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Epithelial tumors of the lacrimal gland are rare and usually develop in the orbital lobe. We report the exceedingly rare occurrence of a primary adenoid cystic carcinoma in the palpebral lobe of the lacrimal gland. A 26-year-old female was referred for evaluation of a gradually enlarging mass in the lateral upper eyelid, previously diagnosed as a chalazion. Computed tomography revealed a heterogeneous round lesion anterior to the orbital rim. Excisional biopsy was compatible with an adenoid cystic carcinoma. After excluding distant metastasis, and as the patient refused adjuvant radiotherapy, a second surgical procedure, with wide local excision, was indicated. Follow-up showed no recurrence. This case highlights the importance of performing a thorough clinical examination when diagnosing any lateral upper eyelid mass. A high index of suspicion for malignant tumors of the lacrimal gland should always be maintained, and a complete excision with histological analysis should be preferred whenever possible. info:eu-repo/semantics/publishedVersion

Background: The treatment of advanced periocular basal cell carcinomas becomes a challenge as surgery may involve highly mutilating procedures. Vismodegib is the first selective hedgehog inhibitor approved for the treatment of locally advanced tumors or metastatic disease. Objective: Analyze the results of treatment with vismodegib for advanced periocular basal cell carcinomas in a real-life setting of a reference center between 2014 and 2020. Methods: Retrospective longitudinal study. The patient's demographic profile, comorbidities, tumor characteristics, and treatment outcomes were analyzed. Results: A total of 13 patients were included. Median follow-up and treatment duration were 15.9 and 10.5 months, respectively. Objective clinical response rate was 76.9%: 30.8% had a complete response and 46.2% a partial response. The median duration of response was 13 months. Progressive disease was observed in 38.5% of cases, with a median of 19 months after the beginning of treatment. Eighty-four percent of the patients had at least one adverse event, and 61.54% needed to interrupt treatment temporarily or permanently to increase tolerability. Study limitations: Being a retrospective study in a real-life setting, the evaluation of objective clinical response was subjective to physician appreciation. Conclusion: Vismodegib is a safe and effective treatment for locally advanced basal cell carcinoma. To prevent recurrences, the drug should be used continually when tolerated. The role of neoadjuvant vismodegib before surgery is being investigated and might add an important step in searching for a definitive treatment for these cases. info:eu-repo/semantics/publishedVersion

BACKGROUND The use of UV-emitting tanning devices for cosmetic purposes is associated with an increased risk of melanoma and non-melanoma skin cancer. Young women are the most frequent users, therefore, there is an increasing concern about the regulation of sunbed use. OBJECTIVE The primary objective is to assess the current legislation on sunbed use among European countries. METHODS We developed a 30-item questionnaire to gather the most relevant information about sunbed use legislation. The questionnaire was sent to Euromelanoma coordinators and to designated coordinators out of the Euromelanoma network. RESULTS We obtained a response rate of 64%. More than 25% of the countries did not report any specific legislation. Roughly one-third of the countries does not have a restriction for minors. Even in countries with a specific legislation, a lack or insufficient enforcement of age limit was observed in up to 100% of the inspections based on the PROSAFE report from 2012. Self-tanning devices were reported in 50%, and almost 40% of countries do not require supervision of use. Although a warning display is required in 77% of cases, a signed informed consent is not required in 80%. In the vast majority of cases, the number of licensed or closed tanning centres is unknown. CONCLUSIONS Despite the evidence of its harmful effects, and its frequent use by young people, many of whom are at high risk of skin cancer because of fair skin, a significant number of European countries lack a specific legislation on tanning devices. In order to limit the access of young people to sunbeds, a more strictly enforced regulation is needed, as well as regulation regarding advertisement, and location of tanning centres, in addition to health promotion campaigns that target the vulnerable population of young women seeking its use for improved cosmesis.

In our modern life world, health and well-being strongly depend on the individual's health behaviours. Motivation is a major factor of health behaviour change, and intrinsically motivated behaviour change is desirable as it is both sustained and directly contributes to well-being. This raises the immediate question what kind of interventions are best positioned to intrinsically motivate health behaviour change. The current state of evidence supports that gamification can have a positive impact in health and wellbeing. In recent years, games and game technology have been used quite widely to investigate if they can help make rehabilitation more engaging for users. The underlying hypothesis is that the motivating qualities of games may be harnessed and embedded into a game-based rehabilitation system to improve the quality of user participation.

We conducted a retrospective study aiming to assess the risk, and associated risk factors, of developing subsequent skin cancers after having a first diagnosis of skin cancer. We included all patients with biopsy-proven skin cancer attending a dermatology clinic between July 2007 and July 2017. We assessed the frequency of new skin cancers, as well as potential demographic and clinical factors significantly associated with occurrence of such neoplasms, that were identified by means of a survival analysis. We analyzed 969 patients with a total of 1584 skin neoplasms (1122 basal cell carcinomas (BCC), 310 squamous cell carcinomas (SCC), 143 melanomas and 9 other neoplasms). 165 patients (17.0%) developed subsequent skin neoplasms. Factors identified in multivariable models to be significantly associated with development of new skin cancers included older age (adjusted HR = 1.04 per year; 95%CI = 1.02–1.05; p p p = 0.030), while having a previous SCC was associated with occurrence of subsequent SCC (adjusted HR = 3.60; 95%CI = 1.93–6.72; p

1. Maria Pereira, MD* 2. Ana Leite, MD* 3. Eduarda Osorio Ferreira, MD† 4. Ana Filipa Duarte, MD‡ 5. Eckart Haneke, MD, PhD‡,§ 6. Osvaldo Correia, MD, PhD¶ 1. *Pediatrics Department and 2. †Dermatology Department, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal 3. ‡Centro Dermatologia Epidermis, Instituto CUFP, Porto, Oporto, Portugal 4. §Dermatology Department, Inselspital, University of Bern, Bern, Switzerland 5. ¶Centro Dermatologia Epidermis, Instituto CUF Faculdade de Medicina, Universidade do Porto, Oporto, Portugal A 7-year-old boy presents to pediatric consultation with a 5-year history of progressively enlarging and multiplying asymptomatic skin lesions distributed over the right thigh, back, and abdomen. He has no other complaints or relevant medical history. Histopathologic analysis of a punch biopsy from the right thigh performed when the child was 3 years old was suggestive of a nevus lipomatodes superficialis. The maternal family history is notable for osteopoikilosis in members of the 3 previous generations, including his mother (Fig 1) and an aunt who has been evaluated for metastatic bone disease because of radiographic findings. There is no family history of similar skin lesions. Figure 1. Right foot radiograph of the mother showing multiple small round hyperdense foci of osteopoikilosis (arrowheads). Physical examination reveals asymmetrically distributed papules and nodules, 10 to 18 mm in diameter, coalescing into plaques in the anterior surface of his right thigh, sacral region, and left lower abdomen. They have irregular contours, a yellow hue, a smooth surface, and a firm rubbery consistency (Fig 2). Figure 2. Right thigh anterior surface papular skin lesions in the child coalescing into plaques, with irregular contours, a yellow tone, a smooth surface, and a firm rubbery consistency. Histopathologic analysis of a punch biopsy sample from the right thigh at this age is again diagnosed as a nevus lipomatodes superficialis. However, further review of the same biopsy yields the features of a typical cutaneous tissue nevus (CTN) with an increase of elastic fibers and irregular arrangement of the collagen tissue fibers. The overlying epidermis is normal, and there is no inflammatory infiltrate and no increase …

PURPOSE: To evaluate the aesthetic and functional outcomes of autologous fat transfer using the SEFFI (superficial enhanced fluid fat injection) technique for reconstruction of the periocular area. METHODS: Autologous fat injections prepared with the 0.5 mL and 0.8 mL SEFFI technique were used in four patients for periocular rehabilitation. RESULTS: Case 1 (C1): A patient with left-sided progressive facial hemiatrophy underwent ipsilateral volumizing with 0.8 SEFFI in the superior, temporal, and inferior periorbital areas, and 0.5 SEFFI in both eyelids. C2: A 21-year-old female with a post trauma frontal scar, left ptosis, and lower eyelid retraction was treated with 0.5 SEFFI applied in the scar area associated with an upper eyelid conjunctivomullerectomy and resection of the lower eyelid retractors. C3: A patient with previous left-eye evisceration and orbital floor and medial wall fractures underwent socket reconstruction with buccal mucosal graft in the lower fornix and 0.5 SEFFI injections in both superior and inferior eyelids. SEFFI was also applied in the intraorbital space for correction of the enophthalmos. C4: A patient with lower lid retraction post blepharoplasty was treated with 0.8 SEFFI injections in lower eyelids and malar areas, complemented with a bilateral lateral cantopexy. CONCLUSIONS: Autologous fat transfer with SEFFI technique is an effective and safe procedure in cases of periocular rehabilitation. info:eu-repo/semantics/publishedVersion

PURPOSE To evaluate the use of autogenous tarsal graft for surgical correction of lower eyelid trichiasis associated with eyelid thinning. METHODS A total of 23 eyelids of 19 consecutive patients with trichiasis were operated with a variant of the Van Millingen procedure. The surgery consisted of interposing a 3-mm high fragment of autogenous ipsilateral upper eyelid tarsus corresponding to the trichiasis segment between the pretarsal anterior lamella and the lower tarsal plate. Preoperative assessment included quantification of eyelid thickness on the midpoint of the segment with trichiasis, biomicroscopy of the eyelid margin and location of the trichiasis. All patients were examined at 1, 3, 6, and 12 months after surgery. Eyelid thickness was measured at 1 month and 12 months of follow up. RESULTS Preoperatively the mean thickness of the eyelid margin was 0.99 mm ± 0.06 standard error. One month postoperatively eyelid thickness increased to 2.08 ± 0.28 standard error, decreasing over time to reach 1. 48 mm ± 0.18 standard error 1 year after surgery, a value that did not differ from that of the control group. Small granulomas were diagnosed only in the early phases of the postoperative period. Trichiasis recurrence was seen in just 1 eyelid. Isolated abnormal lashes lateral or medial to the graft were present in 5 eyelids at the end of the study. CONCLUSIONS Autogenous tarsal grafts placed parallel to the lower tarsus are a good option both to correct misdirected eyelashes and to restore normal lower eyelid thickness.

Cosmetic lower blepharoplasty is one of the most common procedures in an oculoplastic surgeon practice, with lower eyelid rejuvenation having a significant impact on age, attractiveness, and fatigue appearance. This chapter will describe the fundamental aspects of the transconjunctival lower blepharoplasty, with and without fat transposition. Historical background, the complex anatomy of the lower eyelid, patient evaluation, surgical techniques, and the evolving concepts of facial aging will be discussed.

Skin cancer is an important health concern, with an increasing incidence worldwide.To assess the clinical and economic burden of melanoma (MM) and non-melanoma skin cancer (NMSC) at public hospitals in mainland Portugal.We used an administrative database containing a registration of all hospitalizations and ambulatory episodes occurred in Portuguese public hospitals between 2011 and 2015. We assessed all episodes with associated diagnoses of MM or NMSC regarding neoplasm location, metastases occurrence, length of stay, in-hospital mortality and hospital costs.We assessed 15,913 MM and 72,602 NMSC episodes. 14.3% of MM episodes presented with metastases, compared to 1.9% of NMSC episodes. Patients' median age was lower for MM (66 years) than NMSC (76 years). The trunk was the most common location for MM (32.5%), followed by the lower limbs (26.5%). NMSC presented with higher length of stay than MM (median 5 versus 4 days; p 0.001), but with lower in-hospital mortality (7.3% versus 11.9%; p 0.001). MM episodes had higher average hospital costs than NMSC episodes (1197.7 versus 1113.5 €; p 0.001). Overall, NMSC episodes amounted a total of 80.8 million € in hospital costs versus 19.1 million € for MM episodes.Skin neoplasms have substantial impact on healthcare services. NMSC is an important contributor to this burden. NMSC underreporting should be tackled and it should not be downplayed in skin cancer preventative strategies.

Incidence of skin cancer is increasing worldwide and UV exposure at a young age is an important risk factor. To compare sun exposure-related knowledge and behaviour among children during school and holiday periods. A cross-sectional study was undertaken at 12 Oporto public primary schools. Educational sessions for educators were head by dermatologists every spring from 2004 to 2012. An educational activity book, Play and Learn with Jo Spots, was distributed to all primary school children and was explained by the educators every year. A questionnaire about sun exposure and behaviour was given to students in 2004 and 2012. In total, 2,114 students answered the questionnaire (1,233 in 2004 and 881 in 2012). Children practiced more outdoor sports in 2012 than in 2004 (86% vs 56%; p

Introduction: Lentigo maligna (LM) is a rare form of in situ melanoma, frequently seen as a large patch in elderly patients. The aim of this study was to assess clinical and dermoscopic features of LM. Material and Methods: A retrospective study of LM patients presenting to our center between July 2007 and July 2017 was performed. Demographic data, anatomical location, laterality, diameter, Clark level, Breslow stage, “ABCD” signs and dermoscopic features were registered. Facial versus extrafacial LM were compared. Results: We found 21 LM, of which 12 had an extrafacial location and 9 a facial location. Half of the extrafacial lesions were located on an upper limb. The median age at diagnosis was 63 years (ranging from 38 to 84 years). Most LM cases were female (16/21) with phototype II (13/21). More than half of the patients (11/21) had a history of a skin neoplasm or actinic keratosis. The median diameter found was 6 mm (interquartile range = 4.5 mm), ranging from 1 to 15 mm. Five lesions were invasive (median Breslow depth of 0.2 mm), and 4 of them were extrafacial. Discussion: In this study LM was more frequently found in an extrafacial location and as a small patch with a 6-mm diameter medium. The epidemiology of LM/LM melanoma might be changing. Full body examination and dermoscopy are of the utmost importance for the diagnosis. Dermatologists should be aware and search for small lesions outside the face and neck, particularly in middle-aged female patients with photo-damaged skin.

Gossypiboma and textiloma are terms used to describe tumor-like masses caused by retained gauze or surgical sponges after any operation. It is a rare surgical complication, usually difficult to diagnose due to its variable clinical presentation and nonstandard radiological appearance. We describe here a rare case of orbital gossypiboma in a child after surgical correction of an orbital blowout fracture.

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Pachydermoperiostosis is a rare genetic disease that causes major bone and skin changes. Severe ptosis is a prominent finding in this peculiar condition. We report here 2 patients with typical features of pachydermoperiostosis. Their tarsal plates were enlarged and infiltrated by sebaceous gland tissue. One patient also displayed diffuse intratarsal lacrimal gland hyperplasia. As far as we know, this is the first report of ectopic lacrimal tissue in pachydermoperiostosis. Tarsal and skin morphologic changes should be addressed during ptosis correction in these patients.

PURPOSE: To describe the involvement of the lacrimal gland (LG) in blepharophimosis-ptosis-epicanthus inversus syndrome (BPES). DESIGN: Observational, cross-sectional study. PARTICIPANTS: Twenty-one patients with BPES (10 female, 11 male) aged on average 15 years (range, 2-39 years), from 3 Brazilian medical centers and 1 Portuguese medical center. METHODS: Patients had their ocular surface evaluated with slit-lamp biomicroscopy, and tear production quantified with the Schirmer test I. The LG volumes were measured on computed tomography (CT) scans in the BPES sample and in a group of age-matched subjects imaged for nonorbital diseases. Sixteen patients were screened for mutations in the FOXL2 gene. MAIN OUTCOME MEASURES: Lacrimal meniscus height, Schirmer test I, presence of superficial punctate keratopathy (SPK), LG volume, and molecular analysis of the FOXL2 gene. RESULTS: Absence of LG was detected bilaterally in 9 patients (42.8%) and unilaterally in 2 patients (9.5%). When considering only patients with measurable LG, the median volume was 0.22 cm3 in the right eye (range, 0.06-0.36 cm3) and 0.24 cm3 in the left eye (range, 0.08-0.34 cm3). These values were significantly lower than those for the age-matched controls (median = 0.54 right eye and 0.53 left eye; P < 0.05). There was a significant association between deficiency of tear production and LG volume reduction and agenesis. Molecular analysis of the FOXL2 gene revealed the presence of 8 distinct mutations, 4 of them novel ones. A significant reduction of LG size or agenesis was associated with mutations affecting protein size (due to underlying changes in the stop codon location) or the DNA-binding forkhead domain (Fisher exact test, P = 0.021). In 3 probands, the underlying genetic defect was not found. CONCLUSIONS: This is the first study reporting LG volumes in BPES, describing a significant number of patients with LG agenesis. The association between alacrima and BPES is not incidental, and a thorough evaluation of tear production is recommended especially if ptosis surgery is planned. info:eu-repo/semantics/acceptedVersion

The aim of this study is to evaluate the role of general practitioners (GP) in selecting higher risk population for skin cancer screening. GP's training was organized to examine a specific high risk population consisting mainly of fisherman and farmers in a city of North of Portugal. Health care professionals of local health units training was performed by two dermatologists 2 months before the screening. During 8 weeks GPs selected patients with skin cancer suspicious lesions and/or risk factors consecutively from their regular consultation. These selected patients were referred to a dermatologist evaluation. Six dermatologists using manual dermoscopy examined the previously selected patients. One hundred eight patients have been screened, 35 % of which were males and 65 % females, with a mean age of 54 years. Full skin evaluation by dermatologists revealed 31 % of actinic keratosis, 5 % of leucoplasia, 7 % of basal cell carcinoma, 8 % of squamous cell carcinoma, and 1 % of melanoma. Cohen's kappa coefficient between dermatologist and GPs was 0.18. Selective screening with collaboration of GPs allowed the detection of more cases of skin cancer than the nonselective screenings in the literature. Although the diagnostic agreement between GPs and dermatologists was not good, our results indicate that active collaboration of dermatologists with primary health care units for selective skin cancer screening, including post graduated courses on their own health units, can be a way of optimizing early detection of cutaneous pre malignant and malignant lesions.

Purpose Graves lower eyelid retraction (GLLR) is a common and controversial sign of Graves orbitopathy. The authors reviewed the mechanisms and surgical techniques currently used to correct this Graves orbitopathy-related eyelid malposition. Methods A literature search was performed on the MEDLINE database using the keywords "lower eyelid retraction," "Graves orbitopathy," "thyroid ophthalmopathy," "thyroid eye disease," "spacers," and "eyelid surgery." Only articles in English were included. The level of evidence of publications regarding surgical correction of GLLR was evaluated and graded from I to IV, using a rating system adapted from a validated scientific evidence classification method. Results The mechanisms responsible for GLLR are not fully understood and no subtypes of GLLR have been distinguished. The surgical literature of GLLR encompasses mainly descriptions of surgical techniques without objective measurements of the results, and uncontrolled studies. Only 1 randomized controlled trial was identified. To lengthen the lower retractors, a large variety of different materials have been used as spacers. Conclusions The scientific literature on GLLR is vast, however controlled and randomized studies comparing different surgical techniques are lacking. At the present moment it is not clear if bioengineered materials are superior to conventional autogeneous tissue as lower eyelid spacers. The effects of GLLR on lower eyelid movements and contour have not been studied.

Glomus tumor is a rare benign vascular hamartoma derived from the modified smooth muscle cells of the glomus body. Paroxysmal pain is the leading symptom. A 78-year-old woman presented at our clinic with a 50-year history of a slow growing painful purple blue tender nodule under the right fourth finger nail. Dermoscopy and magnetic ressonance confirmed the presence of a large subungual tumor and total surgical excision was performed after partial nail plate avulsion. Histologic examination confirmed a glomus tumor. After 1 year of follow-up, the nail grew with normal shape and neither complications nor recurrence were observed.

Retronychia represents proximal ingrowth of the nail that occurs when the nail embeds backwards into the proximal nail fold. It is suspected when there is a persistent paronychia, particularly in the setting of trauma. Important clinical criteria for diagnosis are inflammation of the proximal nail fold, granulation tissue emerging from under the nail fold, thickening of the proximal portion of the nail plate and interruption of nail growth. The condition is rarely diagnosed and often misinterpreted, and is therefore unnecessarily treated with systemic antibiotics and antifungals. Avulsion of the nail confirms the diagnosis and it is the curative treatment. Conservative treatment with an adhesive technique is a valid option in early cases. We report 20 cases of retronychia diagnosed in our department between 2010 and 2013.

® ) is an oral multikinase inhibitor that blocks tumor cell proliferation and angiogenesis and is used for the treatment of advanced renal cell carcinoma, hepatocellular carcinoma, and other solid tumors including metastatic differentiated thyroid cancer, at a starting dose of 400 mg twice daily (1-4). The most common adverse effects are fatigue, diarrhea, and various other dermatologic side effects (1). Recently, a meta-analysis of dermatological toxicities associated with sorafenib has been published, (5) and a hand-foot-skin reactions (HFSR) was most often found, but also rash/desquamation, alopecia, pruritus and dry skin. Nail involvement has been reported, in particular sub-ungual splinter hemorrhages (6). We describe a patient with longitudinal melanonychia and blue lunulae that developed during sorafenib therapy of metastatic thyroid carcinoma.

BACKGROUND Nail unit melanoma (NUM) is a variant of acral lentiginous melanoma. The differential diagnosis is wide but an acquired brown streak in the nail of a fair-skinned adult person must be considered a potential melanoma. Dermoscopy helps clinicians to more accurately decide if a nail apparatus biopsy is necessary. OBJECTIVE Detailed evaluation of clinical and dermoscopy features and description of conservative surgery of in situ NUM. METHODS Retrospective study of in situ NUM diagnosed and treated with conservative surgical management in the authors' center from 2008 to 2013. RESULTS Six cases of NUM were identified: 2 male and 4 female patients, age range at diagnosis of 44 to 76 years. All patients underwent complete nail unit removal with at least 6-mm security margins around the anatomic boundaries of the nail. The follow-up varies from 4 to 62 months. CONCLUSION Nail unit melanomas pose a difficult diagnostic and therapeutic challenge. Wide excision is sufficient, whereas phalanx amputation is unnecessary and associated with significant morbidity for patients with in situ or early invasive melanoma. Full-thickness skin grafting or second-intention healing after total nail unit excision is a simple procedure providing a good functional and cosmetic outcome.

Auteur(s) : Ana Filipa Duarte1, Osvaldo Correia1,2, Rosa Azevedo3, Maria do Carmo Palmares2, Luis Delgado2 1Centro de Dermatologia Epidermis, Instituto CUF, Apartado 55031, 4050-401 Porto, Portugal 2Department of Immunology, Faculty of Medicine, University of Porto, Portugal 3Department of Pathology, Instituto Portugues de Oncologia, Porto, Portugal Fixed drug eruption (FDE) is a skin drug eruption that usually appears as a solitary or a small number of well circumscribed, cutaneous [...]

A short frenulum is not uncommon and is an important cause of male dyspareunia. Surgical frenuloplasty may alleviate symptoms, but there is a risk of residual scarring leading to further symptoms. Laser frenuloplasty, consisting of frenulum vaporization by laser CO2, is an alternative simple, safe and efficacious procedure. We report three cases of successful treatment of a short frenulum with laser CO2 with good aesthetical results and complete resolution of symptoms.

Background: Eccrine hidrocystomas are rarely described benign cystic lesions, mainly presenting in middle-aged women in the centrofacial area and usually associated with a chronic course, seasonal variability and no proved and consistently efficacious treatments. Case Report: We report 2 patients, a 45- and a 56-year-old woman, with multiple facial eccrine hidrocystomas suggested by dermatoscopy, confirmed by histology and treated with botulinum toxin A with excellent results. Conclusion: Our report stresses the role of dermatoscopy in the diagnosis and follow-up of these lesions, as well as the impact of repeated treatments with botulinum toxin A in eccrine hidrocystomas suggesting it as a first-line treatment for multiple eccrine hidrocystomas because it is easy to use and has no risk of scars.

Congenital atrichia (CA) is a rare form of irreversible alopecia with an autosomal recessive mode of inheritance, usually associated with a mutation in the human hairless (HR) gene located at chromosome 8. Papular lesions may develop as an additional phenotypic feature. Herein we describe a case of CA supported by trichoscopy, histology and genetic analysis. The patient’s single brother had also universal alopecia. To our knowledge this is the second report of a specific pathogenic mutation (c.2818C>T) of the HR, which until now had only been identified in a family with CA and papular lesions, emphasizing the difficulty to establish a strict correlation between HR genotyping and the phenotype.

Despite progress in understanding the molecular basis, the diagnosis of neurofibromatosis 1 (NF 1) is based on clinical criteria, established by the National Institute of Health (NIH) Consensus Conference in 1987. The association of NF1 and piebaldism has been reported, but some authors disagree with this co-occurrence. In the light of present knowledge, we highlight that both entities might co-exist in the same patient.

The prevalence of atopic dermatitis is increasing in western societies and this disease has a significant effect on the quality of life of patients and their families. The pathophysiology is complex, but there are recent insights on innate and adaptive immunity dysfunction in this condition that increases the predisposition to colonization/infection with characteristic types of microorganisms. We provide an up-to-date review of the pathophysiology of atopic dermatitis, highlighting the clinical implications of skin barrier dysfunction and immunological deregulation. We suggest a useful approach for handling exacerbations and infectious events.

Docetaxel is a semisynthetic taxane used in the treatment of several malignancies. A 60-year-old male patient suffering from a non–small cell lung cancer with bone metastasis was under treatment with docetaxel and developed acral erythema involving photoexposed areas. Onycholysis and melanonychia were also observed. Drug-induced acral erythema usually involves the palms and soles, but it may occur in more atypical areas such as the backs of the hands and feet and also the face, as occurred in this patient. The nail changes are usually observed after several cycles of docetaxel, but tend to resolve gradually over several weeks despite continued treatment. Early recognition of skin and nail changes in the setting of docetaxel treatment, especially with an unusual presentation, is of paramount importance not only for patient reassurance, but also to avoid unnecessary withdrawal of drug treatment.

Cutaneous mastocytosis is characterized by increased numbers of skin mast cells that release mediators causing pruritus, urticaria, and flushing. Most pediatric mastocytosis patients exhibit the pattern of urticaria pigmentosa, which typically appears during the first two years of life and resolves spontaneously in late adolescence. However, while the disease is active, patients are frequently symptomatic and uncomfortable, which justifies symptomatic treatment. We report 2 patients, a 14-month-old girl and a 26-month-old boy, with localized cutaneous erythematous lesions with a positive Darier sign. In each, a punch biopsy confirmed the diagnosis of mastocytosis. Treatment was instituted with pimecrolimus cream twice a day and oral antihistamine. An almost complete response was achieved after 4 months of therapy in both patients, with no clinical evidence of recurrence after 4 years and 2 years of follow-up, respectively. In children, the treatment of mastocytosis is directed primarily to avoiding potential mast cell degranulating agents and alleviating symptoms. Topical calcineurin inhibitors act by inhibiting T-cell activation and cytokine release; they may suppress mast cell- mediated reactions by reducing their degranulation. These two cases suggest that in localized cutaneous mastocytosis they are a safe and efficacious alternative to topical steroid therapy.

Author(s): Ferreira, Olga; Duarte, Ana Filipa; Baudrier, Teresa; Mota, Alberto; Simoes, Joana Sobrinho; Azevedo, Filomena

Erythema multiforme major (EMM) is an acute, self-limited mucocutaneous disease characterized by the abrupt onset of symmetrical fixed red papules evolving to target lesions. It is triggered mainly by infections, such as herpes simplex virus (HSV) and Mycoplasma pneumoniae, or drugs. In instances of extensive skin lesions with “giant” targets, prominent involvement of several mucous sites and fever, it may be difficult to distinguish from Stevens-Johnson syndrome (SJS), a rarer, life-threatening reaction which is mainly drug-induced. We report a 7-year old boy with SJS and Chlamydia pneumoniae infection and 3 patients with erythema multiforme (EM) and co-infection with Chlamydia pneumoniae: a 3-year old girl and a 29-year old man developed EMM lesions associated to Mycoplasma pneumoniae and Chlamydia pneumonia and a 20-year old woman with EMM associated to herpes simplex type 2 and Chlamydia pneumoniae infection. None of the 4 patients had history of drug intake in the last two months. Chlamydia pneumoniae is an intracellular bacteria responsible for respiratory infections. Despite the fact that its role in SJS/EMM has been rarely reported, our cases suggest that it may cause SJS and trigger EM when co-infecting a patient, either with Mycoplasma pneumoniae or herpes simplex. We conclude that infection by Chlamydia pneumoniae should be suspected and ruled out in every patient with SJS/EMM, especially in those with signs of respiratory infection.

Background: Nail unit melanoma (NUM) is a rare variant of acral lentiginous melanoma. The differential diagnosis is wide but an acquired brown streak in the nail of a fair-skinned person must be considered a potential melanoma. Dermatoscopy helps clinicians to more accurately decide if a nail apparatus biopsy is necessary. Methods: We report the case of a 61-year-old Caucasian woman with melanonychia occupying the central portion of the right thumbnail plate with 1 year of evolution. Dermatoscopy showed a brown pigmentation overlaid by longitudinal irregular lines. An excisional biopsy was performed, and pathological examination revealed melanoma in situ. For safety reasons, the nail unit was totally removed down to the phalangeal bone 3 weeks later, and a full-thickness skin graft taken from the arm was used for reconstruction. Conclusion: NUMs pose a difficult treatment challenge. Wide excision with phalanx amputation is not satisfactory for patients with in situ and early invasive melanoma. Full-thickness skin grafting after total nail unit excision is a simple procedure providing a good functional and cosmetic outcome.

Unilateral laterothoracic exanthem is a self-limited disease that occurs most commonly in children. It is characterized by unilateral exanthem, often in axillary region. The etiology is unknown, but a viral agent is suspected. We report a 1-year-old white girl with unilateral laterothoracic exanthem associated with Epstein Barr virus infection, suggesting this virus has a possible etiologic role.

Pellagra is a nutritional disease caused by the deficiency of niacin. It presents with a photodistributed rash, gastrointestinal symptoms, and neuropsychiatric disturbances. In the Western world, this disease is mostly confined to alcoholics or the impoverished. However, this condition must be recognized in other clinical settings because it is easily treated and can be fatal if not identified. We describe a case of pellagra caused by esophageal carcinoma and alcoholism; we also review the literature.

Eyelid lesions frequently are a diagnostic challenge. We report a case of a 46-year-old woman with a 5-year history of yellowish symmetric progressively growing papules on the eyelids, resembling xanthelasma. A skin biopsy was performed that revealed the rare variant of clear cell syringoma. The lesions were treated with CO2 laser and surgical excision; there was no evidence of recurrence after 6 months of follow-up.