Giant benign lymphangioendothelioma with positive expression of Wilms tumor 1: A case report.

Benign lymphangioendothelioma (BL, acquired progressive lymphangioma) is a rare, slow-growing lymphatic tumor, first described 40 years ago, with fewer than 50 published cases. Clinically, it presents as a skin-colored or erythematous patch. Definitive diagnosis requires histopathological examination. The immunohistochemical staining profile is still controversial regarding Wilms tumor 1 (WT1) expression, a marker of proliferative and neoplastic, rather than malformative nature. Here, we report a case of a 60-cm-long BL on the breast of an adult female. Biopsy revealed irregular vascular spaces dissecting the collagen bundles lined by swollen endothelial cells but without cellular atypia. Positivity for podoplanin (D2-40), CD31, and WT1 was observed, supporting the neoplastic nature of this lesion. Dermatologists and pathologists must be aware of this entity for early diagnosis and treatment.

Cutaneous Angiosarcoma in an Unusual Location and Without Predisposing Factors.

Cutaneous angiosarcoma is a rare, highly malignant tumour of vascular endothelial origin. It usually arises in the skin and superficial soft tissue, mostly on the head and neck. It presents as a variety of lesions, and so is considered a great mimicker, leading to a delay in diagnosis and evidencing the importance of biopsy with immunohistochemistry confirmation. There are few reports of extremity involvement in patients with pre-existing chronic lymphoedema, or exposure to radiation therapy. We report the case of an 82-year-old woman with lower limb extensive cutaneous involvement, distant metastatic disease, and poor therapy response. Its rare location without predisposing factors highlights the need to raise awareness about this disease. LEARNING POINTS: Extremity involvement of cutaneous angiosarcoma has been rarely described. The marked heterogeneity in presentation leads to a delay in diagnosis and poor prognosis, so the index of suspicion should be high.The cases reported in the literature describe a well-known relationship between cutaneous angiosarcoma and predisposing factors, but its absence should not exclude the diagnosis.This case highlights the importance of recognizing and biopsy suspected skin lesions for immunohistochemistry diagnostic confirmation.

An Exuberant Case of Necrobiotic Xanthogranuloma.

Necrobiotic xanthogranuloma (NXG) is a rare form of inflammatory granulomatous disease of the skin characterized by the presence of pruritic to painful lesions, generally located in the periorbital area, although trunk and proximal extremities may also be involved. We report a case of a 69-year-old male patient with extensive involvement of the trunk, upper and lower extremities, and an associated gamma monoclonal gammopathy. The skin biopsy was consistent with the diagnosis of NXG. Necrobiosis lipoidica and granuloma annulare were the major differential diagnosis in this case. We highlight the importance of searching for clinical and histological features that may differentiate these entities, which is crucial to select an adequate therapeutic and surveillance strategy.

Mycobacterium abscessus infection in a spa worker.

Mycobacterium abscessus (M. abscessus) is a non-tuberculous mycobacterium widely present in the natural environment and is now being increasingly identified as a cause of human disease. However, it can cause skin and soft tissue infection following trauma or surgery. We report a case of a skin ulcer associated with M. abscessus in a spa worker. Frequently, contamination with atypical mycobacteria occurs through tap water. However, in most outbreaks the infection source remains unknown. For an early diagnosis, it is important to perform biopsies and mycobacterium cultures. No specific treatment for skin infection with M. abscessus has been established; it depends on the immune status of the host and the extent of disease. Although it is rare, non-tuberculous mycobacterial infection should be part of the differential diagnosis of cutaneous ulcers, especially on trauma-prone areas.

Surgical Pearl on Reconstructing Surgical Defects of the Nasal Tip.

Repairing surgical defects of the nasal tip is challenging, mainly because of the lack of freely mobile skin available peripherally. The Peng flap is a one-stage cutaneous flap that circumvents this difficulty by recruiting skin from the nasal dorsum and sidewall regions. The design produces a tridimensional shape perfectly adapted to the configuration of the nasal tip and allows for an inconspicuous closure of the defect. We present three examples of full-thickness skin defects involving the nasal tip, reconstructed using a modified version of the Peng flap, and present the experience at our department with this surgical technique.

Reconstrução de defeitos cirúrgicos da ponta nasal em único tempo cirúrgico / Reconstructing surgical defects of the nasal tip in a single-stage procedure

A reconstrução de defeitos cirúrgicos envolvendo a subunidade da ponta nasal coloca desafios particulares já que essa região é rodeada de pele de difícil mobilização. O retalho de Peng é retalho cutâneo passível de ser executado num único tempo cirúrgico que ultrapassa essa dificuldade mobilizando pele do dorso e das vertentes laterais do nariz. O seu desenho permite alcançar forma tridimensional adaptada à configuração da ponta nasal e produz excelentes resultados estéticos. Descreve-se a realização de uma modificação do retalho de Peng em único tempo cirúrgico, em três pacientes submetidos a excisão de carcinomas basocelulares.

Repairing surgical defects of the nasal tip is challenging, mainly because of the lack of freely mobile skin available peripherally. The Peng flap is a one-stage cutaneous flap that circumvents this difficulty by recruiting skin from the nasal dorsum and sidewall regions. The design produces a tridimensional shape perfectly adapted to the conFiguretion of the nasal tip and allows for an inconspicuous closure of the defect. Herein, we describe a modified version of a Peng flap in a single-stage procedure, performed in three patients subjected to excison of basal cell carcinomas.

Cutaneous Richter Syndrome mimicking a lower limb cellulitis infection - a case report and review of the literature.

Richter syndrome (RS) is characterized by the development of a high-grade lymphoma in patients with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). Herein, we present the case of an 85-year-old woman with a 3-year history of stable asymptomatic CLL that developed a cutaneous RS. The patient presented with painless inflammation in the left leg and foot that was initially diagnosed as a cellulitis infection. She was treated accordingly with ceftriaxone and clindamycin. However, after completing the antibiotic regimen, not only did the inflammation persist, but also superimposed painless nodules gradually appeared on the left leg and foot over the course of four months. The histopathological examination of the nodules revealed a large B-cell cutaneous lymphoma. The patient underwent chemotherapy with CVP, followed by R-CHOP, resulting in a reduction of size of the nodules and remission of the inflammation. The patient died five months after the diagnosis owing to a bacterial pneumonia. We identified in previous reports a total of fifteen cases of cutaneous RS. Most cases presented with rapidly growing tumors or multiple erythematous nodules, similar to our case. This case of a cutaneous RS mimicking a cellulitis infection underlines the importance of a low threshold for performing biopsies of suspicious skin lesions in patients with CLL/SLL.

Erythroderma. A clinical and etiological study of 103 patients.

BACKGROUND: Erythroderma is an uncommon and severe dermatological manifestation of a variety of diseases. It is commonly challenging to find the underlying cause. OBJECTIVE: The aim of this study was to analyze the causes of the disease in patients with erythroderma. PATIENTS AND METHODS: Data including the clinical symptoms, laboratory examinations, histopathology and follow-up information were collected from patients with erythroderma admitted to our department between 2000 and 2010. RESULTS: One-hundred and three patients diagnosed with erythroderma were identified during this period (11.9% of all hospitalized patients; hospital incidence = 9.4 cases/year). The mean age of onset was 54.4 years (range: 17-89 years) with a male:female ratio of 1.5:1. The most frequent cause of erythroderma was exacerbation of preexisting dermatoses (65.0%), including psoriasis (44.7%) and eczema (16.5%). Drugs (18.4%) and cutaneous T-cell lymphomas (11.7%) induced most of the remaining cases. No cause could be identified in four cases (3.9%). Apart from erythema and scaling, that were present in all patients, clinical findings were dominated by pruritus (97.1%), followed by edema (56.3%), fever (54.4%), palmoplantar keratoderma (50.5%), nail changes (42.7%), liver or spleen enlargement (41.7%) and lymphadenopathy (40.8%). CONCLUSIONS: Although numerous clinical features and laboratory values were abnormal, most findings were non-specific. The skin biopsy yielded a positive clinical correlation in most cases. Our study had a high percentage of erythroderma secondary to preexisting skin disease and a relatively low percentage of idiopathic erythroderma.

Pigmented lesion of the nipple - a clinicopathological challenge.

Differential diagnoses of pigmented lesions of the nipple include melanocytic nevus, melanosis of the nipple, seborrheic keratosis, pigmented basal cell carcinoma, melanoma and Paget disease. The histologic exam with appropriate immunohistochemistry is a fundamental tool to achieve a correct diagnosis. We present a patient with a pigmented lesion of her right nipple revealing mammary Paget disease and elucidate diagnostic obstacles and prognostic importance of early breast cancer detection.