placed upwards or downwards. After exposure to lipid extraction with acetone/ether, a significant decrease in conductance and increase in TEWL occurred, particularly in the upper surface. The amount of extractable amino acids was much higher from the middle layer than from the outer layers of the SC, and was lowest from the deepest part of SC. These results, indicating a lower efficiency of the lower surface of the SC for water-binding than the desquamating upper surface, suggest that newly formed immature SC does not have the water-holding capacity of the mid portion of the SC, which is probably the layer with the greatest water holding capacity.
Adolescent↗
Interaction between human neutrophils and corneocytes: corneocyte-induced respiratory burst of neutrophils assessed by chemiluminescence.
In an in vitro study to examine whether corneocytes stimulate neutrophils to cause a respiratory burst, we found that stratum corneum (SC) homogenates obtained from the sole of healthy individuals induced a substantial respiratory burst in human neutrophils when assessed by lucigenin-dependent chemiluminescence (CL) in the presence of the fresh human serum. Electron microscopically, the interaction between corneocytes and neutrophils was shown as distinctive deformation of the neutrophils adhering to the surface of the corneocytes that suggested a specific binding between SC and neutrophils. In contrast, in the heat-inactivated serum-supplemented system, the peak intensity of SC-induced CL was significantly decreased, being only slightly higher than that noted in the SC-free background. To circumvent the time-consuming preparation of the SC homogenates, we measured CL with a sheet of SC obtained by stripping with adhesive cellophane tape. In this case we used plasma because the complement was easily activated by cellophane tape itself when serum was used. To evaluate the influence of the location of horny tissue in the SC on CL in neutrophils, we used SC sheets stripped with cellophane tape from various levels of the SC. However, there was no significant difference in CL responses between SC sheets obtained by 1, 2, 3, 5, 10, or 15 strippings. Our findings suggest that when SC comes in contact with serum, it is opsonized by C3b, and such SC causes a respiratory burst of neutrophils following their specific binding on the surface of corneocytes.
Cell Communication↗