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Rare skin

T A Cranga, M A Simpson, P Featherstone
Acute generalised exanthematous pustulosis is a rare drug-induced dermatosis with an incidence of 1-5 cases per million cases per year, characterised by the appearance of hundreds of sterile pustules over erythematous and oedematous skin. Fever and neutrophilia are usually present. It has a rapid course and usually resolves following discontinuation of the precipitating drug or as a result of topical corticosteroid treatment. A patient with AGEP, who presented with generalized pustulosis lesions after the use of Flucloxacillin for cellulitis is described, along with the management and differential diagnosis of this condition...
2016: Acute Medicine
Sergio Pina-Oviedo, Carlos A Torres-Cabala, Roberto N Miranda, Michael T Tetzlaff, Selina Singh, Ronald P Rapini, Victor G Prieto, Phyu P Aung
Leukemia cutis develops in <4% of all acute leukemias. Concurrent acute myeloid leukemia (AML) and Langerhans cell histiocytosis (LCH) is rare, with most cases involving lymph nodes or spleen, and no cutaneous involvement. We report the case of a 59-year-old man who presented with fever, malaise, and fatigue. The CBC showed leukocytosis (30.4 × 10/L, 9% blasts), anemia, and thrombocytopenia. Bone marrow biopsy was diagnosed with AML, not otherwise specified, with mutations of FLT3 and IDH2 (R140Q). The patient developed skin rash on the right flank with the clinical differential diagnosis of herpes simplex virus or varicella-zoster virus infection/reactivation versus leukemia cutis...
October 18, 2016: American Journal of Dermatopathology
Irena E Belousova, Alexey V Samtsov, Dmitry V Kazakov
Unilesional (solitary) mycosis fungoides (MF) is a rare variant characterized clinically by the presence of a single contiguous area of skin involvement covering less than 5% of the body surface and histopathologically by features identical to those seen in classical MF. Angiocentricity (angiodestruction) is mostly a feature of primary or secondary cutaneous lymphomas with an aggressive course and poor outcome, with only very few reports of MF with angiocentric pattern. The authors report an unusual case of solitary patch-stage MF with hemorrhagic features, characterized histologically by epidermotropic and angiocentric (angiodestructive) infiltrate and a reactive B-cell component appearing as lymphoid follicles...
October 18, 2016: American Journal of Dermatopathology
Giang Huong Nguyen, James Y Wang, Kenneth B Hymes, Cynthia M Magro
Adult T-cell leukemia/lymphoma (ATLL) is a rare and often aggressive lymphoid malignancy known to be associated with human T-cell lymphotropic virus type 1. There are 2 broad categories: acute and chronic. In the acute category, there is a leukemic and a lymphomatous variant, whereas in the designated "chronic" form, there is mild peripheral blood lymphocytosis. The intermediate "smoldering" category is without peripheral blood lymphocytosis with only discernible skin involvement. We present a 68-year-old human T-cell lymphotropic virus type 1 seropositive female with a mild peripheral blood atypical lymphocytosis who had indurated nodules on her hands of 2 years duration and a new scaly ichthyosiform eruption on her lower extremities...
October 12, 2016: American Journal of Dermatopathology
A Pandey, Keshri S Yadav, Gaurav Singh, M Chaturvedi
Kyrle's disease is a rare skin disorder which is characterized by hyperkeratotic papules and nodules with a central keratotic plug mostly located in lower limbs. Exact etiology of Kyrle's disease is unknown, but its association has been reported sparsely with renal disorders, uremic patients on dialysis, diabetes mellitus, liver disease and paraneoplastic syndromes, tuberculosis and some fungal diseases. We report Kyrle's disease in a middle aged female suffering from diabetes mellitus with diabetic nephropathy on hemodialysis...
July 2016: Journal of the Association of Physicians of India
Tony Ht Sung, Annie Cw Tam, Jennifer Ls Khoo
Dermatofibrosarcoma Protuberans (DFSP) is a rare malignant soft-tissue neoplasm which is often misdiagnosed due to its indolent clinical course and non-specific radiological appearances. An observation case series was conducted with retrospective review of clinical and radiological data of DFSP patients presenting to a major tertiary hospital in Hong Kong for radiological assessment between November 2006 and February 2016. Seven patients with confirmed histological diagnosis of DFSP were included. Tumour sizes at presentation ranged from 1 to 5 cm, most commonly (n = 6) occurred over chest wall and abdominal wall...
October 18, 2016: Journal of Medical Imaging and Radiation Oncology
Nishitha Thumallapally, Uroosa Ibrahim, Mayurathan Kesavan, Qing Chang, Lynne Opitz, Meekoo Dhar, Sherif Andrawes
Granular cell tumors (GCTs) are soft tissue neoplasms that originate from Schwann cells. They occur predominantly in the oral cavity, skin, and breast tissues. Gastrointestinal GCTs are very rare, accounting for only eight percent of all GCTs, most of which are located in the esophagus. Endoscopic ultrasound has been a breakthrough in diagnosing GCTs because it provides precise information on the depth of tumor invasion, thus narrowing the differential diagnosis of subepithelial lesions in the esophagus. However, the definitive diagnosis requires histological confirmation of the lesion...
September 14, 2016: Curēus
Mridula Krishnan, Joseph Nahas
We present an unusual case of a young 26-year-old male who was diagnosed with Henoch-Schonlein Purpura (HSP). Initial presentation was primarily mild gastrointestinal symptoms, which progressed to a life threatening intussusception and subsequently resolved with prompt glucocorticoid use rather than typical surgical intervention. Of importance, the patient's initial gastrointestinal symptoms without associated skin manifestations made the diagnosis difficult. In conclusion, it is important to recognize uncommon presentations of HSP as it may lead to life threatening complications and surgical intervention may be avoided with prompt treatment...
2016: Case Reports in Rheumatology
Karla Schmitt, Fabian Beier, Jens Panse, Tim H Brümmendorf
Telomere syndromes (syn. Telomeropathies) are inherited disorders hallmarked by accelerated telomere shortening based on a molecular defect within the telomerase/telomere complex. The rare, but well-defined model disorder Dyskeratosis congenita (DKC) characterized by typical skin manifestations and bone marrow failure represents the classical manifestation of telomere syndromes in childhood and adolescence. However, cryptic variants of DKC, clinically manifest through appearance of atypical bone marrow failure, lung fibrosis or liver cirrhosis - especially in adults up to the fifth decade of age - are frequently underdiagnosed...
October 2016: Deutsche Medizinische Wochenschrift
Fatma Nur Korkmaz, Gulsen Ozen, Ali Uğur Ünal, Pınar Kahraman Koytak, Nese Tuncer, Haner Direskeneli
Abstract/ Resumo Behcet's disease (BD) is a multisystem inflammatory disorder characterized by recurrent oral and genital ulcers, skin lesions and uveitis. The nervous system involvement of BD, neuro-Behcet's disease (NBD), is one of the important causes of mortality of the disease. Herein, we present a 29-year-old male with parenchymal NBD who has progressed rapidly and was managed with an uncommon aggressive immunosuppresive combination therapy. The patient first presented six years ago with vertigo and difficulty in talking and walking...
September 28, 2016: Acta Reumatológica Portuguesa
Ting-Hua Yang, Chung-Hong Hu, Hsiou-Hsin Tsai
INTRODUCTION: Pyoderma gangrenosum (PG) is a neutrophilic dermatosis that may be associated with systemic diseases. The association of PG with lymphoid malignancies has rarely been reported. Extranodal natural killer/T-cell lymphoma (ENKTL) is a rare but aggressive entity with a poor prognosis. Here, we report the case of a patient who had idiopathic PG refractory to systemic steroids and subsequently developed ENKTL. CASE REPORT: A 70-year-old man presented with a 2-month history of intermittent fever and multifocal painful papules, plaques, and ulcerations on his extremities...
October 2016: Medicine (Baltimore)
Tara F Carr
OBJECTIVE: The aim of this paper was to review the classification of adverse drug reactions, highlight the known pathophysiology of immediate hypersensitivity reactions, and discuss the utility of diagnostic testing for immunologically mediated immediate reactions to gadolinium-based contrast agents (GBCAs). METHODS: Current literature on immediate reactions to GBCA was reviewed and summarized. RESULTS: Adverse drug reactions to GBCA are rare, and can be attributed to physiologic, immunologic, and nonimmunologic processes...
June 29, 2016: Topics in Magnetic Resonance Imaging: TMRI
Ankit Mangla, Nikki Agarwal, Chou Carmel, Thomas Lad
Erlotinib is one of the most widely used tyrosine kinase inhibitor targeting human epidermal growth factor receptor. Since its introduction, it has revolutionized the treatment of advanced non-small cell lung cancer. Skin rashes and diarrhea are the most often reported side effects of erlotinib however it is also associated with interstitial pneumonitis or interstitial lung disease, which often turns out to be fatal complication of using this medicine. Though reported scarcely in the western world, the association of interstitial lung disease with epidermal growth factor receptor has attracted a lot of attention in the recent times...
September 5, 2016: Rare Tumors
Mohamed Alsharedi, Nadim Bou Zgheib, Yousef Khelfa, Ali Raufi, Nabiha Elmsherghi, Yehuda Lebowicz
Malignant melanoma, as the name implies, is a malignant tumor of melanocytes, found in the skin, eyes, meningeal lining and the mucosal epithelium of the aero-digestive and genitourinary tracts. Malignant melanoma is typically skin malignancy, which rarely presents at extracutaneous site. Here we present a rare case of primary retroperitoneal melanoma and review the findings in comparison with other cases described in literature.
September 5, 2016: Rare Tumors
Min Jung Kim, Min Seok Hur, Byung Gon Choi, Soo Young Kim, Yang Won Lee, Yong Beom Choe, Kyu Joong Ahn
Dermatofibrosarcoma protuberans (DFSP) is a rare disease of dermal fibroblastic origin that accounts for less than 5% of all soft tissue sarcomas in adults. DFSP grows slowly and is an asymptomatic lesion at the initial diagnosis. Herein, we report a case of multiple pedunculated nodules as a variant of DFSP. A 47-year-old man presented with a 7-month history of multiple well-circumscribed, firm, pedunculated nodules on the inguinal area. Histopathologic examination results showed densely packed uniform spindle cells with a storiform and cartwheel pattern, and positivity for CD34...
October 2016: Annals of Dermatology
Yuzo Nagai, Sinsuke Kazama, Daisuke Yamada, Takuya Miyagawa, Koji Murono, Koji Yasuda, Takeshi Nishikawa, Toshiaki Tanaka, Tomomichi Kiyomatsu, Keisuke Hata, Kazushige Kawai, Yuri Masui, Hiroaki Nozawa, Hironori Yamaguchi, Soichiro Ishihara, Takafumi Kadono, Toshiaki Watanabe
Treatment of perianal and vulvar extramammary Paget disease (EMPD), rare intraepithelial malignancies, is often challenging because of its potential to spread into the anal canal. However, there is still no consensus regarding the optimal resection margin within the anal canal. Between 2004 and 2014, six patients (three with perianal EMPD and three with vulvar EMPD) in which the spread of Paget cells into the anal canal was highly suspected were referred to our department. To evaluate the disease extent within the anal canal, preoperative mapping biopsy of the anal canal was performed in five out of six patients...
October 2016: Annals of Dermatology
Min Soo Jang, Dong Young Kang, Jong Bin Park, Joon Hee Kim, Kwi Ae Park, Hark Rim, Kee Suck Suh
BACKGROUND: Pityriasis lichenoides (PL)-like skin lesions rarely appear as a specific manifestation of mycosis fungoides (MF). OBJECTIVE: We investigated the clinicopathological features, immunophenotypes, and treatments of PL-like MF. METHODS: This study included 15 patients with PL-like lesions selected from a population of 316 patients diagnosed with MF at one institution. RESULTS: The patients were between 4 and 59 years of age...
October 2016: Annals of Dermatology
Arie S Parnham, Maarten Albersen, Varun Sahdev, Michelle Christodoulidou, Raj Nigam, Peter Malone, Alex Freeman, Asif Muneer
BACKGROUND: Penile cancer is a rare malignancy that is confined to the glans in up to four out of five cases. Although descriptions of glansectomy exist, there are no contemporary video explanations or large published single centre series. OBJECTIVE: To show the efficacy and safety of glansectomy and split-thickness skin graft (STSG) reconstruction. DESIGN, SETTING, AND PARTICIPANTS: Data were collected retrospectively for patients identified from surgical theatre diaries between February 2005 and January 2016...
October 13, 2016: European Urology
Benjamin Farahnik, Collin M Blattner, Michael B Mortazie, Benjamin M Perry, William Lear, Dirk M Elston
Hailey-Hailey disease or familial benign chronic pemphigus is a rare blistering dermatosis that is characterized by recurrent erythematous plaques with a predilection for the skin folds. For extensive Hailey-Hailey disease that is recalcitrant to conventional therapy, laser ablation, photodynamic therapy, electron beam radiotherapy, botulinum toxin type A, dermabrasion, glycopyrrolate, and afamelanotide have been reported as useful treatments, but comparative trials are lacking. This review discusses the various treatment modalities for Hailey-Hailey disease and a summary of the evidence for the most recommended treatments...
October 13, 2016: Journal of the American Academy of Dermatology
Pernille Henriksen, Malene Landbo Børresen, Kathrin Dahl
Food protein-induced enterocolitis syndrome (FPIES) is a rare non-IgE mediated condition. Symptoms of acute FPIES include vomiting, diarrhoea and dehydration. Symptoms are often misread as acute abdomen or sepsis. The condition can be fatal. There are no biomarkers for FPIES, and skin prick test for cow milk is often negative. Treatment is hydrolysed formula milk. This case story describes a one-month-old boy with diarrhoea and an output of up till 2.3 l per day. With this case story we wish to increase the knowledge of FPIES and emphasize the importance of having the diagnosis in mind when confronted with the very ill, dehydrated infant...
October 10, 2016: Ugeskrift for Laeger
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