Annular erythema associated with Sjögren's syndrome.
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Biomedical subjects
Publications and source records attributed to I Katayama.
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Pompholyx (Dyshidrosis) is a disease of unknown etiology presenting as symmetrical, vesicular hand and foot dermatitis. To clarify the pathogenetic role of sweat and metal allergy, twenty five patients with pompholyx were examined with respect to their perspiration volume using equipment that continuously recorded the local perspiration volume. Patients were also examined for metal allergy by patch testing and oral challenge tests. The perspiration volumes were measured using recently developed equipment for continuous recording of local perspiration volume (Kenz-Perspiro oss-100). The perspiration volume of pompholyx patients was found to be 2.5 times higher than that of age-matched normal controls. Twenty percent of the patients showed sensitivity to chromate, 16% to cobalt, and 28% to nickel on patch testing. Six patients with positive results were challenged orally in a controlled trial with 2.5 mg nickel, 1 mg cobalt, or 2.5 mg chromium. Four of them (67%) showed vesicular reactions on their hands with challenge testing. These results indicated a sensitivity to metal compounds which, in combination with local hyperhidrosis, may contribute to the development of vesicular lesions in pompholyx.
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The present study shows the effect of interleukin-1 beta (IL-1 beta) on some gene expressions and functions of fibroblastic cells (HPLF) derived from human periodontal ligament. HPLF were used at passages number 5 to 10. IL-1 beta increased DNA synthesis in both a dose- and an incubation time-dependent manner. IL-1 beta in combination with tumor-necrosis factor alpha or transforming growth factor beta synergistically stimulated the DNA synthesis in the cells. Since many studies have shown that the c-myc oncogene is involved in cell proliferation and differentiation, the effect of IL-1 beta on c-myc messenger RNA (mRNA) level in HPLF was examined. IL-1 beta induced a marked c-myc mRNA level in the cells at 90 minutes after initiation of the cytokine treatment. On the other hand, IL-1 beta significantly inhibited alkaline phosphatase (ALP) activity of the cells in a dose-dependent manner. Also an inhibitory effect was observed on the liver/bone/kidney ALP mRNA level of the cells, and this inhibition by IL-1 beta was dose- and incubation time-dependent. These results suggest that IL-1 beta is a regulatory cytokine involved in the regeneration of the human periodontal ligament.
Mast-cell-derived mediators showed mitogenic activities on mouse-transformed epidermal cell line Pam 212 cells. These activities were eluted into the low-molecular-weight fractions below a molecular weight of 10 kD on a high performance liquid chromatography TSK 2000G column, and were partially abrogated by antihistamines or anticytokine antibodies, including anti-IL1 alpha, -IL1 beta or IL6 antibodies. Pretreatment of mast cell lines with sodium butyrate enhanced the production of these factors. Calcium ionophore or Concanavalin A (ConA) stimulated mast cells to generate factor production. These results suggest that mast-cell-derived mediators might play some role in epidermal hyperplasia seen in lichenified lesions in atopic dermatitis.
A 45-year-old male was hospitalized on September 2, 1989 with chief complaints of general fatigue and fever. Physical examination revealed hepatomegaly and massive splenomegaly. Laboratory tests on admission showed Hb of 7.5g/dl, PLT 4.8 x 10(4)/microliters and WBC 9,610/microliters with 81% hairy cells. Bone marrow aspirate demonstrated 55.1% hairy cells and moderate myelofibrosis. Cytochemically, hairy cells were positive for tartrate-resistant acid phosphatase (TRAP). Surface markers were SmIg G+ A+ kappa +, CD11b+, CD11c+, CD19+, CD20+, CD21-, CD25+, HC2+, HLA-DR+. From these findings, a diagnosis of hairy cell leukemia (HCL) was made. After administration of deoxycoformycin (DCF) at a dose of 5.0mg/m2 1-2 times monthly, splenomegaly disappeared, as did hairy cells from the peripheral blood. Hematological level returned to within normal range except for the presence of 1.2% hairy cells and mild myelofibrosis in bone marrow aspirates. DCF has so far been effective for this patient. While DCF has been reported to be effective in the treatment of HCL in the West, it has not been determined in Japanese patients with HCL, who have different hematologic features from those of HCL patients in the West.
Psoriasis is a disease of abnormal proliferation and differentiation of epidermal cells. Several cytokines released by keratinocytes are implicated as factors responsible for this pathological condition of the epidermis. In order to elucidate the role of these cytokines in psoriasis, messenger RNA (mRNA) expression of interleukin-1 (IL-1) and IL-6 in psoriatic epidermis was investigated using biotin-labelled complementary DNA (cDNA) of the cytokines. Messenger RNA of IL-1 alpha was weakly detected in some normal healthy epidermis specimens and more strongly in all the perilesional uninvolved psoriatic epidermis specimens. It was also expressed in the transitional zone between uninvolved and fully developed psoriatic skin, but was not expressed in lesional skin. In contrast, IL-6 mRNA was rarely expressed in normal healthy epidermis, but was expressed in perilesional uninvolved psoriatic epidermis, in the transitional zone and in the fully developed lesional epidermis, with the maximum intensity in the transitional zone. Expression of mRNA of IL-6 receptor showed a similar tendency to that of IL-6. It was expressed in psoriatic epidermis, most strongly in the transitional zone, but not in normal healthy epidermis. IL-6 was demonstrated immunohistochemically in psoriatic epidermis, but IL-6 receptor was demonstrated only in the transitional zone. Thus IL-6 and its receptor expression correlated well with the formation of psoriatic lesions where IL-1 may initiate their expression. IL-6 may play an important role in the pathogenesis of psoriasis.
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We reviewed 111 clinical and pathological materials of patients who had been entered as mycosis fungoides (MF) or Sézary syndrome (SS) in the Annuals of the Pathological Autopsy Cases in Japan during the 29-year-period since the Annuals' inception in 1958. One hundred and seven patients were classified. Of these, 64 were in Group 1 (mycosis fungoides, MF), 23 in Group II (Sézary syndrome, SS), and 20 in Group III (non-Hodgkin's lymphoma, NHL). The clinicopathologic features of Groups I and II (MF, SS) were similar to those of cutaneous T cell lymphoma (CTCL) in Europe and the United States. Those of Group III (NHL) were indistinguishable from primary cutaneous non-Hodgkin's lymphoma. It was suggested that some patients with adult T cell leukemia/lymphoma (ATLL) with skin lesions used to be misdiagnosed as MF or SS in earlier years when ATLL was still unknown. It was concluded that CTCL in Japan has clinicopathologic features quite like those of CTCL in Europe and the United States and that they are probably the same disease.
Labial lip biopsy findings from 52 patients with Sjögren syndrome (SjS) and cutaneous manifestations were clinically analyzed. Findings of the labial lip biopsy with more than one focus (a cluster of at least 50 lymphocytes) were positive in 52% (28/52). Sixteen patients who presented with oral dryness had negative labial lip biopsy findings with less than one focus score and five patients without oral dryness had positive results. Cells infiltrating into the salivary gland were categorized as plasma cell rich (Group 1), lymphocyte rich (Group 3), intermediate (Group 2), or no gland in biopsied specimens (Group 4). Sicca complaint and the grade of lip biopsy score were significantly higher in Group 3 than in Group 1; no differences were observed in laboratory findings between Groups 1 and 3. The patterns of age distribution and salivary flow rate were also not statistically significant in these two groups. The patients with plasma cell rich infiltrate presented oral dryness, although their clinical and grade of lip biopsy scores were relatively low; most of these patients have secondary SjS. Some of the patients in Group 4 showed an advanced stage of the disease through their lip biopsy score was grade 1 with atrophic or no gland. These data suggest that labial lip biopsy is a useful screening method for the diagnosis of Sjögren syndrome and that clinical manifestations may differ in parallel with the type of infiltrating cell.
Lymphocyte response to staphylococcal enterotoxin B (SEB) was analysed in 11 cases with Sjögren syndrome (SjS) who developed annular erythema during the course. Increased lymphocyte response against SEB was observed on day 5 in those patients who developed annular erythema at the time of examination. A similar reaction pattern was observed when Con A and PWM, but not protein A, were used as the mitogen. No significant lymphocyte response was observed in the patients in the inactive stage without annular erythema or in normal controls. Furthermore, peripheral blood lymphocytes express LFA-1 after SEB stimulation. These results suggest that lymphocytes from SjS patients react to various types of antigenic or mitogenic stimulation and that they express cell adhesion molecules, especially in patients with active annular erythema.
Annular erythema developed in 22 patients with Sjögren syndrome. Clinically, the annular erythema was subdivided into three forms: Sweet disease-like annular erythema with an elevated border (14 cases); subacute cutaneous lupus erythematosus (SCLE)-like marginally scaled erythema (5 cases); and papular erythema (3 cases). Histopathologically, features commonly seen in annular erythema are deep perivascular and/or periappendageal infiltration of the lymphocytes with an admixture of neutrophils or plasma cells and less frequent epidermal changes suggestive of cutaneous lupus erythematosus. Immunoglobulin or complement deposition along the dermoepidermal junction of lesional skin was observed in 8 of 18 cases, and most of the dermal infiltrates consisted of CD4(+), 4B4(+) cells. The appearance of anti-SS-A(Ro) (100%) and anti-SS-B(La) (77%) was significantly higher in patients with annular erythema. These results suggest that patients with Sjögren syndrome might have a distinct annular erythematous lesion that is both clinically and histopathologically different from SCLE, although close immunologic abnormalities exist in these two diseases.
A 21-year-old male with SLE developed seizure, loss of consciousness and focal signs referable to involvement of the front-temporal brain regions. MRI (magnetic response imaging) image revealed high signal areas in the temporal lobes. By these findings, herpes simplex encephalitis (HSE) was suspected at first. But neither isolation of herpes simplex virus nor HSV specific IgM by ELISA was detected. Acyclovir administration by intravenous infusion was'nt effective but corticosteroid pulse therapy was effective. The level of anticardiolipin antibody was very high. Finally, the diagnosis of CNS-lupus with HSE-like characteristics was made in this case.
ICAM-1 and LFA-1 expression was studied in the lesional skin of ten cases of annular erythema associated with Sjögren syndrome. Most of the infiltrating mononuclear cells around blood vessels expressed LFA-1 in addition to its strong expression on vascular endothelial cells and focal expression on the epidermal basal cell layer in 3 cases. ICAM-1 expression on vascular endothelial cells was similar to LFA-1, although relatively focal and weak expression was observed on mononuclear cells. ICAM-1 expression on keratinocytes was focal and limited to the basal cell layer in annular erythema. These findings suggest that strong expression of ICAM-1 on endothelial cells but not keratinocytes and LFA-1 on mononuclear cells might play some role in the induction of skin lesions in annular erythema associated with Sjögren syndrome.
Biphasic skin reaction with peak response at 1 and 24 h with prominent mast cell degranulation was induced by intravenous application of a monoclonal anti-DNP IgE antibody and subsequent skin test. This reaction was hapten-specific and mast-cell-dependent because no reaction was observed when oxasolone was used as an elicitation antigen or skin test was elicited in genetically mast-cell-deficient mice (W/Wvv). A partial spongiotic reaction and mononuclear cell infiltration into the epidermis were observed in mice with hyperplastic epidermis induced by topical retinoic acid. Cotransfer of DNFB-sensitized lymph node cells with anti-DNP IgE antibodies failed to enhance the skin test reaction in unsensitized mice. These results suggest that, to some degree, IgE antibody may play some role in the development of eczematous skin lesions in the rodent system without the involvement of cellular hypersensitivity.
A case of a 54-year old man with lichen mucosae and Sjögren's syndrome was reported. Clinical and histopathological analysis was made on the presence of sicca features in 6 patients with lichen mucosae. All patients complained either xerophthalmia or xerostomia and 5 out of 6 cases showed specific lymphocytic foci seen in Sjögren's syndrome in addition to lichenoid tissue reaction in the specimen obtained from buccal mucosae. Objective keratoconjunctivitis sicca was demonstrated in 3 cases. Immunologically, abnormal laboratory findings were observed in 5 cases (4 cases with a positive ANA and 3 cases with a positive anti microsome antibody). These findings suggest that lichen mucosae might develop in a close association with Sjögren's syndrome and relationship between these two disease was discussed in this article.
Twenty one patients with livedo racemosa (LRa) and periarteritis nodosa cutanea were screened for the presence of anti-cardiolipin antibody (aCL) by ELISA. 11 out of 21 revealed positive aCL whose clinical features could be divided into 3 groups: 4 patients with livedo with ulceration, 2 with pyoderma gangrenosum-like lesions, and 5 with extensive LRa. #1. The summer ulceration of leg of 4 patients were surrounded by several tiny petechiae where hyaline microthrombi of blood capillaries in the upper dermis were demonstrated. All had multiple cerebral microinfarctions on magnetic resonance imaging (MRI). #2. Pyoderma gangrenosum-like lesions of 2 patients consisted of painful punched-out ulcers on livedoid lesions where intravascular endothelial hyperplasia in a small artery was demonstrated. Both had multiple cerebral microinfarctions. #3. Another clinical manifestation of the patients with positive aCL was extensive form of LRa of 5 patients. All had positive ANA, however none of them exhibited cerebral microinfarctions on MRI. #4. The remaining 10 patients with negative aCL showed no relationship with such clinical and laboratory manifestations. These results indicate that there is a significant correlations between summer ulceration with petechiae, capillary microthrombi of the skin, positive aCL and cerebral microinfarctions on MRI.