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ABSTRACT: We report the case of a 72-year-old man with bilateral testicular involvement by mycosis fungoides (MF). The patient was diagnosed with MF 6 months prior, and testicular involvement was found upon staging. The rare behavior of MF, with early visceral involvement and no hematolymphoid organs affected, had a poor prognosis with patient death 14 months after the original diagnosis despite surgery and chemotherapy. The neoplastic cells showed a phenotypic switch from a CD4 + /CD8 - profile in the skin to a CD4 - /CD8 - in the testis and the same clone, confirmed with T-cell receptor gene rearrangement studies, making this the third reported case of MF affecting the testis and the first with clonality studies to confirm it. The clinical evolution may be related to its distinctive biology showcased in the neoplastic cell's switch to a more aggressive CD4 - /CD8 - profile and the early extension of the disease to an uncommon visceral site.

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ABSTRACT: Granulomatous slack skin (GSS) is a rare subtype of mycosis fungoides, and few cases have been known to spread to the blood, lymph nodes, or viscera. We present a case with early dissemination to the lung. A 27-year-old woman, previously healthy, presented with scattered disseminated scaly patches, associated with vulvar and intergluteal firm swelling and groin-skin induration for 1 year. She also reported mild fatigue and breathlessness on moderate exertion. The patient underwent blood tests, skin biopsies, and computed tomography scan. The skin biopsy showed a mildly atypical T-cell lymphoid infiltrate involving the dermis/hypodermis, with focal epidermotropism, associated with a granulomatous infiltrate and elastophagocytosis. The computed tomography scan revealed bilateral ground-glass lung nodular opacities. Positron emission tomography showed an increased signal in the skin and subcutis around the buttocks, inguinal and mediastinal lymph nodes, and lungs. The lung biopsy confirmed a dense T-cell infiltrate with numerous multinucleated giant cells. Subsequently, esophageal involvement was also observed following biopsy. Molecular analyses demonstrated identical T-cell clones in the skin and lung. After 6 cycles of chemotherapy/localized external radiotherapy, the patient had a partial skin response and stable lung disease. A preferred diagnosis of GSS with systemic spread was made based on clinical/histologic/molecular findings, after considering granulomatous mycosis fungoides and peripheral T-cell lymphoma, not otherwise specified. This case highlights the frequent diagnostic difficulty in distinguishing GSS from an inflammatory granulomatous dermatitis. Pulmonary and esophageal involvements are rare in GSS, and the simultaneous presentation of characteristic cutaneous GSS with systemic disease poses an additional classification challenge.

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INTRODUCTION: A person with diabetes is subject to developing micro and macrovascular complications and prevention requires an active role from the person. So, health literacy should have a preponderant role in the health of people with diabetes but this link is yet not fully understood. The objective of this study is to understand the relationship between health literacy and the prevalence of complications in people with diabetes mellitus type 2 (DM2). METHODS: This is a multicentric transversal observational exploratory study. A survey was conducted with two health literacy instruments, the Medical Term Recognition Test (METER) and Newest Vital Sign (NVS), filled out by people with DM2 coming to consultation in primary health centers in three main regions of Portugal.  Results: In this sample (n=141), 50.6% were male, 41 to 88 years old, and 56% earned more than the minimum wage. Using the METER tool, it was found that 57.4% of the diabetic patients had functional literacy. Adequate literacy was found in 24.1% with the NVS tool. Also with the NVS tool it was found that 36.2% of the sample subjects had s high probability of limited literacy. Utilizing the METER tool, a statistically significant decrease in health literacy was observed in individuals with diabetic complications (p=0.001). There was no significant relation between the presence of diabetic complications and present blood pressure values, low-density lipoprotein, and socioeconomic index. CONCLUSION: In this study, we found a significant relation between lower health literacy and the presence of diagnosed DM2 complications (p=0.001).

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Malignant melanoma (MM), which is amongst the rarest skin cancers, still remains one of the deadliest and most likely to spread, and, in human immunodeficiency virus (HIV)-infected patients, generally has a more aggressive behaviour. Although gastrointestinal (GI) tract metastases are frequent, secondary symptomatic colonic disease is rare. We present the case of a 76-year-old HIV-infected patient, with a 15-month history of GI and constitutional symptoms and a previous diagnosis of malignant melanoma. Diagnostic workup revealed metastatic involvement of the cecum. This case highlights the need to bear in mind the metastatic involvement of the GI tract by MM, and MM itself, especially in HIV-infected patients.

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Richter syndrome represents the transformation of chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma into an aggressive large B-cell lymphoma. Skin involvement is exceptionally rare, with <20 cases reported and its presence as the first presentation of CLL/small lymphocytic lymphoma, as an isolated skin lesion has never been described. Primary cutaneous CD4-positive small/medium T-cell lymphoma (CD4 PCSM-TCL) characteristically presents with a dense dermal infiltrate consisting primarily of small-/medium-sized pleomorphic T-cells and less than 30% large pleomorphic cells. A polymorphous reactive infiltrate composed of B-cells, histiocytes, plasma cells, and eosinophils is also found in differing proportions. Because of these morphological characteristics, the differential diagnosis includes not only other forms of cutaneous T-cell lymphomas but also B-cell lymphomas and reactive lymphoid infiltrates. We report a case of a cutaneous Richter syndrome as the initial manifestation of CLL, mimicking CD4 PCSM-TCL, in a 65-year-old apparently healthy asymptomatic man who presented with a solitary nodule in his arm. The objective of this study is to draw attention to this potential pitfall and describe this rare presentation.

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Abstract: BACKGROUND: Most available studies on the efficacy of topical photodynamic therapy focus on short-to medium-term results. Long-term data are scarce. OBJECTIVE: To evaluate the long-term efficacy of photodynamic therapy with topical methylaminolevulinate to treat Bowen's disease and basal cell carcinoma in the clinical practice setting of a dermato-oncology department. METHODS: The study included patients diagnosed with Bowen's disease or basal cell carcinoma, and who received photodynamic therapy from 2004 to 2008. Treatment protocol and clinical follow-up were standardized. The primary endpoint was clinically observed recurrence in a previous photodynamic therapy-treated area. Descriptive and survival analyses were performed. RESULTS: A total of 31 Bowen's disease lesions and 44 superficial basal cell carcinoma were treated, with a median follow-up of 43.5 months. Recurrence was observed in 14 Bowen's disease lesions (53.8%) and in 11 superficial basal cell carcinoma (33.3%). Significantly higher estimates for recurrence rates were found in patients with Bowen's disease (p=0.0036) or those aged under 58 years (p=0.039). The risk of recurrence was higher in patients with Bowen's disease than in those with superficial basal cell carcinoma and younger patients. CONCLUSIONS: Recurrence should be considered when choosing to treat non-melanoma skin cancer with photodynamic therapy. Younger age and Bowen's disease were independent predictors for long-term recurrence, suggesting the need to establish an extended period of follow-up for this subset of patients.

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BACKGROUND: Most available studies on the efficacy of topical photodynamic therapy focus on short-to medium-term results. Long-term data are scarce. OBJECTIVE: To evaluate the long-term efficacy of photodynamic therapy with topical methylaminolevulinate to treat Bowen's disease and basal cell carcinoma in the clinical practice setting of a dermato-oncology department. METHODS: The study included patients diagnosed with Bowen's disease or basal cell carcinoma, and who received photodynamic therapy from 2004 to 2008. Treatment protocol and clinical follow-up were standardized. The primary endpoint was clinically observed recurrence in a previous photodynamic therapy-treated area. Descriptive and survival analyses were performed. RESULTS: A total of 31 Bowen's disease lesions and 44 superficial basal cell carcinoma were treated, with a median follow-up of 43.5 months. Recurrence was observed in 14 Bowen's disease lesions (53.8%) and in 11 superficial basal cell carcinoma (33.3%). Significantly higher estimates for recurrence rates were found in patients with Bowen's disease (p=0.0036) or those aged under 58 years (p=0.039). The risk of recurrence was higher in patients with Bowen's disease than in those with superficial basal cell carcinoma and younger patients. CONCLUSIONS: Recurrence should be considered when choosing to treat non-melanoma skin cancer with photodynamic therapy. Younger age and Bowen's disease were independent predictors for long-term recurrence, suggesting the need to establish an extended period of follow-up for this subset of patients.

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Rhinophyma is a progressive and disfiguring disorder of the nose characterized by hyperplasia of the sebaceous glands and dermal tissue, representing the end-stage of rosacea. Four male patients affected by a moderate-severe rhinophyma were treated using a combined therapy with carbon dioxide laser and bipolar electrocoagulation. All patients achieved marked cosmetic results with minimal scarring. Various surgical approaches have been described to treat this condition but as yet there is no agreement in the literature on the ideal treatment. This combined method provides a bloodless operative field which allows the sculpting of the hypertrophic areas, leading to a good cosmetic outcome and a pain-free postoperative recovery.

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Neutrophilic dermatosis of the dorsal hands (NDDH) is a rare and recently described disorder regarded as a subset of neutrophilic dermatosis, similar to superficial pyoderma gangrenosum and pustular vasculitis. Many currently consider it to be a localized variant of Sweet Syndrome. We describe the case of a 63-year-old male patient with NDDH associated with hypopharyngeal carcinoma. A biopsy demonstrated changes consistent with those seen in Sweet Syndrome, with leukocytoclasia but no signs of true vasculitis. Treatment with oral prednisolone resulted in complete resolution of the lesions in 1 month, with no recurrences. The clinical presentation, the histological features, and the excellent response to oral corticosteroid therapy allowed us to classify this case as NDDH. We emphasize the rare association of this entity with a solid tumor.

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BACKGROUND: The H1-antihistamine cetirizine, a piperazine derivative widely used in daily practice, is rarely the cause of cutaneous drug reaction. Nevertheless, four cases of fixed drug eruption (FDE) as a result of this drug have been described recently. We present the case of a 45-year-old woman with a multilocalized FDE following oral intake of cetirizine for allergic rhinitis. METHODS: Patch testing with hydroxyzine 1% and 10% in petrolatum (Chemotechnique), and with powdered Zyrtec (cetirizine) and Xyzal (levocetirizine) pills, prepared at 20% in water and at 20% in petrolatum, was performed in both residual lesions and healthy skin. RESULTS: Positive results (++) to these drugs (24 h occlusion and readings at days 2 and 3) were obtained in residual lesions only. These results allowed us to confirm the drug responsible for this FDE and to study cross-reactions between antihistamines of the same chemical family. CONCLUSIONS: To the best of our knowledge, this is the first report of FDE to cetirizine with positive patch testing to hydroxyzine, cetirizine, and levocetirizine. This case highlights the importance of patch testing in the study of cutaneous drug reactions, namely FDE.

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