Notes: Mucous membrane pemphigoid is a rare, chronic autoimmune disease characterized by subepidermal blistering and scarring, predominantly affecting mucous membranes. Ocular involvement frequently occurs and often represents the only manifestation of the disease. We describe a 62-year-old woman with a bilateral 18-month duration of conjunctival hyperaemia, associated with erythema and oedema of the eyelids, lacking any typical ocular signs of mucous membrane pemphigoid such as subconjuctival fibrosis and scarring. Histology was not significant. Direct immunofluorescence of the conjunctiva showed IgG, IgA and complement deposition along the basement membrane zone. Immunoprecipitation analysis of affinity purified laminin-5 revealed a band consistent with the β3 chain of laminin-5. This represents the first case of pure ocular mucous membrane pemphigoid associated with antilaminin-5 antibodies.

Notes: Papillon-Lefevre syndrome is a rare disorder of keratinization inherited as an autosomal recessive trait. It is characterized by thickening of palms and soles, perindonlopathia, tendency to pyogenic skin infections and sometimes mental impairment. The authors report Papillon-Lefevre syndrome in two sisters in whom the familial pedigree shows the autosomal recessive inheritance of the trait. Two oilier important disorders of keratinization transmitted by an autosomal recessive gene. Richner-Hanhart syndrome and Mal de Meleda, are excluded by clinical and metabolic criteria. Systemic therapy with etretinate and acitretin could not be performed because one of the patients has a hepatopathy and the other refuses the treatment. Application of local keratolytics is giving quite good results.

Notes: We report a 28-year-old male with a voluminous growth of the tongue, present for 6 months. The histological examination revealed a squamous cell carcinoma. The patient was also affected by oral leukoplakia, nail dystrophy, reticulated poikiloderma of the neck and hyperkeratosis of palms and soles. On the basis of clinical features and histological findings, as well as findings from the family, the diagnosis of dyskeratosis congenita (DKC) was made.

Notes: A 35-year-old woman presented with a 8-month history of scaling, hyperkeratotic and fissured lesions of the fingertips of the first three fingers of both the hands. She referred the healing of the dermatitis during the summer holidays. She was employed in a small firm where she was used to glue together silver components with glass ones of decorative pieces. For this aim, she applied a glue (Loxeal UV 30–20) cured by exposition to UV light coming from a proper lamp. The material safety data sheet (MSDS) indicated that the glue contained hydroxyethyl methacrylate (HEMA) and hydroxypropyl methacrylate (HPMA). Patch tests performed with the standard SIDAPA series gave negative results; patch tests carried out with an additional one (acrylic adhesive series) showed positive reactions towards glue components (HEMA,HPMA) and towards other acrylates (possible cross-reacting). An inspection performed in the work-place showed that the patient was in contact with the glue not only when she applied it on the components, but particularly when she handled the bottle cap (splashed with the glue) in its opening and closing. After the changing of her occupation, the patient has not presented relapse of the dermatitis. The UV-cured acrylic resins are known for some time to be a frequent cause of occupational allergic contact dermatitis in dentists and in printing industry. The case reported shows a different exposition source towards these resins, i.e. from UV-cured acrylate adhesive employed for sticking metal pieces with glass ones.

Notes: The dermal infiltrates of four patients with the Sézary syndrome were studied by electron microscopy and the data were evaluated quantitatively. The nuclear contour index of lymphocytes was calculated, and many tumour cells had an index greater than 6.5. Dendritic cells were found in all cases. The dendritic cells contained smooth and rough endoplasmic reticulum, moderately well-developed Golgi apparatus, scanty lysosomes and many thin and intermediate filaments; their surface was scalloped with numerous vesicles. Birbeck granules were not found in the cytoplasm of dendritic cells. Dendritic cells comprised 24% of infiltrating cells and were interspersed with lymphocytes; 75% of the lymphocytes were in contact with dendritic cells; 35% of the lymphocytes in contact with dendritic cells had a nuclear contour index higher than 6.5 and 76% had a nuclear contour index higher than 5. The data strongly suggest a functional relationship between lymphocytes and dendritic cells in the dermal infiltrate of Sézary syndrome. They are discussed in relation to the hypothesis that the disease is a consequence of chronic immune stimulation.

Notes: A 44-year-old white woman was admitted to our hospital for the investigation of many roundish, pale-colored, nodular lesions of different sizes, ranging from a few millimeters to some centimeters, symmetrically grouped on the shoulders (〈link href="#f1"〉Fig. 1), trunk, upper limbs, and abdomen. The lesions first appeared at 20 years of age on the upper chest around the clavicular region, and thereafter gradually increased on the trunk and upper limbs; they were completely asymptomatic, appearing on normal skin without a previous history of inflammation and/or trauma. No scarring or atrophy was evident. The smaller lesions exhibited a miliaria-like shape, being evident in particular on stretching of the skin; the larger ones showed a firm-elastic density, no adhesion to surrounding tissue, and deep localization in the skin. No hypopigmented macules or sebaceous adenoma-like lesions were present. Skin biopsy of a nodular lesion from the right shoulder revealed a normal epidermis and a dermis filled with bundles of dense and coarse collagen fibers (〈link href="#f2"〉Fig. 2). Elastic fibers appeared thin and decreased in number under Verhoeff–van Gieson stain (〈link href="#f3"〉Fig. 3). A diagnosis of cutaneous collagenoma was proposed. Routine laboratory investigations were within the normal range. Chest and skeleton (hands, pelvis, and long bones) X-ray, electrocardiogram, and echocardiogram showed no abnormality.〈figure xml:id="f1"〉1〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD1509:IJD_1509_f1"/〉Nodular papules localized on the right shoulder〈figure xml:id="f2"〉2〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD1509:IJD_1509_f2"/〉Histology of a lesion taken from the right shoulder. The picture demonstrates dense and coarse collagen bundles (hematoxylin and eosin stain; original magnification, × 200)〈figure xml:id="f3"〉3〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD1509:IJD_1509_f3"/〉Elastic fibers are clearly diminished in number, thin, and fragmented (Verhoeff–van Gieson stain; original magnification, × 400)In order to evaluate a possible familial cutaneous collagenoma, the 19-year-old son of the patient was also examined. He had a 3-month history of numerous, asymptomatic, skin-colored nodules, ranging from 0.5 to 2 cm, on the upper back. Skin biopsy of a nodular lesion showed the same features as observed in the mother's biopsy. Routine blood and serum tests and X-ray examinations were within normal limits. Electrocardiogram revealed an early R-wave transition, and echocardiogram showed a left ventricular dilation with thickened aorta; aortic steno-insufficiency was diagnosed under cardiologic examination. The diagnosis of familial collagenoma was confirmed, and similar cutaneous lesions were found in the mother's grandmother and aunt.

Notes: In this study the clinico-pathological and immunohistological features, the methods of treatment and follow-up data of 11 patients with follicular centre-cell (B-cell) lymphoma primarily presenting in the skin are reported. All the patients had nodular, tumorous and/or papulonodular skin lesions on the trunk. In nine patients the disease was confined to a circumscribed area of the back. Small papulonodular or plaque-like lesions, as well as large nodules or tumours, were biopsied in six of 11 patients. No clear-cut correlation between the age and clinical morphology of the lesions and their histological growth pattern was found. Interestingly, however, a different immuno-architectural pattern was observed in large, late lesions compared to small, early lesions. Initial treatment consisted of orthovolt radiotherapy (in two patients associated with surgical excision), resulting in complete remission in all patients. Only one patient developed extracutaneous disease, which was limited to a single drainage lymph node appearing simultaneously with a cutaneous relapse. Five other patients had recurrent disease in the skin close to the initial site. The median disease-free period was 15·5 months. On relapse, radiotherapy alone or in combination with short courses of chemotherapy was performed. This resulted in a second complete remission. All the patients are still alive and in complete remission, with a median survival of 37 months. These results confirm the favourable prognosis of patients affected with primary cutaneous follicular centre-cell lymphoma limited to the trunk. Orthovolt radiotherapy proved to be the most suitable treatment for both initial lesions and relapses limited to the skin.

Notes: Skin biopsies from seven patients with mycosis fungoides in various clinical stages (patches, plaques, nodules) were studied immunohistochemically and ultrastructurally, with the aim of investigating and quantifying the distribution of Langerhans cells and their relationships to mycosis cells. Our findings have revealed that in patches and plaques both Langerhans cells and mycosis cells were numerous in the epidermis. Notwithstanding this, in all the specimens examined, only one Langerhans cell forming close contact with a mycosis cell was detected. In the nodules, Langerhans cells and mycosis cells were sparse in the epidermis and no contacts were seen between them. Moreover, in all the patients studied, only a single Langerhans cell was found in the dermal infiltrate without any closely related mycosis cells. Conversely, numerous interdigitating cells have been found in the dermis of patches and plaques, often lightly adhering to mycosis cells. In the nodule, a few scattered interdigitating cells were seen, but often these had close contacts with neoplastic lymphoid cells. These findings indicate that close apposition between Langerhans cells and mycosis cells, which led previous authors to hypothesize a persistent stimulatory action of Langerhans cells on T lymphocytes, eventually leading to the malignant transformation of the latter, is unusual in mycosis fungoides. Therefore, if such a pathogenic role may be attributed to accessory cells in mycosis fungoides it is more probably exerted by dermal interdigitating cells and not by Langerhans cells, as previously proposed.

Notes: We have investigated the occurrence, immunohistochemical profile, ultrastructural features and relationships to lymphocytes of the non-lymphoid accessory cells in the dermal infiltrate of five patients affected by B celt lymphoma with secondary involvement of the skin. Typical nonlymphoid accessory cells were found in all cases. Most of these cells had ultrastructural features which resembled those of the poorly differentiated dendritic reticulum cells described in follicular lymphomas of the lymph nodes. The immunohistochemical findings of DRC-1 +, C3b r+ dendritic cells often arranged in follicular-like structures with neoplastic B cells and only few, scattered OKM1+, OKM5+ mononuclear phagocytes support the hypothesis that the vast majority of the non-lymphoid cells observed in our cases were poorly differentiated dendritic reliculum cells.These results and previously published reports indicate that the organization of the dermal infiltrate of B cell lymphomas tends to reproduce the typical arrangement of the B zone of the lymphoid tissue, although with a lesser degree of differentiation, similar to that observed in lymph node follicular lymphomas.