Quality of life in heterosexual menopausal women: The indirect effect of sexual and marital satisfaction, menopause representations, and psychological morbidity.

Menopause may negatively impact Quality of Life (QoL). Our study used a cross-sectional design and research participants were 99 women in natural menopause. In our study, we analyzed the relationship between age at menopause onset, hormone therapy use, duration of couple relationship, menopause duration, psychological morbidity, marital satisfaction, menopause representations, and QoL following the Wilson and Cleary Health-Related QoL conceptual model. The authors found that negative representations, lower marital satisfaction, psychological morbidity, and shorter duration of menopause contributed to lower QoL. Moreover, the authors found that psychological morbidity and menopause representations (identity and control/cure dimensions) had an indirect effect between marital satisfaction and vasomotor, psychosocial, and sexual QoL. Also, we found that age at the onset of menopause showed a moderating effect in the final model. Future studies should replicate these results in a longitudinal design and analyze how the variables that showed a moderating role and indirect effects will function as moderators and mediators, respectively, over time.

Primary cutaneous adenoid cystic carcinoma of the abdomen: a rare entity.

Adenoid cystic carcinoma is a rare neoplasm that arises from secretory glands, most frequently from the salivary glands. Primary cutaneous adenoid cystic carcinoma is microscopically identical to adenoid cystic carcinoma developing at other tissues. Therefore, differentiating between a primary cutaneous adenoid cystic carcinoma and an extracutaneous adenoid cystic carcinoma with cutaneous metastases is pivotal to determine its prognosis and management. We describe a case of primary cutaneous adenoid cystic carcinoma on the abdomen that was successfully treated with wide excision.

Adult blaschkolinear acquired inflammatory skin eruption (BLAISE) with simultaneous features of lichen striatus and blaschkitis.

Blaschkitis and lichen striatus are generally distinguished in the literature by the age of onset, lesion distribution, and histopathology. However, there is currently no clear consensus among authors about whether to consider blaschkitis and lichen striatus different clinical entities or a spectrum ofthe same disease. We present a case of adult BLAISE with features of both lichen striatus and blaschkitis, which seems to support the theory that these clinical entities may in fact represent a spectrum of the same pathological process.

Photoallergic reaction to cyamemazine.

A 50-year-old man presented with a scaly erythema of the face, upper chest, forearms, and dorsum of the hands. He has been treated with cyamemazine for 6 months. Photopatch tests were performed and the patient was diagnosed with photoallergic reaction to cyamemazine. The drug was discontinued and a course of oral steroids was prescribed. The patient was advised to avoid light exposure. There has been no evidence of recurrence during a six-month follow-up period. Photoallergic reactions are much less frequent than phototoxic disorders. It is well known that several drugs including neuroleptics of the phenothiazine family may produce a skin eruption on light-exposed areas by dose-dependent (phototoxic) or photoallergic mechanisms. It is believed that photopatch testing, which is the clinical investigation of choice for suspected photoallergic reactions, is significantly underused in Europe and probably world-wide.

Dermatology inpatient consultation in a Portuguese university hospital.

BACKGROUND: Cutaneous findings are frequent in hospitalized patients. There are few reports regarding this subject. OBJECTIVES: To identify the frequency and the impact on clinical courses of dermatologic conditions in patients in the inpatient setting and compare the data with other similar studies. METHODS: Retrospective review of 274 hospitalized patients in non-dermatology inpatient departments who were observed by a dermatology consultant in a Portuguese central university hospital during a year. RESULTS: A total of 282 consultations were performed. The services requesting consultation most frequently were internal medicine (33.7%), surgery (10.3%), and pediatrics (8.9%). Skin infections (33.2%), eczemas (9.5%), and drug eruptions (7.3%) were the most common diagnoses. Admission diagnosis was modified in 9 cases (3.3%) by the dermatology consultant. CONCLUSION: Dermatoses are frequently misdiagnosed by non-dermatologists. Common skin diseases were responsible for most of dermatology inpatient consultations. However, in some cases the dermatology consultation changed the primary main diagnosis and had an important impact on the clinical course.

Letter: Pellagra as the initial presentation of Crohn disease.

Pellagra is a nutritional disease caused by the deficiency of niacin. We describe a case of pellagra as the initial presentation of Crohn disease, which has been rarely described in the literature.

Frontal fibrosing alopecia: a review of eleven patients.

BACKGROUND: Frontal fibrosing alopecia (FFA) is a primary lymphocytic cicatricial alopecia with a distinctive clinical pattern of progressive frontotemporal hairline recession. OBJECTIVES: Our purpose was to describe the clinical and histopathological features as well as the response to treatment of eleven cases of FFA diagnosed at the Trichology Consultation, over three years. METHODS: A retrospective case note review was performed of eleven adult patients with FFA. The clinical data as well the histopathologic findings and laboratory tests were accessed. The patients were treated with different drugs, depending on the stage of the disease. The age of onset of the alopecia ranged from 45 to 80 years. Ten patients (90.9%) were postmenopausal women. All patients had progressive and symmetrical alopecia localized to the frontal and temporal hairline. Seven patients (63.6%) had marked decreased to complete loss of eyebrows and in four patients axillar alopecia was also evident. Laboratory investigations were normal. Scalp biopsy specimens from the anterior hairline showed similar findings. No significant improvement was observed in the majority of cases. CONCLUSION: Differential diagnosis should take into account several other conditions. It seems there is no effective treatment proven with an appropriate level of evidence in the management of FFA.

IL-31 and IL-8 in Cutaneous T-Cell Lymphoma: Looking for Their Role in Itch.

The itch associated with cutaneous T-cell lymphoma (CTCL), including Mycosis Fungoides (MF) and Sézary syndrome (SS), is often severe and poorly responsive to treatment with antihistamines. Recent studies have highlighted the possible role of interleukins in nonhistaminergic itch. We investigated the role of IL-31 and IL-8 in CTCL, concerning disease severity and associated itch. Serum samples of 27 patients with CTCL (17 MF and 10 SS) and 29 controls (blood donors) were analyzed for interleukin- (IL-) 31 and IL-8; correlations with disease and itch severity were evaluated. IL-31 serum levels were higher in CTCL patients than in controls and higher in SS than in MF. Also, serum IL-31 levels were higher in patients with advanced disease compared to those with early disease, and they correlated positively with lactate dehydrogenase and beta 2-microglobulin levels, as well as with the Sézary cell count. Itch affected 67% of CTCL patients (MF: 47%; SS: 100%). Serum IL-31 levels were higher in itching patients than in controls and in patients without itching. There was no association between serum IL-8 and disease severity, nor with itching. Serum IL-8 levels correlated positively with peripheral blood leukocyte and neutrophil counts in CTCL patients. Our study suggests a role for IL-31 in CTCL-associated itch, especially in advanced disease and SS, offering a rational target for new therapeutic approaches. Increased serum IL-8 observed in some patients may be related to concomitant infections, and its role in exacerbating itch by recruiting neutrophils and promoting the release of neutrophil proteases deserves further investigation.

[Cutaneous Manifestations in Mastocytosis: Update]. / Manifestações Cutâneas nas Mastocitoses: Atualização.

INTRODUCTION: Mastocytosis is characterized by the clonal expansion of morphological and immunophenotypically abnormal mast cells in different organs. The skin is the most frequently affected tissue. Virtually all children and more than 80% of adult patients with mastocytosis show cutaneous lesions. MATERIAL AND METHODS: The present article describes the symptoms and signs in cutaneous mastocytosis, based on the review of recently published international consensus guidelines. DISCUSSION: According to the 2016 World Health Organization classification, mastocytosis can be divided in cutaneous mastocytosis, systemic mastocytosis and mast cell sarcoma. Cutaneous mastocytosis is subclassified in three subtypes: maculopapular cutaneous mastocytosis, diffuse cutaneous mastocytosis and cutaneous astocytoma. Telangiectasia macularis eruptiva perstans is no longer considered a distinct entity. CONCLUSION: Based on the age of onset, cutaneous manifestations of mastocytosis can be variable. The classification of cutaneous mastocytosis has recently been updated. Typically, in patients with childhood-onset mastocytosis, the disease occurs as cutaneous mastocytosis and shows spontaneous resolution around puberty. In contrast, adult patients, despite having also cutaneous lesions, often show systemic involvement and the course of the disease is usually chronic.

Introdução: As mastocitoses caraterizam-se pela expansão clonal de mastócitos, com acumulação de mastócitos morfológica e imunofenotipicamente anormais em diferentes órgãos. A pele é o órgão mais frequentemente envolvido. Virtualmente, todas as crianças e mais de 80% dos adultos com mastocitose apresentam lesões cutâneas.Material e Métodos: O presente artigo descreve os sinais e sintomas associados à mastocitose na pele, tendo por base a revisão das normas de orientação de consenso internacionais, recentemente publicadas.Discussão: De acordo com a classificação proposta pela Organização Mundial de Saúde em 2016, a mastocitose divide-se em mastocitose cutânea, mastocitose sistémica e sarcoma de mastócitos. A mastocitose cutânea pode subdividir-se em três subtipos: a mastocitose cutânea maculopapular (também denominada urticária pigmentosa), mastocitose cutânea difusa e mastocitoma cutâneo. A telangiectasia macular eruptiva perstans já não é considerada uma entidade independente.Conclusão: As manifestações cutâneas da mastocitose são variáveis, dependendo da idade de início da doença. Recentemente a classificação da mastocitose cutânea foi atualizada. Nas crianças, a mastocitose ocorre como mastocitose cutânea que tende à regressão espontânea durante a adolescência. Quando tem início na idade adulta, a mastocitose é geralmente sistémica, sendo a forma mais frequente a mastocitose sistémica indolente, que normalmente também cursa com manifestações cutâneas e tem um curso crónico.

Systemic mastocytosis with KIT V560G mutation presenting as recurrent episodes of vascular collapse: response to disodium cromoglycate and disease outcome.

BACKGROUND: Mastocytosis are rare diseases characterized by an accumulation of clonal mast cells (MCs) in one or multiple organs or tissues. Patients with systemic mastocytosis (SM), whose MCs frequently arbor the activating D816V KIT mutation, may have indolent to aggressive diseases, and they may experience MC mediator related symptoms. Indolent SM with recurrent anaphylaxis or vascular collapse in the absence of skin lesions, ISMs(-), is a specific subtype indolent SM (ISM), and this clonal MC activation disorder represents a significant fraction of all MC activation syndromes. The V560G KIT mutation is extremely rare in patients with SM and its biological and prognostic impact remains unknown. CASE PRESENTATION: A 15-year old boy was referred to our hospital because of repeated episodes of flushing, hypotension and syncope since the age of 3-years, preceded by skin lesions compatible with mastocytosis on histopathology that had disappeared in the late-early childhood. Diagnosis of ISM, more precisely the ISMs(-) variant, was confirmed based on the clinical manifestations together with increased baseline serum tryptase levels and the presence of morphologically atypical, mature appearing (CD117+high, FcεRI+) phenotypically aberrant (CD2+, CD25+) MCs, expressing activation-associated markers (CD63, CD69), in the bone marrow. Molecular genetic studies revealed the presence of the KIT V560G mutation in bone marrow MCs, but not in other bone marrow cells, whereas the screening for mutations in codon 816 of KIT was negative. The patient was treated with oral disodium cromoglycate and the disease had a favorable outcome after an eleven-year follow-up period, during which progressively lower serum tryptase levels together with the fully disappearance of all clinical manifestations was observed. CONCLUSIONS: To the best of our knowledge this first report of a patient with ISM, whose bone marrow MCs carry the KIT V560G activating mutation, manifesting as recurrent spontaneous episodes of flushing and vascular collapse in the absence of skin lesions at the time of diagnosis, in whom disodium cromoglycate had led to long term clinical remission.

Adult mastocytosis: a review of the Santo António Hospital 's experience and an evaluation of World Health Organization criteria for the diagnosis of systemic disease.

BACKGROUND: Mastocytosis is a clonal disorder characterized by the accumulation of abnormal mast cells in the skin and/or in extracutaneous organs. OBJECTIVES: To present all cases of mastocytosis seen in the Porto Hospital Center and evaluate the performance of World Health Organization diagnostic criteria for systemic disease. METHODS: The cases of twenty-four adult patients with mastocytosis were reviewed. Their clinical and laboratorial characteristics were assessed, and the properties of the criteria used to diagnose systemic mastocytosis were evaluated. RESULTS: The age of disease onset ranged from 2 to 75 years. Twenty-three patients had cutaneous involvement and 75% were referred by dermatologists. Urticaria pigmentosa was the most common manifestation of the disease. One patient with severe systemic mast cell mediator-related symptoms showed the activating V560G KIT mutation. The bone marrow was examined in 79% of patients, and mast cell immunophenotyping was performed in 67% of the participants. Systemic disease was detected in 84% of cases, and 81% of the sample had elevated serum tryptase levels. All the diagnostic criteria for systemic mastocytosis had high specificity and positive predictive value. Bone marrow biopsy had the lowest sensitivity, negative predictive value and efficiency, while the highest such values were observed for mast cell immunophenotyping. Patients were treated with regimens including antihistamines, sodium cromoglycate, alpha-interferon, hydroxyurea and phototherapy. CONCLUSIONS: Cutaneous involvement is often seen in adult mastocytosis patients, with most individuals presenting with indolent systemic disease. Although serum tryptase levels are a good indicator of mast cell burden, bone marrow biopsy should also be performed in patients with normal serum tryptase, with flow cytometry being the most adequate method to diagnose systemic disease.

Case for diagnosis.

We report a clinical case of a rare variant of pemphigus - pemphigus herpetiformis - which combines the clinical features of dermatitis herpetiformis with the immunological findings of pemphigus. Due to its atypical presentation, it is frequently misdiagnosed as dermatitis herpetiformis. It is basically characterized by the herpetiform pattern of skin lesions, severe pruritus and by the presence of eosinophilic spongiosis confirmed on histopathology. We call attention to the excellent response to dapsone.