Search

The current coronavirus disease 2019 pandemic has exceeded any epidemiologic prevision, but increasing information suggests some analogies with the major viral outbreaks in the last century, and a general warning has been issued on the possibility that coinfections can make the differential diagnosis and treatment difficult, especially in tropical countries. Some reports have noted that the presence of high dengue antibodies can give a false-negative result when testing for severe acute respiratory syndrome coronavirus 2. Mucocutaneous manifestations are very frequent, with an apparent overlap among different pathogens. However, strong clinicopathologic correlation might provide some clues to address differentials. Waiting for laboratory and instrumental results, the timing and distribution of skin lesions is often pathognomonic. Histopathologic findings characterize certain reaction patterns and provide insights on pathogenetic mechanisms. Unfortunately, skin assessment, especially invasive examinations such as biopsy, takes a back seat in severely ill patients. A literature retrieval was performed to collect information from other epidemics to counteract what has become the most frightening disease of our time.

Pseudoxanthoma elasticum (PXE)-like papillary dermal elastolysis (PDE) is an acquired, rare, elastic tissue disorder that presents with multiple asymptomatic, nonfollicular yellowish or normochromic papules, coalescing into symmetrical cobblestone plaques, with a predilection for the neck of postmenopausal women. The condition develops slowly, in months to years, and, although clinically similar to PXE, it is devoid of any systemic implication, being only of esthetic concern. The etiology is unknown, but it is speculated that there is a multifactorial pathogenesis, with the contribution of intrinsic aging, exposure to ultraviolet radiation, abnormal elastogenesis, and genetic factors. The diagnosis is confirmed by histopathology, with special stains specific for elastic fibers revealing loss of elastic plexus in the papillary dermis and the presence of melanophages. Its prevalence is probably underestimated, which reinforces the importance of better clinical and histologic identification to distinguish it from inherited PXE and to avoid extensive systemic investigations and unnecessary medical visits for the patient.

Summary Atypical fibroxanthoma (AFX) has been considered as the non-infiltrating precursor lesion of pleomorphic dermal sarcoma (PDS), which shows an aggressive clinical behavior, because of its extensive invasion of the deeper skin layers. Although these two tumors may represent two stages of the same disease, it can be difficult to differentiate between them, because of their similar clinical and histological features 1. Furthermore, they must be distinguished from a spindled variant of squamous carcinoma, melanoma and leiomyosarcoma 2. AFX/PDS still remains a diagnosis of exclusion, that needs to combine immunohistochemical markers for a definitive diagnosis. Usually AFX/PDS shows positivity for CD10, CD99, CD68, vimentin and lysozyme, while S100, HMB45, MART-1, cytokeratins, CD34, CD31, desmin and h-caldesmon are absent. We report a case of 89-year-old male, with a history of squamous cell carcinoma removed from the right ear, presented to our department with a recently growing, ulcerated and bleeding 2 cm nodule on the scalp. After surgery the tumor recurred with infiltration to the cranial theca. The final histological diagnosis was “pleomorphic dermal sarcoma” (PDS), which showed an unexpected positivity for HMB45. We present, to the best of our knowledge, the first case of AFX/PDS with an aberrant diffuse expression of HMB45 and an aggressive biological behavior, that leads us to a difficult exclusion diagnosis.

An increasing body of evidence has been produced in a very limited period of time to improve the understanding of skin involvement in the current Coronavirus 2019 disease pandemic, and how this novel disease impacts the management of dermatologic patients. A little explored area is represented by the therapeutic approach adopted for the different skin manifestations associated with the infection. An overview of the current scenario is provided, through review of the English-language literature published through October 30, 2020, and comparison with the personal experience of the authors. As dermatologists, our primary aim is to support patients with the highest standard of cares and relieve sufferance, even with lesions not being life-threatening. With asymptomatic COVID-19 patients, patients discomfort related to skin lesions should not be undervalued, and intervention to accelerate healing should be provided. Consensus protocols are warranted to assess the best skin targeted treatments in COVID-19 patients.

Background Hidradenitis Suppurativa is a chronic inflammatory skin disease with a strong negative impact on physical and psychological health. Obesity, especially visceral adiposity, but also smoking or unhealthy nutritional habits, have been reported to be significantly associated with hidradenitis suppurativa. Design and methods Case-control study in 35 patients of both sexes affected with hidradenitis suppurativa, and 35 healthy subjects matched for sex, age, and geographic origin (Sardinia, Italy). Results Several anthropometric measures (body mass index, waist circumference, body composition), lifestyle (Mediterranean diet adherence, and physical activity level), and the perceived physical and mental health status were evaluated in case and controls. Hidradenitis patients showed significantly higher values of body mass index, waist circumference, body composition, fat mass, and lower values of physical and mental health status compared to controls, while both groups showed a similar moderate physical activity level which can be assumed to counteract the negative effects of obesity or poor nutritional pattern in hidradenitis patients. However, none of the evaluated variables were correlated with the severity of the disease, assessed by means of the Hurley stage system or the Sartorius score. Instead, the Sartorius score showed a positive correlation with the duration of hidradenitis, mainly imputable to the diagnostic delay and the consequent long lasting inflammatory status. Conclusions Although nutritional factors and lifestyle can be important and modifiable factors in the hidradenitis suppurativa course, the detrimental effect of chronic inflammation and delayed management are clearly prevalent and heavily influence the disease burden.

The study aimed to verify whether exercise training in older adults can improve social behavioral rhythms (SBR) and if any modification is maintained over time. Older adults (n=120) from a previous randomized controlled trial (RCT), were randomly allocated to either a moderate-intensity exercise group or a control group. SBR was evaluated at t0, t26, and t48 weeks (during the COVID-19 lockdown), using the brief social rhythms scale (BSRS). Seventy-nine participants completed the follow-up (age 72.3±4.7, women 55.3%). An improvement in the BSRS score was found in the exercise group at 26 weeks (p=0.035) when the exercise program was concluded, and it was still maintained at 48 weeks (p=0.013). No improvements were observed in the control group. To conclude, social behavioral rhythms (SBR), previously found as a resilience factor in older adults during Covid-19, appear to improve after a moderate 12 weeks exercise program, and the improvement persisted even after stopping exercise during the COVID-19 lockdown.

Vaccines are actually the most effective strategy to control the COVID-19 spread and reduce mortality, but adverse reactions can occur. Skin involvement with novel messenger RNA coronavirus vaccines seems frequent but is not completely characterized. A real-world experience in the recent vaccination campaign among health care workers in Sardinia (Italy) is reported. In over a total of 1577 persons vaccinated, 9 cases of skin adverse reactions were observed (0.5%). All reactions have been reported to the Italian Pharmacovigilance Authority. Eight occurred in women (mean age 46 years), and five were physicians and four nurses. All patients had a significant allergology history but not for the known vaccine excipients. After dose one, no injection site reactions were observed, but widespread pruritus (n = 3), mild facial erythema (n = 1), and maculopapular rash (n = 3) occurred in the following 24–48 h in three patients. These three patients were excluded from the second dose. Of the remaining six patients, one developed mild anaphylaxis within the observation period at the vaccination hub and five delayed facial erythematous edema and maculopapular lesions, requiring antihistamines and short-course corticosteroid treatment. Spontaneous reporting is paramount to adjourning vaccination guidance and preventive measures in order to contribute to the development of a safe vaccine strategy. Dermatologist’ expertise might provide better characterization, treatment, and screening of individuals at high risk of skin adverse reactions.

Acquired brachial cutaneous dyschromatosis (ABCD) is a relatively newly described, acquired disorder of pigmentation characterized by geographic-shaped, gray-brown, hyperpigmented patches and interspersed with hypopigmented macules, involving the dorsal aspects of the forearms in postmenopausal women. There is a suggested relationship with hypertension and antihypertensive medication intake, specifically angiotensin-converting enzyme inhibitors, or a cumulative effect of sun damage, as possible triggers. ABCD is benign, asymptomatic, and more of an esthetic concern. Topical depigmenting agents, chemical peels, and laser therapy may be helpful.

Acral persistent papular mucinosis (APPM) is a subtype of localized lichen myxedematosus (LM) characterized by the chronic development of white to skin-colored papules and limited to the extensor surfaces of the hands and distal part of forearms, in the absence of systemic or laboratory manifestations. There is a strong predominance in women. Histopathology shows focal accumulation of mucin in the upper portion of the dermis sparing the grenz zone. The etiology is unknown. It is a benign condition, although dynamic changes occur with the progressive development of additional lesions. No specific treatment is required, and patients should be reassured about the prognosis.

Pilomatrix carcinoma is a rare tumor that is generally not diagnosed clinically. An 80-year-old man presented with a 5-month history of rapidly growing nodule of the submandibular area. Histological examination revealed a pilomatrix carcinoma, an aggressive malignancy with metastatic potential.

BackgroundCutaneous melanoma (CM) is one of the most aggressive types of skin cancer. Currently, innovative approaches such as target therapies and immunotherapies have been introduced in clinical practice. Data of clinical trials and real life studies that evaluate the outcomes of these therapeutic associations are necessary to establish their clinical utility. The aim of this study is to investigate the types of oncological treatments employed in the real-life clinical management of patients with advanced CM in several Italian centers, which are part of the Clinical National Melanoma Registry (CNMR).MethodsMelanoma-specific survival and overall survival were calculated. Multivariate Cox regression models were used to estimate the hazard ratios adjusting for confounders and other prognostic factors.ResultsThe median follow-up time was 36 months (range 1.2-185.1). 787 CM were included in the analysis with completed information about therapies. All types of immunotherapy showed a significant improved survival compared with all other therapies (p=0.001). 75% was the highest reduction of death reached by anti-PD-1 (HR=0.25), globally immunotherapy was significantly associated with improved survival, either for anti-CTLA4 monotherapy or combined with anti-PD-1 (HR=0.47 and 0.26, respectively) and BRAFI+MEKI (HR=0.62).ConclusionsThe nivolumab/pembrolizumab in combination of ipilimumab and the addition of ant-MEK to the BRAFi can be considered the best therapies to improve survival in a real-world-population. The CNMR can complement clinical registries with the intent of improving cancer management and standardizing cancer treatment.

Restrictive measures to contain the coronavirus disease 2019 (COVID-19) pandemic might produce different effects on other infective diseases, especially those affecting the most intimate sphere of sexuality. The epidemiology of syphilis could reflect the consequences of whether people are avoiding or not risky behaviors. To understand the course of syphilis during the COVID-19 outbreak, we performed a retrospective observational study of all new diagnoses observed at the STDs Service of the Dermatology Clinic at Cagliari, part of the Italian sentinel surveillance system. All incident cases diagnosed during the first 6 months of each year, from 2016 to 2020, thus including the recent lockdown period, were retrieved from the database. Of the 87 cases studied, 18 occurred during the first 6 months of 2020, almost all patients (88%) presenting with early phases of the disease and reporting unprotected sexual intercourses in spite of community containment and social distancing. Comparison with the previous 4 years found no significant statistical differences that hospital access and management limitations had not impaired the management of patients with syphilis. We alert the medical community of the possible increase of sexually transmitted diseases, as society returns to normal.

Primary localized cutaneous nodular amyloidosis (PLCNA) is a rare condition due to the plasma cell proliferation and skin deposition of immunoglobulin light chains, without systemic amyloidosis or hematological dyscrasias. The association with autoimmune connective tissue diseases has been reported, especially with Sjogren’s syndrome, and in a few cases with systemic sclerosis. Herein, we describe three cases of PLCNA occurring in women with a diagnosis of limited cutaneous systemic sclerosis and review the literature on the topic to highlight a stereotypical presentation. Moreover, we support the usefulness of dermoscopy, characterized by a yellow–orange waxy pattern surrounded by telangiectasias, for a rapid and non-invasive diagnostic assessment. Thus, when asymptomatic nodules occur on lower limbs of women affected with limited systemic sclerosis, and dermoscopy identifies yellow–orange blotches, a diagnosis of PLCNA can be considered and further confirmed by histopathology. Monitoring for systemic amyloidosis development is advisable, although the risk of progression is considered very low.

Cutaneous reactive angiomatoses are a group of uncommon angioproliferative (capillary) conditions presenting as multiple, erythematous–violaceous, livedoid patches and plaques, sometimes evolving toward necrosis and ulceration, mainly localized on lower limbs and occasionally widely distributed. On histopathology, these diseases show different patterns of dermal intravascular or extravascular lobular or diffuse hyperplasia of endothelial cells, pericytes, and histiocytes, without significant cellular atypia. They include less recent conditions such as reactive angioendotheliomatosis (RAE) and acroangiodermatitis (pseudo-Kaposi sarcoma) and relatively newer pathologies such as diffuse dermal angiomatosis (DDA), reactive intravascular histiocytosis (RIH), glomeruloid RAE, and angiopericytomatosis (angiomatosis with cryoproteins). Most cases are associated with an infectious, immunological, vasculopathic, or neoplastic systemic disease leading to ischemia and consequent vascular proliferation, and skin lesions often improve or disappear after the management of the associated illnesses.

Aquagenic (palmoplantar) keratoderma is an uncommon hereditary or sporadic condition, whose pathogenesis is largely uncovered, but characterized by a peculiar transitory palmar and more rarely plantar skin wrinkling after brief water exposure. Association with cystic fibrosis is frequent and related to mutations on the same cystic fibrosis transmembrane conductance regulator gene, usually the ΔF508 mutation, either homozygous or heterozygous. However, aquagenic keratoderma is frequently idiopathic or secondary to other internal organ dysfunctions, as well as drug-induced. Diagnosis is mainly clinical and easily confirmed with an immersion test, if one is aware of this entity. Histopathology is rarely performed, but highly informative of the transient alterations of the stratum corneum and eccrine glands. Sporadic form may resolve spontaneously after a few years or have periods of remission during the life course. For very disturbing conditions, topical aluminum hydroxide and botulinum toxin injections are the most used treatments.

Cutaneous mucinoses are a wide spectrum of disorders, characterized by the deposition of glycosaminoglycans, also known as acid mucopolysaccharides in the dermis, follicles, or in the epidermis. Major cutaneous mucinoses include lichen myxedematosus, scleredema, mucinoses associated with altered thyroid function, reticular erythematous mucinosis, papulonodular mucinosis associated with connective tissue diseases, and cutaneous focal mucinosis. In the last years, several new presentations in adults have been described. They include (a) obesity-associated lymphedematous mucinosis and pretibial stasis mucinosis, (b) cutaneous mucinoses associated with drug exposure, (c) cutaneous mucinoses after physical and mechanical traumas, (d) cutaneous mucinosis after knee replacement, and (e) nodular mucinosis of the breast (NMB).

Cutaneous dermal mucinoses are a group of conditions characterized by abnormal deposition of mucin (hyaluronic acid and sulfated glycosaminoglycans) in the dermis. They can be classified either as localized or generalized forms and occur primarily or secondarily to systemic disorders. Obesity-associated lymphedematous mucinosis and pretibial stasis mucinosis are uncommon and relatively newly recognized disorders occurring in obese patients or in patients with venous insufficiency. Clinically, patients present with papules and nodules progressing to plaques and arising in an erythematous and edematous basis on the legs, especially the shins. Histopathologic examination indicates mucin deposition in the superficial portion of the dermis, angioplasia with an increase in small blood and/or lymphatic vessels, vertically running vessels, slight fibrosis, and no inflammation. Laboratory workup and histopathologic testing are helpful in differentiating these entities from pretibial myxedema (thyroid dermopathy), which represents the main differential diagnosis. Obesity-associated lymphedematous mucinosis and stasis mucinosis are not different entities but belong to a spectrum of mucinoses secondary to an increased body mass index, chronic lymphedema, and/or chronic venous insufficiency.

Cutaneous clear-cell squamous cell carcinoma (ccSCC) is a rare variant of SCC composed of clear cells that lack cytoplasmic glycogen or evidence of tricholemmal keratinization. We report a previously undescribed variant of ccSCC with psammomatous calcification and intratumoral giant cell granulomas. The differential diagnosis with trichilemmal carcinoma is outlined according to the criteria of the 4th edition of World Health Organization (WHO) classification. Our findings outline that psammomatous calcification may occur inside the keratinous pearls of the neoplastic lobules triggering an intratumoral giant cell granulomatous reaction. The prognostic significance of this histopathological presentation is unknown but the potential for psammomatous bodies formation in cSCC should be considered to avoid diagnostic pitfalls. This article is protected by copyright. All rights reserved.

New and emerging types of cutaneous vascular (capillary) proliferations have been described or better categorized in the last few years. They include reactive angioendotheliomatosis, acroangiodermatitis (pseudo-Kaposi sarcoma), diffuse dermal angiomatosis, intravascular histiocytosis, glomeruloid angioendotheliomatosis, and angiopericytomatosis (angiomatosis with cryoproteins). Clinically, they are characterized by multiple, red violaceous, and purpuric patches and plaques, sometimes evolving toward necrosis and ulceration with a wide distribution but a propensity to involve the extremities. Histologically, they are characterized by different patterns of intravascular or extravascular lobular or diffuse hyperplasia of endothelial cells, pericytes, and sometimes histiocytes. Although these angioproliferations can histologically have a pseudoangiosarcomatous pattern, they are reactive in that they originate from the (sub)occlusion of vascular lumina by different localized or systemic disorders. The vascular proliferation stops after the inducing hypoxic stimulus has been withdrawn. Among them, diffuse dermal angiomatosis of the breast is a variant of diffuse dermal angiomatosis involving middle-aged women with macromastia, obesity, smoking, and vasculopathic disorders, considered a distinct disorder in the spectrum of cutaneous reactive angiomatoses. It presents with reticulated erythematous to purple patches with sometimes a tendency to ulcerate and bleeding, appearing on large, pendulous breasts. The pathogenesis is related to tissue hypoxemia resulting from subclinical torsion, compression, and increased venous hydrostatic pressure due to the macromastia, aggravated by the associated ischemic conditions such as hypertension and diabetes. There is no evidence-based therapy, but reduction mammoplasty is a viable treatment option. This should be evaluated in all patients who fail conservative therapy.

A great heterogeneity of skin manifestations has been increasingly associated with SARS-CoV2 infection, and especially exanthematous eruptions are considered among early presenting signs in symptomatic patients. In this report, a 58-year-old patient presented with fever, generalized rash and systemic symptoms as urgent consultation to the Dermatology Clinic of Cagliari amidst the Italian outbreak in late March 2020. After a negative nasopharyngeal swab for SARS-CoV-2, secondary syphilis was confirmed by serological tests and skin biopsy, underlining that, even during a global pandemic, other common and important diseases should not be overlooked. The case also suggests thought on the ineffectiveness of distancing and social containment measures when dealing with sexually transmitted diseases. Significance for public health Even during a pandemic, despite community containment and social distancing, sexually transmitted diseases persist, and syphilis should never be forgotten. As highlighted by present report, the potential impact on public health is substantial, considering that secondary syphilis is a highly contagious generalized bacteremic phase of the disease. Resurgence of syphilis is an ongoing unsolved problem for at least a decade, which takes a back seat to the current dramatic COVID-19 spreading. However, limitation to outpatients’ visits and hospital screening services due to the lockdown measures are likely to favor syphilis circulation, delay diagnosis and adequate treatment. Dermatologists are in the leading position to support the medical community in such difficult situations, keeping focused on good clinical practice to promptly recognize subtle but potentially severe diseases like syphilis.

Psoriasis is a major chronic inflammatory skin disease, affecting about 3% of the population in Italy, whose management require experienced specialists in order to guarantee high‐quality standards of care. The pandemic coronavirus (2019‐nCoV; COVID‐19) has changed the approach to all patients requiring close contact during a visit, including dermatologic consultations. In Italy, true outbreak begun in Lombardy, by February 21, 2020 with exponential contagion, surpassing China in the number of deaths.

Background Patients with multiple actinic keratoses (AKs) should be treated with field-directed therapy. Such treatments challenge patients' adherence due to out-of-pocket costs, length of treatment and severity of local skin reactions (LSRs). Effective physician-patient communication (PPC) may buffer therapy-related distress, thus improving quality of life, treatment satisfaction and adherence. Objectives We evaluated the interplay between PPC, LSR intensity (safety) and lesion clearance rates (effectiveness) on treatment satisfaction, quality of life and treatment adherence among patients with multiple AKs receiving topical field-directed therapies. Methods In this observational, multicentre, longitudinal, cohort study, we included 1136 adult patients with discrete, clinically detectable, visible, multiple (three or more lesions in a 25 cm2 area), Grade I/II AKs, for whom the attending dermatologist has prescribed treatment with a topical field-directed therapy. We matched self-reported data and medical information recorded by dermatologists in standard clinical forms. Patients were followed up at two time points (T1: 8 days; T2: 25-30 days) RESULTS: Most patients were elderly, married, men with poor socio-economic status and multiple lesions of the scalp or face. The majority (n = 961) had a prescription of ingenol mebutate (IMB) and 175 received either diclofenac 3% in hyaluronic acid (DHA) or imiquimod 5% (IMQ). Clearance rate at 1 month was 84%. Most patients felt very supported (n = 819, 73%) and rated dermatologist's explanations very clear (n = 608, 54%). Treatment satisfaction (effectiveness and convenience scales) increased along the follow-up, especially for those on IMB (Δpre-post = -4.00; other: Δpre-post = -0.25; interaction P Conclusion Communication clarity was associated with patient-reported outcomes and adherence beyond AK-related clinical parameters. Our study questions the current episodic approach to AK management and provides the rationale to develop chronic care models fostering patients' engagement and treatment alliance.

Introduction: Scleromyxedema is a rare fibromucinosis, associated with monoclonal gammopathy, and other comorbidities with unpredictable prognosis. It usually affects middle-aged adults, without gender predilection. Incomplete understanding of the pathogenesis depends on the limited number of cases, but consolidated multicenter experience has produced the European guidelines for clinical management of isolated cases. Areas covered: A synthesis of current knowledge on pathogenesis and treatment of scleromyxedema is retrieved from PubMed database. Expert opinion: Understanding of scleromyxedema remains a challenge, without substantial progress in the explanation of mucin deposition origin, paraprotein role and factors primarly implicated in the disease progression. However, the definition of diagnostic criteria and lines of treatment have increased the awareness and early recognition of the disease in recent years. Timely treatment with high dose immunoglobulin has proven efficacy and tolerability, becoming first line treatment, eventually associated with thalidomide and/or systemic steroids. Very aggressive treatment, such as melphalan are no more recommended, while bortezomib and/or autologous stem cell transplant are considered in very recalcitrant cases. No promising new options are under evaluation. Future perspectives, besides etiopathogenesis improvements, are definition of prognostic criteria, to distinguish rapidly disabling disease from slow progression, and improvement of imaging, to support the diagnosis and management protocols.

Skin diseases in the elderly are frequent and sometimes misleading, due to age-related changes that alter the clinical presentation. Clinicopathologic correlation is often the clue for distinguishing among various pathologies for which clinical signs are more striking than the histopathologic findings. Pitfalls are always lying in wait to ambush inexperienced dermatologists, but usually the microscope provides subtle hints to solve the quandary. In an attempt to familiarize physicians with emerging skin care demands of older patients, we have reviewed major diagnostic challenges in this group of patients: age-related changes, more common skin pathologies in mature patients, manifestations of comorbidity, skin tumors, and paraneoplastic syndromes.

Epithelial-mesenchymal transition (EMT) has been suggested to have a driving role in the acquisition of a metastatic potential by melanoma cells. Important hallmarks of EMT include both E-cadherin downregulation and increased expression of N-cadherin. This switch in distinct classes of adhesion molecules leads melanoma cells to lose contact with adjacent keratinocytes and interact instead with stromal fibroblasts and endothelial cells, thus promoting dermal and vascular melanoma invasion. Consequently, tumor cells migrate to distant host tissues and establish metastases. A key regulator in the induction of EMT in melanoma is the Notch1 signaling pathway that, when activated, is prompt to upregulate N-cadherin expression. By means of this strategy, melanoma cells gain enhanced survival, proliferation and invasion properties, driving the tumor toward a more aggressive phenotype. On the basis of these statements, the present study aimed to investigate the possible association between N-cadherin and Notch1 presence in primary cutaneous melanomas and lymph node metastases. Our results from immunohistochemical analysis confirmed a positive correlation between N-cadherin and Notch1 presence in the same tumor samples. Moreover, this study highlighted that a concomitant high expression of N-cadherin and Notch1, both in primary lesions and in lymph node metastases, predicts an adverse clinical outcome in melanoma patients. Therefore, N-cadherin and Notch1 co-presence can be monitored as a predictive factor in early- and advanced-stage melanomas and open additional therapeutic targets for the restraint of melanoma metastasis.

Cutaneous mucinoses are a heterogeneous group of disorders having as a common denominator the deposition of an excess of mucin (acid glycosaminoglycans, mostly hyaluronic acid) in the dermis or in the hair follicles. They are divided into primary and secondary forms. Primary cutaneous mucinoses are characterized by specific clinical lesions in which mucin deposition is the distinctive histological sign, while in secondary mucinoses, mucin deposition is simply an accessory histological sign. Disorders of collagen and elastic fibers are divided into hereditary and acquired forms. Both forms can be further divided into those diseases characterized by an increase and those by a decrease in collagen and elastic tissue. A peculiar variant of disorders of dermal connective tissue includes the primary perforating dermatoses.

Leiomyosarcoma is a relatively rare soft tissue tumor whose clear-cell variant has only been reported in leiomyosarcomas of the uterus. We report here for the first time a primary cutaneous clear-cell leiomyosarcoma in the trunk skin of a 49-year-old man, characterized by a very indolent clinical and dermoscopic presentation, mimicking a dermatofibroma. Genetic analysis of the otherwise healthy patient revealed a germline mutation in the retinoblastoma 1 gene (RB1); the same mutation was found in his son, who had previously developed retinoblastoma. Moreover, the mother of the patient had died of uterine leiomyosarcoma with clear-cell changes. Mutations in the RB1 gene occur commonly in human neoplasms. In this patient, we were able to link his clear-cell variant of cutaneous leiomyosarcoma with the loss of retinoblastoma protein expression, as revealed by immunohistochemical staining analysis.

Scleredema adultorum of Buschke is a rare fibromucinous, scleroderma-like connective tissue disease most commonly found in a post-infectious setting or linked to hematological disorders or diabetes. Lichen sclerosus et atrophicus is an autoimmune condition only in 2.5% of cases localized exclusively at an extragenital site, occurring in up to 34% of patients in association with other autoimmune diseases such as vitiligo, thyroid disorders, alopecia areata, lichen planus, morphea, pernicious anemia and systemic lupus erythematosus. In particular, a stronger link with an autoimmune background in lichen sclerosus et atrophicus has been observed in women who showed higher prevalence for autoimmune conditions and circulating autoantibodies. Literature reveals a genetic susceptibility linked to specific HLA types. We report three patients who developed lichen sclerosus et atrophicus superimposed on skin involved by scleredema adultorum of Buschke. Although the association of lichen sclerosus et atrophicus with scleredema adultorum of Buschke could be coincidental, both diseases could be considered part of the spectrum of sclerodermoid disorders with common underlying pathogenetic mechanisms; which could explain the sequential or simultaneous occurrence of both lesions in our patients.

Bowen's disease is a cutaneous squamous cell carcinoma (SCC) in situ with a potential risk of progression to invasive SCC. Despite the high number of approved treatments, elderly patients with extensive lesions of critical sites may represent a therapeutical challenge, especially in cases of treatment failure or recalcitrant disease. Here, we report the successfully use of topical Imiquimod 3.75% to treat an extensive Bowen's disease of the cheek in an elderly.

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin signs) and AESOP (adenopathy and extensive skin patch overlying a plasmacytoma) syndromes are rare paraneoplastic conditions due to an underlying plasma cell dyscrasia. We report a 70-year-old patient with the rare coexistence of POEMS and AESOP syndromes and in whom skin signs, that differ both clinically and histologically, were the clues to the diagnosis of a plasma cell disorder. Vascular endothelial growth factor-A overexpression seems to be the common pathogenetic link of the different clinicopathological presentations of the skin lesions.

Scleromyxedema is a rare fibromucinous disorders, with several clinical and pathological overlaps with scleroderma and scleredema. Etiopathogenesis remains uncovered, and no explanation has been provided either for the origin of mucin deposition or for the paraprotein role. The disease does not show gender predilection and affects mainly middle-age adults. The course is unpredictable, and prognosis remains guarded for renal, cardiac, and neurologic complications, especially in the setting of dermato-neuro syndrome. A valuable recent progress is the consensus definition of diagnostic criteria and lines of treatment, which hold the promise to improve the early recognition and management of this rare condition worldwide. High-dose intravenous immunoglobulin has been suggested as the first-line treatment either alone or associated with systemic steroids and/or thalidomide. In very recalcitrant cases, adjunctive bortezomib and/or autologous stem cell transplant might be considered. Melphalan treatment was associated with very toxic side effects and actually is no longer recommended.

While histological analysis represents a powerful tool for the classification of melanocytic lesions as benign or malignant, a clear-cut distinction between a nevus and a melanoma is sometimes a challenging step of the diagnostic process. The immunohistochemical detection of tyrosinase, cardinal melanogenic enzyme during melanocytic maturation, has often been helpful in formulating a differential diagnosis due to the peculiar staining pattern in nevocytes compared with melanoma cells. Tyrosinase distribution in nevi appears to overlap with the cytoarchitectural changes observable within these lesions, that result in epidermal or superficial dermal nevocytes being larger and strongly expressing melanocytic differentiation antigens, such as tyrosinase, compared with deeper dermal nevus cells. Our study aimed to evaluate the immunohistochemical expression pattern of tyrosinase in different histological types of acquired dysplastic melanocytic nevi, including junctional, compound, and intradermal nevi. Moreover, to estimate whether in nevocytes the expression of tyrosinase was associated with their differentiation state, we investigated the expression of two recognized markers of pluripotency, CD34 and nestin. In all examined nevi, our analysis revealed a remarkable immunoreactivity for tyrosinase in junctional and superficial dermal nevocytes and a decreasing gradient of staining in dermal nevocytes, up to become negative in deeper dermis. Meanwhile, junctional and dermal nevocytes were lacking in CD34 protein. Furthermore, nestin immunostaining showed an opposite distribution compared with tyrosinase, leading us to look into the tyrosinase/nestin expression pattern in melanocytic nevus as a tool to better understand the final stages of differentiation of melanocyte precursors toward their ultimate anatomical site into the epidermis.

Background: The clinical and dermoscopic changes of melanocytic nevi under the effects of photo-epilation are poorly known. Objective: Prospective study on clinical and dermatoscopic effects of photoe-pilation on melanocytic nevi. Methods: Eighteen patients exposed to photo-epilation gave consent to a follow-up programme from 2008 to 2017 at the Videodermatoscopic Office of the Dermatology Clinic of Cagliari (Italy), with clinical and dermoscopic assessment. Results: A mean 2-year follow-up was recorded for 73 lesions. The most frequent clinical change was nevi colour, clearer than basal pictures, sometimes with increased pigmentation at the periphery, or residual islands of pigmentation, with vanishing borders. Acute burn with crusting was noted in few cases. Only one case showed progressive growth. Corresponding dermoscopic findings were bleaching of nevi, followed by pigment network disruption, appearance of white structureless areas and grey–blue globules. Complete regression occurred in 32.8% of the lesions. The growing nevus was characterized by peripheral globules. Excision for histopathological examination showed a compound nevus with mild atypia. Conclusions: Although malignant modifications of nevi after photo-epilation have not been described, the clinical and dermoscopic changes we observed suggest to extend the follow for more than 24months, as the potential long-term adverse effects of photo-epilation on melanocytic nevi are unknown.

Cutaneous mucinoses are a group of conditions characterized by increased amounts of acid mucin in the dermis. They can be generalized or localized and occur isolated or in the setting of systemic diseases. Obesity-associated lymphedematous mucinosis is a distinct variant of mucinosis occurring in obese patients without any thyroid dysfunction. So far, only few cases of this rare condition have been reported in the English literature. Here, we describe two new cases and discuss some histological differences with the pretibial myxedema.

Dysplasia and squamous cell carcinoma of the upper aerodigestive tract show significant neoangiogenesis appearing as subepithelial and epithelial microvascular irregularities that can be detected by Image-Enhanced Endoscopy such as Narrow Band Imaging and Storz Professional Image Enhancement System. In the present study, the most advanced endoscopic enhancement systems were coupled with Contact Endoscopy (Enhanced Contact Endoscopy). This original method improved the identification and the understanding of the neoangiogenetic changes of the chorion in 42 patients with leukoplakia, erythroplakia, and leuko-erythroplakia of the oral cavity and oropharynx. The physiologic and pathologic mucosa was described in five obvious vascular patterns observed at Enhanced Contact Endoscopy ranging from normal to squamous cell carcinoma, passing through inflammation, hyperplasia, and dysplasia. Each vascular pattern was then compared to histology, showing that the microvascular architectural changes seen with Enhanced Contact Endoscopy are almost constant. Sensitivity, specificity, positive predictive value, and negative predictive value in the differentiation between healthy mucosa and inflammation versus pathologic hyperplasia, dysplasia, and carcinoma were, respectively, 96.6, 93.3, 98.2, 87.5, and 95.9 %. Sensitivity and specificity were 100 % in differentiation between non-malignant lesions versus squamous cell carcinoma. Our preliminary experience shows that accuracy of Image-Enhanced Endoscopy in the diagnosis of precancerous lesions and squamous cell carcinoma of the oral cavity and oropharynx can be increased if associated to Contact Endoscopy.