For adult patients with atopic dermatitis it was shown that 1 year of house dust mite avoidance reduced the allergen exposure, but an improvement of overall disease activity was not demonstrated.

Since it has been hypothesized that atopic dermatitis represents a cellular immune reaction to exogenous aeroallergens, we investigated whether lesional skin contains allergen-specific T-cells and which lymphokines they might secrete. Using phytohaemagglutinin or grass pollen for the cloning procedure, we established a series of T-cell lines from the skin of two patients. When rechallenged with the allergen, three out of 12 dermal lines which had been cloned with the pollen extract and three out of 20 epidermal lines cloned with PHA were found to proliferate specifically. With one exception, allergen-specific lines were CD4+, CD8-, alpha/beta receptor +. The reaction pattern to the single components of the grass allergen extract was assessed with the line UH-D3. Further, the proliferative response to Lolium perennis was inhibited by HLA-DR antibody, indicating its dependence on structures of the MHC class II complex. Only one out of four CD4+ allergen-reactive lines secreted considerable interferon-gamma activity but all secreted interleukin-4. The relative predominance of IL-4 points to a possible role of skin-derived T-cells in the synthesis of IgE. The identification of allergen-specific T-cells in lesional skin of patients with atopic dermatitis is consistent with the hypothesis that their dermatitis represents a T-cell-mediated immune reaction.

Paraneoplastic pemphigus (PP) is an autoimmune disease, which is frequently associated with non-Hodgkin's lymphoma. Autoantibodies against components of the cytoplasmic plaque of epithelial desmosomes are usually present in the sera and are believed to play a major pathogenic part in acantholysis and suprabasal epidermal blistering. However, another typical histological feature of PP, interface dermatitis with keratinocyte dyskeratosis, is shared with skin diseases that involve epithelial damage mediated by T cells. Here, we present the detailed characterization of the cutaneous T-cell response in a patient with PP and demonstrate a selective epidermal accumulation of activated CD8+ T cells together with an increased local production of interferon-gamma and tumour necrosis factor-alpha, and a strong expression of HLA-DR and ICAM-1 on keratinocytes. Apoptosis was identified as a key mechanism of keratinocyte death, and appeared independent of the FAS/FAS ligand (FAS-L) pathway, as epidermal expression of FAS was not increased compared with normal skin, and FAS-L was undetectable on the protein and mRNA level. Triple therapy with high-dose corticosteroids, cyclophosphamide and intravenous immunoglobulins reduced levels of pemphigus-like autoantibodies and reversed the cutaneous inflammatory reaction leading to long-standing clinical remission. Our findings support the concept of a major contribution of cytotoxic T lymphocytes to the immunopathology of paraneoplastic pemphigus.

The Schimmelpenning-Feuerstein-Mims syndrome (SFM syndrome) is a rare and variable multisystem defect consisting of congenital, extensive linear nevus sebaceus and associated abnormalities in different neuroectodermal organ systems. We present the history of a 52-year-old female patient with disproportionate hyposomia and asymmetric constitution. From birth she suffered from a right-sided, extensive nevus sebaceus following Blaschko’s lines extending on the scalp, neck, right arm and trunk. At the age of 5 years, she developed a generalized growth retardation, along with deformations of bones. At the age of 11, hypophosphatemic rickets was diagnosed causing this growth retardation. Moreover, the patient developed a precocious puberty at the age of 9 years. When we saw the patient 40 years after the diagnosis had been made, phosphaturia had returned to normal. Specific therapy of hypophosphatemic rickets is straightforward and efficient in preventing late complications like growth retardation. We suggest to conduct appropriate laboratory tests in early childhood in patients with an extensive systematized sebaceous nevus or with additional signs of growth retardation or skeletal involvement, in order to exclude hypophosphatemic rickets associated with SFM syndrome.

The purpose of this randomized trial was to evaluate the efficacy of combination chemoimmunotherapy compared with chemotherapy alone. A total of 124 patients were randomized to receive intravenous cisplatin (35 mg m 72 , days 1 -3), carmustine (150 mg m 72 , day 1, cycles 1 and 3 only), dacarbacine (220 mg m 72 , days 1 -3) and oral tamoxifen (20 mg m 72 , daily) in combination with (n=64) or without (n=60) sequential subcutaneous IL-2 and IFN-a. In those patients who received sequential immunotherapy, each cycle of chemotherapy was followed by outpatient s.c. IL-2 (10610 6 IU m 72 , days 3 -5, week 4; 5610 6 IU m 72 , days 1, 3, 5, week 5) and s.c. IFN-a (5610 6 IU m 72 , day 1, week 4; days 1, 3, 5, week 5). The overall response rate of patients treated with the combination of chemotherapy and IL-2/IFN-a was 34.3% with seven complete responses (10.9%) and 15 partial responses (23.4%). In patients treated with chemotherapy, only, the overall response rate was 29.9% with eight complete responses (13.3%) and 10 partial responses (16.6%). There was no significant difference in median progression free survival (0 months vs 4 months) and in median overall survival (12 months vs 13 months) for combined chemoimmunotherapy and for chemotherapy, respectively.