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Savalia savaglia is an ecosystem engineer listed as Near-Threatened by the IUCN, even though effective management and proper monitoring efforts to assess its distribution is still lacking. The record of large, long-established colonies can indicate the occurrence of areas with limited human local pressure. These areas may be considered as proxies for the creation of baselines of reference useful to design restoration strategies. The aim of this work was to update the distribution of S. savaglia Mediterranean populations to develop an Ecological Niche Model, highlighting potential areas for future monitoring programs. Occurrence data were collected and harmonized into a single dataset using the scientific literature and validated observations to feed a presence-only MaxEnt model, obtaining a basin-level potential distribution of the species. The results of our study can support decision-makers in marine spatial planning measures including the preservation of mesophotic environments and prioritizing areas for conservation., (Copyright © 2022. Published by Elsevier Ltd.)

Background: Pemphigus vulgaris is an autoimmune disease characterized by blisters and widespread erosions, involving skin and mucous membranes, caused by autoantibodies to desmoglein 1 and 3. This pathology is associated with increased morbidity and mortality if untreated. The treatment of pemphigus vulgaris requires multiple immunosuppressive agents, but often it is particularly resistant., Objective: To evaluate the efficacy and safety of rituximab therapy in refractory pemphigus vulgaris., Methods: Five patients diagnosed as having pemphigus vulgaris were treated with anti-CD20 monoclonal antibody (rituximab). Each patient was treated with rituximab intravenously at a dosage of 375 mg per square metre of body surface area once weekly for 4 weeks., Results: All the patients presented clinical resolution. No adverse effects were observed. It is important to observe the clinical evolution in the future, but our experience is still limited to a short lifetime and follow-up., Conclusion: In our experience rituximab has been an effective and safe treatment for refractory pemphigus vulgaris.

Psoriasis is a common skin disorder that needs a long-term management, not only because, of its prevalence but also because of the profound impact it can have on patients quality of life. Drugs may result in exacerbation of a preexisting psoriasis, in induction of psoriatic lesions on clinically uninvolved skin in patients with psoriasis, or in precipitation of the disease in persons without family history of psoriasis or in predisposed individuals. The knowledge of the drugs that may induce, trigger, or exacerbate the disease is of primary importance in clinical practice. By reviewing the literature, there are many reports on drug-induced psoriasis, but the data are not univocal. We propose, when possible, the use of a probability score from the authors to obtain a better classification and further understanding of drug-induced psoriasis.

Attenuated familial adenomatous polyposis and Muir-Torre syndrome linked to compound biallelic constitutional MYH gene mutations.Peculiar dermatologic manifestations are present in several heritable gastrointestinal disorders. Muir-Torre syndrome (MTS) is a genodermatosis whose peculiar feature is the presence of sebaceous gland tumors associated with visceral malignancies. We describe one patient in whom multiple sebaceous gland tumors were associated with early onset colon and thyroid cancers and attenuated polyposis coli. Her family history was positive for colonic adenomas. She had a daughter presenting with yellow papules in the forehead region developed in the late infancy. Skin and visceral neoplasms were tested for microsatellite instability and immunohistochemical status of mismatch repair (MMR), APC and MYH proteins. The proband colon and skin tumors were microsatellite stable and showed normal expression of MMR proteins. Cytoplasmic expression of MYH protein was revealed in colonic cancer cells. Compound heterozygosity due to biallelic mutations in MYH, R168H and 379delC, was identified in the proband. The 11-year-old daughter was carrier of the monoallelic constitutional mutation 379delC in the MYH gene; in the sister, the R168H MYH gene mutation was detected. This report presents an interesting case of association between MYH-associated polyposis and sebaceous gland tumors. These findings suggest that patients with MTS phenotype that include colonic polyposis should be screened for MYH gene mutations.

Background: Some studies have shown that cutaneous and mucosal melanoma biopsy specimens harbour human papillomavirus (HPV), suggesting that this virus may play a role in development and progression of the tumour., Objectives: To investigate the presence of HPV DNA and the prevalence of different high-risk mucosal HPV genotypes in primary melanoma (PM) and in acquired dysplastic melanocytic naevi (ADMN)., Methods: Fifty-one PMs from 18 men and 33 women (median age 55.5 years), 33 ADMN from 15 men and 18 women (median age 35.1 years) and 20 control skin samples from nine men and 11 women (median age 43.5 years) were studied. All diagnoses were made after histological analysis. HPV DNA analysis was made using two different polymerase chain reaction-enzyme-linked immunosorbent assay (PCR-ELISA) methods, namely MY-PCR and GP-PCR., Results: Using GP-PCR, mucosal HPVs were detected in 14 PMs (27%; P = 0.0166) and eight ADMN (24%; P = 0.0367), while with MY-PCR, mucosal HPVs were found in 11 PMs (22%; P = 0.04) and five ADMN (15%; P not significant). All control skin samples were negative for mucosal HPVs with both DNA amplification procedures., Conclusions: Using our PCR-ELISA methods, the detection of mucosal high-risk HPV genotypes in 24% of precursor lesions (ADMN) and in 27% of PMs adds to the body of evidence indicating a colocalization of mucosal HPV and tumoral melanocytic pathologies.

Serum antibodies against human intracisternal A-type particle (HIAP) endogenous retrovirus have been found to be associated with various autoimmune pathologies. To evaluate the presence of serum antibody reactivity to HIAP proteins in systemic sclerosis, a Western blot analysis was performed on sera from 42 patients with systemic sclerosis, in comparison with 18 sera from patients with primary biliary cirrhosis and 52 healthy subjects. A positive Western blot was found in 55.5% of serum samples from patients with primary biliary cirrhosis and in 66.0% of patients with systemic sclerosis. None of the 52 healthy subjects showed positive results. Although this difference may be attributable either to an autoimmune response to antigenically related cellular proteins or to a specific antibody response to HIAP proteins expressed as an incidental consequence of attendant pathological processes, the high prevalence of antibodies against HIAP proteins demonstrated in patients with systemic sclerosis may be considered a hallmark of this disease.

To determine demographic, epidemiological, clinical features and risk factors of sexually-transmitted diseases (STD) in adolescents and young adults referring to a STD centre in Northern Italy, patients diagnosed with a STD and aged 13-20 years, were prospectively evaluated from the year 1991. Teenagers showed an appreciable and increasing risk of a broad spectrum of STD, and represented 3.6-13.3% of patients yearly diagnosed with a STD in a nine-year period. More than 10% of patients were under the age of 18, and did not report condom use in the six months preceding diagnosis in 96.7% of cases. A significant increase in the female gender was found since 1993 (but our series was massively influenced by the recent immigration of female sex workers, who declared frequent condom use), while male homosexuals and drug addicts represented a minority. When excluding subjects engaged in prostitution, over 50% of patients declared only 0-1 sexual partners during the last six months, but a significant increase in sexual promiscuity was observed over time (1997-1999 versus 1991-1996) (p<.02). Over two thirds of our teenagers never used a condom prior to the diagnosis of STD, and the apparently increased temporal trend to condom utilization proved related only to the recent immigration of sex workers; lack of condom use proved significantly related to the male gender, and a lower education. Non-gonococcal STD and human Papillomavirus infection accounted for over 60% of overall diseases, while the frequency of molluscum contagiosum, gonorrhea, HSV genital ulcer, and Chlamydia disease ranged from 6.1 to 7.4%, and that of syphilis, phthiriasis, trichomoniasis, and HIV infection varied from 1.3 to 4.7%. Non-gonococcal STD and syphilis were increasingly diagnosed over time, especially in immigrant prostitutes (and despite their frequent condom use). According to our surveillance study of teenagers, a number of demographic, epidemiological, and clinical features of STD showed significant variations over time: recent immigration, spread of prostitution, increased sexual promiscuity, and infrequent condom use, are relevant and/or persisting risk factors. A permanent monitoring of STD in young adults and adolescents is strongly warranted, to allow a timely diagnosis and an appropriate treatment, and to plan preventive strategies specifically addressed to this target population, with special attention to immigrants and sex workers.

A case of necrobiotic xanthogranuloma without typical periorbital involvement is described at the ultrastructural level. The patient, a 58-year-old Italian man, presented in 1995 with a brief history of nodulo-papular lesions commencing on the lower limbs, and mild paraproteinemia. During 6 years of follow-up, anemia, neutropenia with marked lymphopenia, and increased ESR were found, while serum cholesterol and triglyceride levels decreased from hyper to hypo values. Systemic diseases, such as diabetes, malignancy, or extracutaneous lesions, often associated with NXG, have not developed. Conventional histology was distinctive for NXG, and immunohistochemistry confirmed that dermal histiocytes were not of Langerhans cell lineage. At ultrastructure, regeneration and degeneration ("regen-degen") features were observed in some individual deep dermal histiocytes, which have not been previously documented in the literature. Identification of giant histiocytes showing 'regen-degen'' aspects might prove to be a useful ultrastructural diagnostic marker for NXG.

In order to assess the relationship between a diagnosis of sexually-transmitted disease (STD), sexual behaviour, condom use, and other social, demographic, and epidemiological variables in a cohort of young adults who referred to a STD centre during a 9-year period, all patients aged 13-20 years were prospectively evaluated, with special attention paid to sexual behaviour, and use of condom or other contraceptive techniques. The 284 assessed young adults represented 6.1% of all patients with a diagnosis of STD: an increasing temporal trend was noticed (from 3. % in 1991, up to 10.4% nel 1999: p<.0001). On the whole, 70.1% of subjects aged 20 years or less never used a condom during the 6 months preceding the diagnosis of STD: only 21.8% of patients reported regular condom use, and 4.9% more subjects referred occasional use, while in the remaining 3.2% of cases other contraceptive methods were employed. Among under age patients (10.6% of study population), the rate of condom use was 3.3% only, while male homosexuals always denied the use of barrier methods. Although a progressive increase in condom use was observed through the study time (from 0% encountered in 1991-1994, to 57.6% of 1999: p<.0001), this phenomenon proved linked only to the massive increase of immigrant sex workers, which occurred since 1997. When excluding from analysis any probable female prostitutes, lack of condom use tested related to the male gender (p<.05), and a low education (p<.04). Over 50% of patients reported 0-1 partners during the last 6 months, even though an increase in sexual promiscuity was observed during the last three years, compared with 1991-1996 (p<.02). Among the 297 different STD episodes (mostly nongonococcal cervicitis-vaginitis and urethritis, and HPV infection), an increasing incidence of nongonococcal STD and syphilis was found, but no correlation was observed between the spectrum of diagnosed STD, sexual behaviour, and condom use. In particular, in 69.9% of the 103 episodes nongonococcal disease occurred despite regular (65 cases), or occasional (7 episodes) condom use. Moreover, no significant relationship was disclosed between STD occurrence, condom use, and other analyzed social, demographic, epidemiological, and clinical variables. According to our survey, an increased risk of STD seems to involve young adults, and to be related to the variation of multiple demographic, epidemiological, and behavioral features. While immigration and prostitution had the major impact during the last three years, sexual promiscuity and infrequent condom use represent persistent; risk factors in this age population. Besides their diagnostic and therapeutic role, STD centres are able to carry out permanent monitoring of STD, as a starting point to plan adequate information campaigns and specific prevention strategies.

Ninety-two patients suffering from herpes zoster were enrolled in a double-blind controlled study aimed at evaluating the efficacy and tolerance of the drug neuramide. Neuramide (N) and placebo (P) were administered to patients intramuscularly twice daily for 28 days as follows: group N + N (patients always treated with neuramide); group N + P (patients treated with neuramide for 1 week, then with placebo); group P + N (patients treated with placebo for 1 week, then with neuramide); group P + P (patients always treated with placebo). During the first week, all patients were also treated with standardized doses of acyclovir. The presence and extent of clinical symptoms were evaluated during the first 4 weeks, while the appearance, degree and duration of postherpetic neuralgia were evaluated both during treatment and over a 6-month follow-up period. There were no significant differences between the four groups of patients when subjective parameters (such as pain and paresis at the lesion site) were examined. However, clinical examination at the end of treatment showed that treatment with neuramide was therapeutic. Indeed, the times for recovery and for regeneration of epithelium were significantly shorter when neuramide was administered for 3 weeks of the treatment period. Furthermore, the change from vesicles to crusts was significantly faster in the neuramide group than in the placebo group. Postherpetic neuritis occurred in the first months of follow-up. However, in groups N + P and P + P, the symptoms lasted throughout the 6-month observation period, while in the other groups this period was shorter. Indeed, there were significant differences (p < 0.05) in terms of the above complications between the following groups: N + N and N + P; N + N and P + P; N + P and P + N; P + N and P + P. No significant differences were observed between the N + N and P + N, or N + P and P + P groups. Taken together, these data demonstrate that neuramide treatment for at least 3 weeks significantly reduces the risk of postherpetic neuritis development.

Pretibial epidermolysis bullosa (PEB) is a rare form of localized epidermolysis bullosa dystrophica (EBD), a heterogeneous group of inherited, blistering diseases characterized by scarring, loss of dermal-epidermal adhesion and altered anchoring fibrils (AF). Mutations in the type VII collagen gene (COL7A1) underlie EBD and in a dominant PEB family a glycine substitution mutation has been identified. We report a 33-year-old man affected by PEB showing abnormal AF and reduced immunostaining for type VII collagen. Mutation search in the COL7A1 gene revealed a 14 bp deletion in the 115 exon-intron boundary (33563del14), which resulted in the in-frame skipping of exon 115 with elimination of 29 amino acids from the pro-alpha1(VII) polypeptide chain. As a consequence, procollagen VII failed to be processed to mature collagen VII and accumulated at the dermal-epidermal junction, as revealed by immunofluorescence staining using a NC-2 domain-specific antibody. The proband's father was a clinically unaffected heterozygous carrier of mutation 33563del14, whereas the maternal pathogenetic mutation has still not been identified. This represents the first report of a recessive deletion mutation in PEB and extends the range of EBD phenotypes associated with mutation 33563del14.

The aim of this study was to evaluate the presence of inflammatory phenomena and elastic fiber phagocytosis in mid-dermal elastolysis. The pathological and ultrastructural features of 5 Caucasian female patients (ranging from 26 to 40 years) with acquired diffuse asymptomatic areas of skin wrinkling have been reviewed. The clinical features of all cases were characteristic of this condition and only in one patient were erythematous urticaria-like, non pruriginous patches also observed. In 4 cases a history of prolonged sun bathing was present and in 3 cases there was a short history of oral contraception. The pathological study confirmed the typical absence of elastic fibers in the midreticular dermis. In two cases elastic fibers were still detectable in the periadnexal dermis. Hematoxylin and eosin sections showed a mild perivascular infiltrate in two cases, while in three patients histiocytes were scattered among collagen bundles. Multinucleated giant cells containing fragmented elastic fibers were detectable in one patient. Ultrastructural analysis revealed large mononuclear cells with phagocytic aspects toward elastic fibers in all cases.

Keratosis lichenoides chronica (KLC) is a rare chronic disorder of keratinization characterized by lichenoid hyperkeratotic papules arranged in a linear pattern, erythematosquamous plaques and seborrhea-like dermatitis on the face. Adults between 20 and 50 years of age are usually affected, but the disease is very uncommon in childhood. Our purpose was to study the clinical and histopathologic findings and course of KLC in one pediatric case. Detailed clinical data were studied. Two punch biopsies were performed and histopathologic features were compared with those of other reported cases of KLC. In our patient, a 4-year-old boy, the clinical features of the lesions did not deviate notably from those of other cases of KLC. The histologic pattern of the papules was typical of KLC, while that of the erythematosquamous plaques showed some dyskeratotic keratinocytes. The histologic pattern of the erythematosquamous lesions is peculiar in our case, whereas only a nonspecific pattern is reported in the literature. The papular and erythematosquamous lesions showed similar histopathologic features suggesting that they could be different degrees of evolution of the same lesion.

Background: Xanthoma disseminatum (XD) is a rare mucocutaneous xanthoma nowadays classified as a benign non-Langerhans cell histiocytosis., Objective: We studied 7 cases of XD with a long follow-up and reviewed the literature in order to compare and discuss previous reports., Methods: We studied clinical manifestations, course, histopathologic, ultrastructural and immunohistochemical findings of 7 cases of XD followed for 1-20 years., Results: We report our findings and compare our cases with those described in the literature., Conclusions: XD and Langerhans cell histiocytosis were distinguished by clinical differences, as well as ultrastructural and immunohistochemical methods. Three clinical patterns of XD appeared to emerge in our group of patients and in the cases reviewed from the literature.

Nail pathology shares some common features with skin pathology, but it also has its own peculiar aspects. The anatomical and physiological characteristics of the nail unit probably play a major role in determining these pathological differences. Although the presence of keratohyaline granules is a normal feature of the skin, there is no granular layer in the normal nail matrix. As a consequence, nail matrix hypergranulosis should be considered a separate entity from skin hypergranulosis. In our review of 150 longitudinal nail biopsy specimens, keratohyaline granules were seen in the nail matrix of 24 cases of lichen planus, 29 cases of spongiotic trachyonychia, 10 cases of psoriasis, and three cases of Hallopeau acrodermatitis. In all cases, the presence of keratohyaline granules was associated with the absence of the normal keratogenous zone. Similar nail matrix features were detectable in three cases of malignant melanoma, two cases of primary systemic amyloidosis, and one case of histiocytoid hemangioma compressing the nail matrix. Our data suggest that inflammatory and compressive insults to the nail matrix cause both disappearance of the keratogenous zone and matrix keratinization with the formation of keratohyaline granules. Skin hypergranulosis reflects a hyperplasia of a normal skin component. In the nail matrix, however, hypergranulosis represents the appearance of structures not normally present. Nail matrix hypergranulosis should be considered a pattern of nail matrix reaction to different inflammatory insults. It is therefore more analogous to epidermal parakeratosis than to epidermal hypergranulosis.

A clinical, histological and ultrastructural study of a 38-year-old woman with generalized progressive hyperpigmentation is reported. Hyperpigmentation started in early infancy, progressed during childhood and has increased to a dark bronze-brown color with 'mottling' in the last 8 years. Histology and electron microscopy revealed epidermal hypermelanosis and 'negroid' distribution of melanosomes in keratinocytes. In our family, autosomal dominant phenotype transmission with 'variable expression' is suggested. The literature on the entity has been reviewed. This case is another rare example of familial progressive hyperpigmentation.

Pseudoxanthoma-elasticum-like papillary dermal elastolysis is a rare acquired idiopathic non-inflammatory elastolytic disorder. This disease, described in aged females, is characterized by asymptomatic skin lesions of the neck and supraclavicular regions clinically resembling pseudoxanthoma elasticum. Histological and ultrastructural examinations show a total loss of the elastic fibres in the papillary dermis. We report another case of this entity in a 69-year-old Italian woman.

The aim of this study was to evaluate the long-term effects of intermittent Cyclosporin A treatment of severe plaque psoriasis. For this purpose we considered the clinical records of 26 patients who had been intermittently treated with Cyclosporin A for 2 to 4 years. All 26 patients had severe plaque-type psoriasis (PASI score > 18) that was unresponsive to conventional treatment. The initial Cyclosporin A dosage was 5 mg/kg/day in 8 cases and 3 mg/kg/day in 18 cases. In all patients, Cyclosporin A treatment was prolonged until complete or nearly complete remission of psoriasis (mean 2 months; range 1-4 months). All patients subsequently underwent a 2-4 months maintenance treatment with Cyclosporin A dosages that were gradually reduced until tapering off. In order to maintain clinical improvement after Cyclosporin A withdrawal, patients were treated with topical steroids, topical tar, emollients and UVA exposure and/or eliotherapy. Cyclosporin A treatment (2.5-3 mg/kg/day) was reintroduced only when clinical relapses reached a PASI score of 12 or more. Duration and dosages of Cyclosporin A cycles were always adapted for the purpose of obtaining an improvement acceptable to the patient (PASI < 8) rather than total clearance of psoriasis. So far, the 26 patients have undergone 3-5 cycles of therapy with low doses of Cyclosporin A. None of these 26 patients interrupted Cyclosporin A treatment because of side effects. In conclusion, in our experience cyclic CyA treatment is effective for the long-term treatment of psoriatic patients.

Congenital symmetrical interdigital hyperkeratosis is a rare disorder described by Frei in 1923, characterized by localized hyperkeratosis of the interdigital spaces of the hands and feet. The authors report the case of a 7-year-old girl affected by this condition. The skin hyperkeratotic lesions appeared localized exclusively to the interdigital spaces of the hands. Histologic examination showed orthokeratotic hyperkeratosis, hypergranulosis and acanthosis. Keratolytics failed to improve the clinical picture.

Background: Risk of basal cell carcinoma (BCC) recurrence appears to be related to tumor location, tumor size, treatment modality, radicalness of excision, and histologic type. Recurrences of BCC on the trunk and extremities are rare, with 97% of all recurrent lesions being located in the head and neck region., Objective: This report concerns a man who had a BCC of the back removed with electrodesiccation and curettage in 1980 at the age of 36. In the following 10 years the patient experienced five recurrences and finally developed infiltration of the thoracic spine that precluded further attempts at radical excision of the neoplasm., Methods and Results: The pathology of the BCC in our patient revealed a number of histologic features that have been associated with aggressive behaviour. These include an infiltrative growth pattern characterized by an irregular and acute tapered profile of the tumor cell groups and fibroblast-rich stroma, infiltrating invading tumour edges, poorly peripheral palisading, nuclear pleomorphism, and perineural invasion., Conclusion: Basal cell carcinomas that occur in sunlight-protected areas of the trunk of middle-aged individuals can occasionally exhibit aggressive and highly invasive features as well as a marked tendency for local uncontrollable recurrences. The prognosis for patients with this variety of BCC is poor, especially when progressive infiltration of deep tissues does not permit, as in our case, any possible further radical surgical procedure.

We present a case of linear reversible non-inflammatory alopecia mucinosa in a 25-year-old Nigerian patient. The patient exhibited an arciform bald patch on the scalp and follicular mucinosis in both lesions. The possible relationships between the unusual linear arrangement of alopecia mucinosa along Blaschko's lines and lichen striatus are discussed.

Five of 10 patients receiving long-term recombinant interferon alpha-2b therapy complained of considerable hair loss. The pull test and the trichogram showed a telogen count consistent with a telogen effluvium. In 2 patients the telogen count progressively improved despite continuing the treatment, whereas in 3 patients the trichogram continued to show an increased telogen count until interferon therapy was discontinued. Pohl-Pinkus hair constrictions were also present in 2 patients.

Two patients showed hypertrophic lichen planus at the sites of previous surgical procedures in their lower extremities after granulomatous reactions. Knots of implanted sutures too close to the epidermis may have caused the inflammatory reaction. Although granulomatous reactions occurring after surgical procedures are fairly common, no cases of lichenoid lesions have been reported previously.

A 37-year-old man had red or brownish papules on his face, chest, and groin, where they coalesced into plaques. Endoscopic examination revealed the presence of small xanthomatous papules on the mucous membrane of the stomach. A biopsy specimen revealed a dense infiltrate of histiocytes, foam cells, Touton giant cells, and inflammatory cells. The diagnosis of non-X histiocytosis was confirmed by immunohistochemical studies of the infiltrate.

The presence of melanocytes in 11 clear cell acanthoma cases was investigated by silver impregnation. Melanocytes were always present, their density showing great variability from case to case and within each individual neoplasm. The possible existence of a pigmented counterpart to clear cell acanthoma is discussed.

The Authors studied, from 1986 to 1988, 84 patients (24 men and 60 women, in an age group that went from 13 to 75 years, with an average of 42 years) in whom DLE was diagnosed. In these patients, a direct immunofluorescent assay was carried out on the affected skin, on the non-affected photoexposed skin, and on the non-affected non-photoexposed skin: furthermore, the serum of these patients was tested for the presence of antinuclear antibodies (ANA), antibodies to extractable nuclear antigens (anti-ENA), and antibodies to double-stranded DNA (anti-DNA). In the study, a significant correlation between the presence of ANA and entity of cutaneous involvement at the Lupus Band Test was found. Furthermore, it was noticed that anti-ENA and/or anti-nDNA tend to be present in association to a positive Lupus Band Test in all 3 biopsied tissues.

Twenty-six children with a symmetric papular or papulovesicular acrolocated eruption were studied to evaluate a possible link with viral infection. A history of a recent immunization was present in four patients, two of whom had a polio vaccine virus isolated from their stool samples. There was serologic evidence of a recent Epstein-Barr virus infection in one patient, adenovirus infection in another, a concurrent Epstein-Barr virus and adenovirus infection in a third, and cytomegalovirus infection in a fourth. Viral cultures were positive in two patients for adenovirus and in two others for rotavirus. Although some of the virologic findings could have been coincidental, this study confirms that multiple viral infections can be related to most papular and papulovesicular acrolocated eruptions of childhood. Virologic investigations in the earlier phases of the eruption are recommended to identify the etiologic factors of these lesions.

The case of a 65-year-old man affected by chronic erythroderma due to Sézary syndrome and massive lymphomatous infiltration of the nails is reported. The clinical features of the nail lesions, consisting in thickening and discoloration of the nail plates, were not diagnostic. The nail biopsy showed a typical epidermotropic T cell lymphomatous involvement of all the nail constituents. Immunohistochemical characterization of the nails and skin lymphomatous infiltration showed identical results, confirming the neoplastic etiology of both the chronic erythroderma and the nail dystrophy.

Fifteen patients affected by Progressive Systemic Sclerosis have been studied. With immunofluorescence, specific antibodies against collagen type IV and laminin clearly outlined the microvessels, while endothelial cells showed a brilliant heavy fluorescence for vimentin antibodies. In the deep dermis, fibronectin proved to have increased. At electron microscopy, microvessels appeared with occluded lumina due to the presence of swollen endothelial cells. Endothelial cytoplasm was filled with intermediate filaments (vimentin type), generally condensed into peripherally located bundles or in perinuclear rings. The perivascular basal lamina was thickened and laminated. Although these changes do not demonstrate a specific pattern, representing a common step in several connective tissue disorders, the data tend to confirm a clear involvement of the microvasculature in Progressive Systemic Sclerosis.