[Low-dose prophylactic oral isotretinoin treatment for 18 years in a patient with epidermodysplasia verruciformis and numerous squamous cell carcinomas]. / Niedrig dosierte prophylaktische orale Therapie mit Isotretinoin über 18 Jahre bei einer Patientin mit Epidermodysplasia verruciformis und zahlreichen Plattenepithelkarzinomen.

Epidermodysplasia verruciformis (also known as Lewandowsky-Lutz dysplasia) is an extremely rare autosomal recessive genodermatosis, in which the skin is unusually sensitive to human papilloma viruses (HPV). It is associated with a high risk of developing non-melanocytic skin tumors. Treatment with keratolytic retinoids is currently considered to be the most effective therapy. Retinoids have a broad spectrum of activity and inhibit the growth of squamous cell carcinoma and other malignant tumors. We report the case of an 81-year-old woman who had been receiving prophylactic treatment with oral isotretinoin at a daily dose of 1.0-0.33 mg/kg bodyweight for about 18 years because of her epidermodysplasia verruciformis (HPV types 9 & 57 detected). We observed a reduction of the incidence of squamous cell carcinomas of the skin and presume a causal relationship between the treatment with this retinoid and the reduction of squamous cell carcinomas of the skin in this patient.

Progress of multiple cutaneous and subcutaneous melanoma metastases of the face during imiquimod treatment.

A 68-year-old man developed a local recurrence with multiple cutaneous and subcutaneous nodules three months after excision of a primary malignant melanoma of the temple. Despite extensive surgery and adjuvant irradiation, another local recurrence occurred. Following further local progression during dacarbazine chemotherapy, topical treatment with imiquimod was begun and the chemotherapy was changed to fotemustine. During this treatment further local progression occurred and two months later regional lymph node and distant metastasis were detected. The patient died from his tumor disease eighteen months after the first diagnosis of malignant melanoma.

Novel topical therapy for mild-to-moderate plaque psoriasis: focus on calcitriol.

The benefits of vitamin D derivatives for the treatment of chronic plaque psoriasis are well documented. Of importance is how compatible they are with, and how they compare to, other established treatments for psoriasis. This paper reviews 15 studies with calcitriol 3 µg g(-1) ointment (Silkis(®)/Vectical(™) ointment; Galderma Laboratories) applied twice daily for up to 52 weeks. The ointment was found to be effective and safe for the treatment of mild-to-moderate plaque psoriasis including sensitive skin areas. Calcitriol 3 µg g(-1) ointment was found to be as effective as, or even superior to, other established treatment modalities and more highly tolerated than other local treatment modalities in most studies' comparisons. It could be combined with other psoriasis treatment modalities such as ultraviolet B phototherapy or topical corticosteroids in order to achieve a faster response and in order to reduce the risk of adverse events.

Chronic graft-versus-host-disease-like dermopathy in a child with CD4+ cell microchimerism.

We report the case of an 11-year-old boy suffering from a severe progressive chronic skin disease with clinical features of progressive systemic scleroderma, systemic lupus erythematosus and dermatomyositis. Skin biopsies revealed fibrosis and lichenoid changes and muscle biopsy a myositis. Immunohistology of the skin showed a lichen-ruber-like pattern. Despite repeated extensive investigations, no autoantibodies were detectable. Some of these findings looked like those described in juvenile dermatomyositis. Finally, it could be demonstrated that the boy showed microchimerism with approximately 1% maternal CD4+ lymphocytes in his peripheral blood leukocytes. Furthermore maternal cells could be demonstrated in inflamed muscle tissue. So a graft-versus-host-disease-like pathomechanism appears to be likely. Several systemic therapies have been used with limited success to improve the condition including corticosteroids, azathioprine, cyclosporine A and mycophenolate mofetil. A distinct improvement of erythemas and sclerosis could be achieved by means of low-dose UVA1 phototherapy which was applied with escalating single doses of 3-12 J/cm2 for 35 consecutive days.

Intralesional recombinant interferon beta-1a in the treatment of basal cell carcinoma: results of an open-label multicentre study.

Although effective conventional therapies are available to treat basal cell carcinoma (BCC), undesirable side effects, including scarring, and in some cases permanent damage, often occur in problematic areas of the body, especially around the eyes, mouth, and cartilage of the nose and ears. In previous studies, intratumoural injection of recombinant interferon beta-1a (rIFN-beta-1a) has been shown to result in complete remission (CR) in 47% to 86% of patients with BETACC. The primary objective of the study was to determine the response rate to rIFN-beta-1a, in a larger BETACC patient population. Secondary objectives included evaluating the effect of tumour type/size on response as well as residues, cosmetic results, and relapse rate after CR. The safety profile of intratumoural rIFN-beta-1a in BETACC patients was also evaluated. This was an open-label, multicentre study involving 139 patients with BETACC (diameter between 5.0 and 20 mm). Intratumoural injections of rIFN-beta-1a (1.0 x 106 IU) were administered three times a week for 3 weeks. The response was determined 16 weeks after start of treatment and the status of patients was followed for up to 5 years. At 16 weeks, the response rate to intratumoural rIFN-beta-1a was 66.9% (95% CI, range 58.2-74.8%). There was no significant difference between the response rates for patients with solid or other BETACC tumour types. Similarly, tumour size did not significantly affect the response rate. The cosmetic result of treatment was rated as good or very good in 83% of responders. The relapse rate after CR was 4.5% (median follow-up 2 years). All patients showed local inflammatory reactions, which were generally considered to be the adverse drug reactions (ADRs). Systemic ADRs mostly consisted of flu-like symptoms and occurred in 32/139 patients. No ADRs were considered to be the serious. These results show that intratumoural injections of rIFN-beta-1a are effective in the treatment of BETACC in the majority of patients. In addition, rIFN-beta-1a is safe and generally well tolerated. rIFN-beta-1a represents an effective alternative treatment for BETACC.