Painless rash in a transplant patient.

Dermatophytes are common keratinophilic fungi responsible for superficial skin infections. Deep dermatophytosis is a rare form of invasive skin infection described in immunocompromised patients. We report the case of a 65-year-old man with a history of an orthotopic liver transplant for hepatocarcinoma 6 months earlier, who presented with small painless erythematous papules in lower limbs, some of which were umbilicated. Skin biopsy showed an intense non-necrotizing granulomatous reaction in the dermis around fungal structures. Trichophyton rubrum was identified as the causal agent through culture and internal transcribed spacer sequencing.

Epidermolysis Bullosa Pruriginosa successfully treated with concomitant topical and systemic agents.

Epidermolysis bullosa pruriginosa, a genetic mechanobullous disease, manifests at birth or late in life and is characterised by intense pruritus, resulting in lichenified or nodular prurigo-like lesions and scarring most prominent on the shins. Treatment is unsatisfactory. We report a patient treated with success using a combination of topical and systemic agents.

Plasmablastic lymphoma: an atypical cutaneous presentation of a rare entity.

Plasmablastic lymphoma is a very rare B-cell lymphoma typically associated with immunosuppression: It occurs primarily in the oral cavity, although some cases were reported in other organs and tissues.To date, only 10 cases of primary cutaneous plasmablastic lymphoma have been described. Clinically, primary cutaneous plasmablastic lymphoma presents as non-specific cutaneous lesions (purple nodules, erythematous infiltrated plaques). In previously described cases, as in this case, histology and immunohistochemistry are required to make the diagnosis. Owing to the rarity of this entity, there is no established therapy, which makes its management an individualized, patient-based decision.

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.

Giant cutaneous horn on the lower lip.

Cutaneous horn is a conical hyperkeratotic projection of the skin composed of compact keratin. A wide range of pathologic conditions may be found at its base, including a significant proportion of malignant tumors. A notable, giant cutaneous horn uncovering a keratoacanthoma/well-differentiated squamous cell carcinoma is presented, highlighting the importance of histopathological examination to rule out malignancy because clinical features cannot assure a correct diagnosis.

Chronic Urticaria in the Real-Life Clinical Practice Setting in Portugal: Baseline Results from the Non-Interventional Multicentre AWARE Study.

INTRODUCTION: There is a paucity of information regarding chronic urticaria patients' care in a real-world setting. The objective of this study was to report and evaluate the baseline characteristics of Portuguese chronic urticaria patients refractory to H1-antihistamines included in the AWARE study. MATERIAL AND METHODS: This is a non-interventional cohort study. Adult patients with a diagnosis of chronic urticaria with symptoms for at least two months, refractory to H1-antihistamines, consulting one of the 10 participating urticaria centers throughout Portugal have been included in the study. Baseline sociodemographic data, medical history, clinical parameters, medication, weekly urticaria activity score, and dermatology quality of life index have been collected. RESULTS: Seventy six patients were included, of which 76.3% were women. The majority of patients had a diagnosis of chronic spontaneous urticaria (88.2%) and 39.5% had angioedema. Around 91.0% of patients were medicated with non-sedative H1-antihistamines and 35.4% with a third line therapy. Median dermatology quality of life index was 5.0 and median weekly urticaria activity score was 13.0. DISCUSSION: The baseline results suggest that patients with chronic urticaria refractory to H1-antihistamines are being under-treated in the real-world setting. CONCLUSION: The AWARE study demonstrates the real impact of chronic urticaria on Portuguese patients refractory to H1-antihistamines treatment, and 30% report a very large or extremely large deleterious effect on their quality of life. The follow-up of these patients will allow evaluating strategies aimed at optimizing disease control.

Introdução: A informação sobre os doentes com urticária crónica em ambiente de vida real é escassa e este estudo teve por objectivo reportar e avaliar as características basais dos doentes portugueses com urticária crónica refractários aos anti-histamínicos H1 incluídos no estudo AWARE. Material e Métodos: Estudo de coorte não intervencional. Foram incluídos doentes adultos com diagnóstico de urticária crónica sintomáticos durante pelo menos dois meses, refratários aos anti-histamínicos H1, seguidos em 10 centros de urticária em Portugal. Foram recolhidos dados basais sociodemográficos, história clínica, parâmetros clínicos, medicação, índice semanal de atividade de urticária e índice de qualidade de vida dermatológico. Resultados: Foram incluídos 76 doentes, dos quais 76,3% mulheres. A maioria dos doentes estava diagnosticado com urticária crónica espontânea (88,2%) e 39,5% apresentavam angioedema. Cerca de 91,0% dos doentes estavam medicados com anti-histamínicos H1 não sedativos e 35,4% com terapêuticas de terceira linha. A mediana do índice de qualidade de vida dermatológico foi 5,0 e a mediana do índice semanal de atividade de urticária foi 13,0. Discussão: Os resultados basais sugerem que os doentes com urticária crónica refratários ao tratamento com anti-histamínicos H1 estão sub-tratados em ambiente de vida real. Conclusão: O estudo AWARE vem demonstrar o real impacto da urticária crónica nos doentes portugueses refratários ao tratamento com anti-histamínicos H1 onde mais de 30% reporta um impacto elevado ou extremamente elevado da doença na sua qualidade de vida. O seguimento destes doentes permitirá avaliar estratégias para otimização do controlo da doença.

Atopic dermatitis-like non-erythrodermic leukemic variant of CD3(-/+dim) CD4(+) cutaneous T-cell lymphoma preceded by cutaneous papular xanthomatosis.

We report a patient with cutaneous papular xanthomatosis who 4 years later developed a CD3(-/+dim)/CD4(+) T-cell lymphoma. Pruritic xerotic non-erythrodermic skin, eosinophilia and hyper-IgE were present and erroneously classified as atopic dermatitis. Flow cytometry and DNA ploidy analysis of both blood and skin lymphocytes, skin histology and blood T-cell receptor gene rearrangement studies confirmed diagnosis of T-cell lymphoma. Monoclonal CD3(-/+dim)/CD4(+) T-cells were especially prone to the synthesis of IL-13, a cytokine that is involved in IgE-secretion, and comprised both a medium (diploid) and large (hyperploid) sized T-cell populations with a similar immunophenotype. The majority of the normal residual T-cells were large granular lymphocytes, expressed activation-related and natural-killer-associated markers and secreted high levels of interferon gamma, suggesting that they might correspond to active cytotoxic cells directed against the neoplastic T-lymphocytes.

[Three years incidence of dermatophytes in a hospital in Porto (Portugal)]. / Incidência de dermatófitos, durante três anos, num hospital do Porto (Portugal).

We evaluated the incidence of dermatophytes isolated at our hospital in the years of 1997 to 2000 and correlated it with anatomical site and age. Trichophyton rubrum was the predominant species in all anatomical sites, excluding scalp, followed by Microsporum canis, the leading agent of tinea capitis. All dermatophytosis, except tinea capitis by M. canis and Trichophyton schoenleinnii appeared mainly in adult patients. Our results revealed no substantial differences to other portuguese studies regarding the major agents. We found a relatively high incidence of T. schoenleinnii as second tinea capitis agent.

Breast cancer: 2 case reports.

Breast cancer is the most frequently diagnosed life-threatening cancer in women and the leading cause of cancer death among them worldwide. It includes a heterogeneous collection of diseases with various histologically defined subsets, clinical presentations, responses to treatment and outcomes. We describe 2 cases of female patients with ductal breast carcinoma. Dermatologists may have an important role in diagnosing such diseases.

[Psoriasis induced by infliximab]. / Psoríase induzida por infliximab.

INTRODUCTION: Recently, the tumor necrosis factor-α inhibitors (anti-TNF-α) have been extensively used in clinical practice, in the treatment of several immune-mediated disorders, such as inflammatory bowel diseases, rheumatoid arthritis and more recently in psoriasis. CASE REPORT: A 35-year-old woman, suffering from Crohn's disease, diagnosed in 1994, successfully treated with infliximab at the dosage of 5 mg/Kg every 8 weeks, since 10 months ago, was referred to our Dermatology Department due to the development of erythematic-scaling confluent plaques on the scalp, back and umbilical fold. The skin biopsy confirmed the clinical diagnosis of psoriasis. Given the severity of the skin lesions and its relation with the anti-TNF-α, we decided to discontinue infliximab. DISCUSSION: Psoriasis results from the combination of polygenic predisposition and several triggering factors. Paradoxically, it has been described an increase of psoriasis induced by biologic agents. The pathogenic mechanism of such paradoxical effect has not yet been clearly elucidated.

Disseminated superficial porokeratosis in a patient with cholangiocarcinoma: a paraneoplastic manifestation?

Porokeratosis refers to a group of hereditary or acquired disorders of epidermal keratinization and is characterized histologically by the presence of a cornoid lamella. The clinical variant referred to as disseminated superficial porokeratosis has been described in the literature in association with immunosuppressive conditions that include organ transplant, infections and immunosuppressive treatments. The association of disseminated superficial porokeratosis with solid organ malignancies has seldom been described, only 5 such cases having been published. The present report refers to a patient with lesions of disseminated superficial porokeratosis of sudden onset shortly before diagnosis of a cholangiocarcinoma.

[Psoriasis in the era of biologics]. / Psoríase na era dos biológicos.

Chronic plaque psoriasis is an immune-mediated, inflammatory skin disease with a heavy burden on quality of life of patients. Conventional systemic therapies, including cyclosporine, methotrexate, acitretin and photo(chemo)therapy, have proved to be effective, but the risk of toxicity prevents their prolonged and continuous use. Advances in the understanding of psoriasis immunopathogenesis have led to the development of drugs, designed to selectively interfere with the immune mechanisms that induce psoriasis, called biologics. These agents have proven to be a convenient, safe and effective alternative to conventional treatments, and have become an important part of the dermatologist therapeutic armamentarium. This review will focus on the mechanisms of action, guidelines for usage, efficacy data and safety concerns of the main biologics used in Europe for the treatment of moderate to severe plaque psoriasis: etanercept, infliximab, adalimumab. Although efalizumab's marketing authorization, approved since 2003, has been suspended recently (February 2009) across the European Union, it will also be briefly discussed.