[Multiple basaliomas in pityriasis rubra pilaris]. / Multiple Basaliome bei Pityriasis rubra pilaris.

This is a report of a 73 year old patient with pityriasis rubra pilaris and multiple basal cell carcinomas which especially appeared on the face. One of the tumors was located in the anal and perianal region. This is the first report of basal cell carcinomas in a patient with pityriasis rubra pilaris. Further observations will clarify whether there exists a closer relationship between the presence of basal cell carcinoma and pityriasis rubra pilaris. Therefore more attention should be paid to the existence of basal cell carcinoma in patients with pityriasis rubra pilaris.

Experience with low-dose thalidomide therapy in chronic discoid lupus erythematosus.

BACKGROUND: Low-dose thalidomide therapy (median dose 100 mg/day, 50-200 mg/day) in chronic discoid lupus erythematosus was studied with regard to efficacy, tolerance, and toxicity in 22 patients. Intense contraceptive precautions were taken in women patients of childbearing age. METHODS: An open uncontrolled trial was conducted. Age, the total drug intake, disease duration, extent/severity, and adverse reactions were studied with regard to the final clinical outcome. The follow-up duration was 1.8 years (range 1 month to 3 years). RESULTS: With the exception of age (inverse correlation, P < 0.01), the parameters studied did not influence the final clinical amelioration: complete responders numbered 54.5%, partial responders 22.7%, and 13.6% were withdrawn from the trial with complaints of intolerance. The initial (first month) clinical response correlated significantly with the final one (P < 0.01). Drowsiness (40.9%) and somnolence (18.2%) were the most common side-effects, without affecting seriously the daily life of the participants. No case of real neurotoxicity was confirmed. Relapses occurred within 39.4 +/- 21.4 days after drug withdrawal, presenting a milder clinical picture. CONCLUSIONS: In the context of a predictable final outcome, low-dose thalidomide therapy is effective as an alternative choice in cases resistant to the usual treatment.

Current profile of active leprosy in Greece; a five-year retrospective study (1988-1992).

The epidemiological characteristics of newly diagnosed, active leprosy cases (incidence, N = 16 Greeks and 4 expatriates) and relapsed cases (recurrences, N = 25, all Greeks) were studied. Most of the cases were multibacillary, over 50% being lepromatous. The relapses were analyzed by sex, disease duration and residence (rural or urban). Most of the newly diagnosed cases presented with nonreactional skin lesions (70%). The relapses were self-reported and detected mainly because of type 2 leprosy reactions (56%). The main source of the infection for new cases was members of their former extended family. The statistical trend of leprosy in Greece is a continuing decline in a country which already has a very low endemicity.

Sunlight and air temperature affect autoantibody activity and skin involvement of bullous pemphigoid.

BACKGROUND AND DESIGN: Sunlight and air temperature are environmental parameters with considerable influence upon autoimmunity. Their impact on idiopathic bullous pemphigoid (BP) was studied 1) in a prospective series of 27 patients with regard to clinical activity and 2) in a retrospective series of 232 cases during a 7-year period with regard to immunoserology. RESULTS: Skin involvement and the onset of disease showed an immediate and cumulative pattern of response to sunlight and air temperature. Immunoserologic analysis showed a strong correlation of autoantibody expressivity with these two environmental measurements. CONCLUSIONS: This study verifies previous sporadic clinical and experimental data concerning the influence of environmental factors on BP. Accordingly, preventive measures against exposure to sunlight and high temperature are recommended.

Immunohistochemistry in histoid leprosy.

BACKGROUND: Histoid leprosy is a rare form of multibacillary leprosy as the result of secondary or even primary resistance to dapsone. The etiopathogenesis has not been clarified up to now. METHODS: An immunohistochemical study was carried out for the expression of various markers on epidermal and dermal cell populations using sections of frozen skin specimens from 5 patients with histoid leprosy as compared to specimens from 7 tuberculoid and 7 lepromatous patients. RESULTS: Dendritic epidermal cells, identified by monoclonal antibodies against CD1, HLA-DR, CD45, and CD36, were found reduced in histoid leprosy as compared to both tuberculoid and lepromatous groups. A gradual reduction of keratinocytic HLA-DR expression from tuberculoid to lepromatous to histoid leprosy was observed. The pattern of CD36, CD4, and CD8 expression of lymphomonocytic cells in the dermis of histoid lesions was similar to that of tuberculoid leprosy, but without the formation of an organized granuloma. CD45+ cells as well as activated lymphocytic cells, expressed by the activation immunophenotype (CD1, HLA-DR, CD25, CD71, EGF-R) were found frequently in all groups. CONCLUSIONS: The in situ immunohistochemical findings support a modified hypersensitivity reaction of the cellular type that results in an inhibition of the lesional expansion, but not in the destruction of the bacilli within the histoid lesion.

Re-evaluation of the natural course of bullous pemphigoid. A prospective study.

BACKGROUND: Few studies of prospectively collected data regarding the natural course of bullous pemphigoid have been performed. METHODS: The following factors were combined both quantitatively and qualitatively to obtain results: gender, clinical activity based on the estimation of the body area involvement, disease duration, relapses, coexistence with other disease states, and serology Twenty-seven consecutive patients were followed up for 1 year. RESULTS: Most disease activity (85.2%) is exhibited in the first year after onset. There is a lack of parameters with clear predictive significance. The extent and severity of skin involvement are equally distributed between the sexes and not affected by the disease duration. The generalized form of the disease is predominant (86.5%). The average clinical activity in relapses within the year of follow-up is 48% of the initially observed attack. The probability for recurrence is higher in seropositive patients (overall 37%). Coexistence with other disease states seems to be a random chance event. CONCLUSIONS: The lack of parameters with predictive importance underlines the necessity of a thorough follow-up to prevent treatment-related complications in elderly patients.