Gunhild Lange Wantzin

Familial lichen planus. More frequent than previously suggested

In a follow-up study of 140 patients with lichen planus we found fifteen patients with a clinically and/or histologically verified family history of the disease, which gives a percentage much higher than that found in previous studies. The reason for this discrepancy might be caused by difficulties in the detection of the disease in family members. Familial lichen planus was found to be different from nonfamilial lichen planus, especially in its effect on young persons and in being a more widespread type with a tendency to result in relapse. The high incidence of familial lichen planus supports the hypothesis that genetic factors are of etiologic importance in lichen planus.

Dose response and time course for induction of T6− DR+ human epidermal antigen-presenting cells by in vivo ultraviolet A, B, and C irradiation

In vivo ultraviolet (UV) exposure of human skin abrogates the antigen-presenting function of T6+ DR+ Langerhans cells and induces the appearance of antigen-presenting T6- DR+ epidermal melanophages. UV-exposed epidermal cells containing T6- DR+ epidermal antigen-presenting cells, in contrast to unexposed epidermal cells containing T6+ DR+ Langerhans cells, potently activate autoreactive regulatory T cells in the absence of exogenous antigens. Autoreactive T cells may be important for regulation of other immune responses such as those which occur in photosensitive lupus erythematosus and in immune surveillance of UV-induced skin cancers. It is therefore imperative to determine the factors that govern their appearance in the skin. It was found that UVB and UVC, but not UVA, induced a dose-dependent appearance of T6- DR+ epidermal melanophages. The optimal time of appearance was 2 or 3 days after UVB and UVC exposure. In contrast, UVA was a poor inducer of T6- DR+ cells at all doses and all time points tested. Although UVA was a poor inducer of T6- DR+ epidermal cells, UVA radiation resulted in depletion of T6+ DR+ Langerhans cells from the epidermis, as did UVB and UVC radiation. This differential effect of UV wave bands on the immunocompetent cells in human skin may be related to the greater potential of UVB exposure to induce skin cancers and to exacerbate systemic lupus erythematosus.

Early diagnosis of cutaneous T-cell lymphoma by DNA flow cytometry on skin biopsies

Seven patients with mycosis fungoides early plaque stage with nondiagnostic histology had single‐cell DNA content measured by flow cytometry for estimation of clonal ploidy. A total of 63 skin specimens were examined by histology and DNA measurements concurrently during the course of the disease. In addition, six patients had blood samples studied. All seven patients demonstrated aneuploid DNA histograms when the specimens were obtained from skin lesions. In 36 specimens the aneuploid peaks were hyperdiploid. By sequential studies one patient demonstrated two different aneuploid cell clones, one located in the hyperdiploid region and one located in the hypotetraploid region. All patients developed mycosis fungoides which were histologically confirmed, and the time from first aneuploid DNA histogram until diagnostic histology varied from 5 to 21 months (median, 12 months). In six of the patients a normal diploid DNA histogram was found of peripheral blood lymphocytes. The finding of aneuploidy in patients with early mycosis fungoides who still have a nondiagnostic histology emphasizes the value of flow cytometry as a complementary diagnostic aid which facilitates an early diagnosis.

Extracutaneous Spreading with Fatal Outcome of Mycosis fungoides in a Patient Treated with Ciclosporin A: A Word of Caution

A patient with mycosis fungoides in the tumor stage was treated with ciclosporin A orally in daily doses of 5 mg/kg body weight for 5 weeks. Three weeks after cessation of treatment the patient died with dissemination of the cutaneous lymphoma to the lungs, myocardium, liver, pancreas, kidneys, adrenals, and stomach. Treatment with ciclosporin A may have contributed to the death of this patient.

Increased number of circulating suppressor T-Lymphocytes in Sun-induced multiple skin cancers

The distribution of peripheral lymphocyte subsets was studied in fifteen patients with multiple nonmel‐anoma skin cancers, selected according to history of ultraviolet (UV) or X‐ray exposure. The skin cancer was associated with previous heavy exposure to UV light in seven patients, and past exposure to x‐rays in eight patients. In the UV group, the helper T‐lymphocytes/suppressor T‐lymphocytes (Th/Ts) ratio was abnormally low (P < 0.01) compared with the ratios of the x‐ray and control groups. The low Th/Ts ratio was associated with an absolute increase in the number of Ts. This suggests that heavy sun exposure may cause a permanent increase in the number of Ts in certain persons. These extra T‐lymphocytes may in turn prevent immune rejection of transformed keratinocytes.