Bullous SLE: response to methotrexate and relationship with disease activity.

We report on a case of 40-year-old female with systemic lupus erythematosus (SLE) who developed a severe bullous eruption on sun-exposed areas. The bullous manifestation was associated with a flare of lupus serologies, whereas the previous manifestations of the disease were quiescent. Due to prior intolerance to many drugs, she was given oral methotrexate (10 mg/week). The drug administration was followed by a rapid and full resolution of cutaneous lesions. Five months later she developed a class III lupus nephritis. Our case is the first report of efficacy of methotrexate in bullous SLE and rises the question of the relationship between bullous eruption and SLE activity.

Florid cutaneous and mucosal papillomatosis with acanthosis nigricans revealing a primary lung cancer.

This is the report of an 80-year-old patient with diffuse brownish hyperpigmentation and velvety thickening of the skin with onset 1 year before. Warty lesions on his limbs were present as well as papillomatous and verrucous lesions on his lips, mouth and eyelid conjunctivae with hyperkeratosis of the nipples. Biopsies, performed at different sites, showed histological pictures consistent with a diagnosis of acanthosis nigricans (AN) with florid cutaneous and mucosal papillomatosis. This type of AN is frequently associated with internal malignancy. In our patient serum levels of tissue polypeptide antigen, carcinoembryonic antigen, cytokeratin fragment and squamous cell carcinoma antigen were high and chest computed tomography scan indicated a large tumour infiltrating the right lung and extending to the mediastinum. Cytological examination of bronchial drainage revealed the presence of neoplastic cells, non-small cell type carcinoma. The most frequent cancer associated with malignant AN is gastric adenocarcinoma. Lung tumour has rarely been reported with AN. Malignant AN is sometimes associated with other cutaneous and mucosal warty lesions, as in our patient. These various skin and mucosal lesions are the expression of a systemic epithelial disorder and may help clinicians to suspect a malignant form of AN.

Functional profile of expanded suppressor/cytotoxic lymphocyte population in a patient with actinic reticuloid.

This study was undertaken to gain insight into the functional properties of the expanded suppressor/cytotoxic lymphocytes characteristically found in actinic reticuloid. Peripheral blood cells, either whole mononuclear cell populations or selectively enriched populations, were obtained from a patient with well-established actinic reticuloid. The patient had an expansion of circulating T lymphocytes expressing the suppressor/cytotoxic phenotype, i.e., CD3+, CD4-, CD8+. A proportion of these cells also expressed the Fc receptor for IgG. Functional studies, including pokeweed mitogen-driven immunoglobulin synthesis, mitogen and alloantigen response, natural killer and antibody-dependent cellular cytotoxicity were within normal ranges and suggested a polyclonal rather than monoclonal expansion. The present functional data extend previous phenotypic studies and support the hypothesis that a chronic reactive immunoregulatory disorder is involved in actinic reticuloid, as has been hypothesized for other T cell chronic proliferations.

Basal keratinocyte subsets: ultrastructural and morphometric features.

Basal keratinocytes reportedly comprise two ultrastructurally recognizable populations, usually referred to as serrated basal keratinocytes (SBK) and non-serrated basal keratinocytes (NSBK). The former are responsible for dermal-epidermal anchoring, whereas the latter serve in epidermal turnover (stem cells). The size and shape of these cells were investigated by electron microscopy and computer-assisted morphometric analysis. The results showed that perimeters of both the nucleus and cytoplasm of NSBK were consistently smaller than those of SBK. The shape factor (contour index) was lower in NSBK, indicating a more regular membrane surface. Present quantitative results substantiate previous subjective reports on the ultrastructural differences between NSBK and SBK and provide additional evidence that the basal cell layer in human epidermis is actually made up of at least two types of morphologically distinct keratinocytes.

Trichothiodystrophy without photosensitivity. Biochemical, ultrastructural and DNA repair studies.

A case of trichothiodystrophy (TTD) without photosensitivity is reported in an 8-year-old girl. Electron microscopic (EM) examination of the keratinocytes showed fibrillary bundles in the cytoplasm thinner and less electron dense than those of the normal cells and large membrane-bound vacuoles filled with granular-filamentous material. These findings could indicate a disturbance in the protein metabolism in tissues of ectodermal origin, explaining also the functional abnormalities of the central nervous system in TTD patients. The results of cellular DNA repair studies after UV irradiation in cultured fibroblasts showed normal levels of unscheduled DNA synthesis. This finding indicates that UV hypersensitivity is not constantly present in TTD.

[Atrophic zoniform nevus]. / Nevo atrofico zoniforme.

The first, in our knowledge, case of naevus atrophicus zoniforme in italian literature is here reported. A 13 year old girl had a thirteen years history of involved numerous depressed areas of skin of the right anterior region of the chest and of the right upper arm. The lesions were round, grey-bluish, smooth, depressed, completely asymptomatic. No inflammatory changes were noticed in affected patches nor in surrounding normal skin. The histologic changes were compatible with dermal atrophy: epidermis was quite normal; dermis war very thin; the thickness of hypodermis was increased. Some large nervous structures were visible within deep dermis. Histochemical changes mainly involved elastic fibers, whose thickness was considerably increased. The diagnosis of naevus atrophicus zoniforme is discussed, in comparison to other idiopathic atrophies. In conclusion, the main characters of naevus atrophicus zoniforme, according to literature data, are confirmed in our case: 1) congenital appearance 2) zoniforme distribution 3) dermal atrophy 4) thickening of elastic fibers 5) occurrence of large nervous structures in the dermis.

Studies on dapsone induced haemolytic anaemia. I. Methaemoglobin production and G-6-PD activity in correlation with dapsone dosage.

The present study was undertaken on the hypothesis that methaemoglobin production and haemolytic anaemia following dapsone administration could be ascribed to an impairment of glucose-6-phosphate dehydrogenase-enzymatic activity. Analysis of the kinetic parameters of the G-6-PD (Vmax and KM) was performed in ten patients, normal with respect to G-6-PD, suffering from various dermatoses. It was concluded that haemolytic anaemia after dapsone therapy is not due to a functional impairment of the enzyme. The close relationship between dapsone dosage, methaemoglobin production and anaemia make reasonable the hypothesis that a toxic dapsone derivative (DDS-NHOH) could be responsible for the methaemoglobin formation and the haemolytic anaemia.

In situ identification of mononuclear cells infiltrating cutaneous carcinoma: an immuno-histochemical study.

The present study was undertaken in order to investigate the exact nature of the cells surrounding cutaneous tumours. Formalin-fixed, paraffin-embedded sections were tested with a horse antihuman T lymphocyte serum and with IgG, IgA and IgM rabbit antihuman serum. All the sections were respectively treated with rabbit anti-horse or swine antirabbit peroxidase-labelled serum. The advantages offered by the immunoperoxidase technique are briefly discussed. T cells are in overwhelming proportion in comparison with IgG, IgA and IgM bearing cells. This seems a further demonstration that mononuclear infiltrate surrounding cutaneous carcinoma mainly represents a cell-mediated immune response.