It is well established that the mast cell is the primary effector cell in urticaria, but the mechanism of its skin-specific activation should be elucidated to understand the pathogenesis of urticaria. Cutaneous mast cells, but not those from other sites, are selectively activated by C5a and substance P (SP). We observed the spontaneous release of SP in skin chambers attached on skin of patients with severe chronic urticaria. In vitro stimulation of skin slices with SP revealed heterogeneity among the skin donors. Some released LTB4, TNFα, and histamine; Others released LTB4 or TNFα and histamine or histamine alone. Such heterogeneity may account for the cell infiltration observed in some cases of chronic urticaria. Recent work has revealed that about one third of patients with urticaria have circulating histamine-releasing autoantibodies against the high affinity IgE receptor (FcεRI) and, less commonly, against IgE (“autoimmune urticaria”). HLA analysis has suggested the presence of a genetic diathesis for this subgroup of urticaria, but neither clinical nor histopathological investigations distinguished this subgroup from the other. Cysteinyl leukotriene receptor antagonists, such as montelukast and zafirlukast, have been reported to be effective for treating patients that did not respond to histamine receptor antagonists, but others have reported no effect with these drugs. For patients with autoimmune urticaria, the effectiveness of immunosuppressive therapies, such as cyclosporin, intravenous immunoglobulin, and plasmapherasis, have been reported in parallel with the reduction of circulating autoantibodies.
In order to determine the prevalence of Herpes Zoster (HZ) in the year 2000, 1,065 cases from 11 medical facilities were analyzed. 1) There was a peak month, August, in HZ frequency but no seasonal difference. 2) The ratio of males to females was 1 : 1.4. The widest peak of age ditributions was centered on the 60s and small one on the 20s. 3) The lesion most frequently occurred in the region of Th1–8. 4) The incidence of HZ generalisatus was in 1 of 50 patients, and was most frequent in the region of V1–3 in people in their 70s. 5) PHN remained in 1 of 20 patients, and was most frequent in the region in the region of Th9–12 in people in their 70s. 6) About 80% of the patients was medicated with antivirotics. 7) Ocular complications had emerged in 13% of the patients in the region of V1–3, and Ramsay-Hunt syndrome had emerged in 1%. 8) The basic disease was recognized in 8.8%. 9) 3.6% of patients had been infected more than twice. 10) In a comparison of age distributions between hospitals and clinics, over sixties were mainly noted in the former group, and under fifties were dominant in the latter one.
The serum samples of 18 mothers of infants with neonatal lupus erythematosus (NLE) observed at Nara Medical University and its associated hospitals over a period of 24 (1977–2000) years were analysed for anti-Ro/SSA and La/SSB antibody profiles. The antibodies were examined by double immunodiffusion (DID), using swine spleen extracts as the antigen source. Molecular analysis of anti-Ro/SSA antibody responses to proteins in the sera was carried out by enzyme-linked immunosorbent assay (ELISA), using recombinant 52 kd and 60 kd fusion proteins ; analysis of anti-La/SSB antibody was performed using recombinant 48 kd fusion protein. The mothers who had both anti-Ro/SSA and La/SSB antibodies had a greater risk of bearing infants with NLE than did the mothers who had only the anti-Ro/SSA antibody. The mothers of children with the skin lesion of NLE were positive for high titers of 48-kd protein. The mothers of children with complete heart block (CHB) were positive for high titers of 52-and 60-kd proteins. The data suggest that anti-La/SSB antibodies influence the development of the skin lesion of NLE, and that anti-52kd Ro/SSA antibodies influence the development of CHB of NLE. Oral corticosteroid therapy beginning before the 16th week of gestation may be effective in the prevention of CHB.
We report two cases of contact dermatitis with a reactive widespread eruption showing flame figures histopathologically. Case 1 was a 64-year-old man who developed pruritic edematous erythema with vesicles over his entire body after contact with lacquer on his right hand. A patch test showed a positive reaction for urushiol with provocative erythema on his palms. Case 2 was a 21-year-old woman who suffered a second-degree burn on her right arm and underwent topical treatment with Theradia pasta®. She subsequently exhibited indurated erythema with vesicles on the right forearm. Indurated erythema developed on the left arm and trunk where the ointment had not been applied. She was positive for a patch test with Theradia pasta®. Biopsy specimens from these two cases showed flame figures in the dermis. Eosinophilic cellulites (Wells’ syndrome) may be observed in a hypersensitive state associated with contact dermatitis, as shown in these two cases.
A case of relapsing polychondritis with tracheal stenosis was successfully treated by steroid therapy and stainless-stent. A 59-year-old male had redness and swelling of both ears, saddle nose, and severe main bronchial stenosis. He was treated with steroid therapy and implantation of a stainless stent. After the therapy, his various symptoms improved. He showed high titer of type II collagen antibody in the acute phase of the disease and a marked fall in antibody level with the treatments. We suggest a possible role for type II collagen antibody in relapsing polychondritis.
We report a 47-year-old woman with erythema exudativum multiforme (EEM) who presented with ulcerative colitis (UC). She developed multiple erythema with vesicular formations, mainly on the extremities and accompanied by slight deterioration of the conjunctiva and lip, at the same time that the UC symptoms appeared. The biopsy revealed a keratinocyte necrosis, interface dermatitis in the epidermis, and perivascular infiltration of mononuclear cells. leading to the diagnosis of EEM. We were able to rule out drug-induced eruptions and infectious eruptions. UC is rarely associated with EEM. There has been no report of UC association with EEM in Japan. It is necessary to take into consideration the complication of UC occuring with EEM.