Paraneoplastic pemphigus (PNP) is a new variant with severe erosive lesions on the oral and ocular mucosaeand variable cutaneous lesions ; it resembles Stevens-Johnson syndrome. It is mainly associated with hematological malignant tumors, particularly non-Hodgkin’s B-cell lymphoma. The anti-keratinocyte cell surface IgG autoantibodies in the sera react predominantly with multiple plakin family proteins, suchas plectin, desmoplakins I/II, BP 230, envoplakin and periplakin, by either immunoprecipitation or immunoblot analysis. Desmogleins 3/1, which are antigens for classic types of pemphigus, are also involved in PNP. PNP is intractable to various treatments, and the prognosis is very poor. In addition, lethal respiratory conditions like bronchiolitis obliterans are frequently associated with PNP. Therefore, correct diagnosis in the early stages and proper treatments are extremely important for the management of PNP patients. Dermatologists must became very aware of the conditions of this life-threatening disease.
We have collected serum samples from 178 cases with various types of autoimmune bullous diseases seen in the recent 3.5 years in our university hospital or its affiliated hospitals. We examined these samples by various techniques, mainly immunofluorescence and immunoblotting analyses, and studied their usefulness in the final diagnosis of each case. Among the 178 cases, we made definite diagnoses in 142 cases (79.8%) and possible diagnoses in 32 cases (18.0%) ; 4 cases showed no positive results and could not be diagnosed. The results of antigen analyses agreed with the clinical diagnosis in 146 cases (82.0%), and then determined the final diagnosis from multiple possible clinical diagnoses in 12 (6.7%). In 16 cases (9.0%), the results indicated final diagnoses which were different from their original clinical ones. These results indicate that various analyses of autoantigens, in addition to clinical, histopathologic and immunopahologic features, can lead to a final diagnosis in almost all cases of autoimmune bullous diseases. In order to make correct diagnoses and select proper treatments, these tests for autoantigens should be performed in various types of autoimmune bullous dermatosis, particularly in cases with atypical clinical and histopathologic features.
Alopecia totalis/universalis (AT/AU) patients 15 years old or older, who visited Juntendo University Hospital, Department of Dermatology between 1994 to 1998 were enrolled in this study . AT patients (108 cases) and AU patients (175 cases) were treated with one of four different regimens for more than six months, and the efficacy, recurrence, and adverse effects of each modality were compared. Group 1 (58 cases) was treated by topical immunotherapy using squaric acid dibutyl ester (SADBE) ; Group 2 (85 cases), by topical corticosteroid ointment ; Group 3 (104 cases), by systemic and topical corticosteroids ; and Group 4 (36 cases), by oral 8-methoxypsoralen and general UVA irradiation combined with systemic and topical corticosteroids. The therapeutic effects were quantitatively compared by scoring the state of hair regrowth. The results indicated the following percentage figures for moderate and marked improvement in each group : and Group 1, 41.4% (15.5%) ; Group 2, 37.6% (23.5%) ; Group 3, 57.7% (43.3%) ; and Group 4, 72.2% (61.1%). The recurrence rate was 66.6% for Group 1, 30.0% for Group 2, 35.6% for Group 3 and 13.6% for Group 4. The efficacy rates of Groups 3 and 4 were superior to those of Groups 1 and 2. Group 1 showed a low rate of marked improvement and a high recurrence rate, while Group 4 showed a high rate of marked improvement and a low recurrence rate. Adverse effects caused by the application of topical and systemic corticosteroids were frequently observed in Groups 2 to 4, but they improved after tapering the dose of corticosteroids. Among the four treatments tested, general PUVA therapy combined with systemic and topical corticosteroids was the best both in terms of efficacy and recurrence rate and is recommended as an excellent treatment for adult alopecia totalis/universalis.
To evaluate the value of skin biopsies in assessing the prognosis of acute graft-versus-host disease (GVHD) in postallogeneic blood stem cell transplantation, the association between histopathologic findings and clinical severity of acute GVHD was statistically examined. We retrospectively reviewed 16 cases diagnosed as acute cutaneous GVHD by skin biopsies. Neither the degree of liquefaction degeneration of the basal layer, the number of eosinophilic necrotic epidermal keratinocytes, the number of satellitoses, the presence of eosinophilic necrosis of the skin appendages, nor the degree of dermal perivascular lymphocytic infiltration were associated with either clinical grading of acute GVHD or transplant-related mortality. In addition, there was no association between histological grading and clinical grading of acute GVHD. This study demonstrates that skin biopsies do not play a role in predicting clinical severity of acute GVHD.
We treated 23 refactory cases of psoriasis vulgaris with narrow-band UVB (311 nm) therapy using TL01 lamps. The standard regimen for narrow-band UVB which is popular in European countries was used in this study. The in-patients were treated 5 times a week, and one out-patient was treated 3 times a week. Excellent or good effects were achieved in 82.6% of the patients. The remission rate was 65.2% (15 cases). Three patients developed photo-Köbner’s phenomenon during the treatments and were withdrawn. None of the patients had severe acute side effects, such as bullous formation. Narrow-band UVB therapy was found to be highly effective for patients with refractory psoriasis resistant to standard topical treatments and PUVA therapy.
We have experienced 33 cases of anaphylactoid purpura (AP) in adults (≧20 years old) in the past 6 years. Ten of these cases in the elderly (≧60 years old) were analyzed for their back ground. In the elderly, the frequencies of arthralgia (30%/52%) and abdominal complaints (10%/43%) were significantly lower than those in the younger adults (from 20 to 59 year old), but the frequencies of leg edema (70%/8%), ascites (20%/0%), bloody vesicles (40%/0%), mucosal purpura (10%/0%) and body weight loss (20%/0%) were higher. The frequencies of renal involvement were high (70%/65%) in both groups. These results indicate both systemic complications and skin lesions have variability in the elderly. Eight of these 10 cases in the elderly had underlying diseases, including malignant tumors in 5 of them. Bacterial infection also proceeded in 9 cases. These results suggest that AP in the elderly may be strongly related to underlying diseases and, therefore, that we should classify it as a dermadrome.
We performed chemical peeling (CP) using glycolic acid at a low dose (5~20%) once a week on 38 patients with acne vulgaris . They had previously been treated with long-term oral administration (6~72 months, average 16.1 months) of antibiotics (DOXY 50~200 mg/day, average dose 95 mg/day). In all 38 patients (10 male, 28 female), the total dose of antibiotics was reduced to an average of 9.2 mg/day after 10 CP treatments. Twenty-eight of them (73.7%) did not require further treatment with antibiotics and were able to stop taking them completely. We also performed the CP-treatment with good results on five patients, including pregnant patients or drug allergic patients, in whom long-term oral antibiotics were not advisable. There were no significant side-effects other than three patients who complained of slight irritation when the 5% glycolic acid peel was performed. CP is an efficient and useful method for the treatment of all stages of acne, and CP using glycolic acid at a low dose is a safe and easy method for dermatologists to perform.
During treatment with several antibiotics for subcutaneous scrotal abscesses and an epidural abscess caused by MRSA infection, a 77-year-old man developed an erythema at the right medial malleolus where an intra-venous indwelling catheter was inserted. Multiple nodules and abscesses rapidly developed on the right lower extremity. Histopathology of a nodule showed neutrophilic abscesses and granulomatous reactions. PAS staining demonstrated fungal elements in the abscess as well as in the granuloma. Candida tropicalis was isolated from the dermal specimen and the ourulent exudate. Fungi did not grow on repetitive cultures of his blood. The plasma level of (1→3) -β-D-glucan, a major structural component of yeast cell walls, was extremely high (2,105 pg/ml, cut off value-11). The skin lesions healed in three months after consecutive treatment with fluconazole, amphotericin B, itraconazole, and terbinafin. The plasma (1→3) -β-D-glucan level rapidly dropped in parallei with the skin symptoms and reached the cut off value after 11 months of treatment. We consider that plasma (1→3) -β-D-glucan level is a valuable marker for the diagnosis and treatment of deep cutaneous mycoses.