scholarly journals Traversing the Spectrum of Non-Langerhans Cell Histiocytosis: A Case of Rosai-Dorfman Disease with Features of Necrobiotic Xanthogranuloma

2021 ◽  
Vol 55 (5) ◽  
Author(s):  
Blythe N. Ke ◽  
Erika Belinda T. Chen ◽  
Kevin Jer V. David ◽  
Sarah Faye V. Obbus ◽  
Val Constantine S. Cua ◽  
...  
Keyword(s):  
Clinical Manifestations ◽  
Giant Cells ◽  
Skin Lesions ◽  
Neck Mass ◽  
Langerhans Cell ◽  
Cervical Lymph Nodes ◽  
Negative Results ◽  
Skin Nodule ◽  
Rosai Dorfman Disease ◽  
The Face

Introduction. Non-Langerhans cell histiocytoses (non-LCH) are a group of rare diseases with varied clinical manifestations and overlapping features seen among the subtypes. Here, we present a case of Rosai-Dorfman disease with features of necrobiotic xanthogranuloma. Case. A 45-year-old female presented with a 10-year history of an enlarging neck mass with normal overlying skin accompanied by dysphagia and multiple asymptomatic pink to yellowish-brown papules, nodules, and plaques on the face, trunk and extremities. Biopsies of a skin nodule and plaque revealed granulomatous dermal infiltrates (lymphocytes, foamy histiocytes, and Touton giant cells), emperipolesis and areas of necrosis. CD1A and Fite-Faraco staining showed negative results while CD68 and S100 positively stained the tissues of interest. Histopathology of the neck mass paralleled these findings in addition to being negative for lymphoid markers. Patient had monoclonal gammopathy and thyromegaly with enlarged cervical lymph nodes on further tests and imaging. Intralesional and systemic steroids were given which led to flattening of skin lesions and improvement in dysphagia, respectively. Conclusion. Diagnosis and classification of a particular type of non-LCH may be difficult due to similarities across its subtypes. Hence, it is our belief that these diseases may occur on a spectrum. Treatment involves a multidisciplinary approach for the best possible care.

NONTUBERCULOUS ACID-FAST BACILLI IN CERVICAL ADENITIS

PEDIATRICS ◽  
1957 ◽  
Vol 20 (4) ◽  
pp. 688-697
Author(s):  
Haddow M. Keith ◽  
Lyle A. Weed ◽  
Gerald M. Needham
Keyword(s):  
Giant Cells ◽  
Skin Lesions ◽  
Mycobacterium Ulcerans ◽  
Cervical Region ◽  
Tubercle Bacillus ◽  
Cervical Lymph Nodes ◽  
Caseous Necrosis ◽  
Acid Fast Bacilli ◽  
Cervical Adenitis ◽  
Bona Fide

THE TUBERCLE bacillus is the most common cause of lymphadenitis with caseous necrosis. Such a condition in the cervical region, while less frequent than in previous decades, still occurs occasionally. While other agents, for example, Coccidioides, Histoplasma, Brucella, Pasteurella and Miyagawanella, are known to produce a similar histopathologic reaction, they are not commonly associated with lesions in the cervical lymph nodes, especially in children. Therefore the finding of acid-fast bacilli in such lesions has generally been considered bona fide evidence of tuberculosis, although previous reports from this clinic indicate that such is not necessarily true. It is recognized that there are other acid-fast bacilli, such as the lepra and smegma bacilli, which may or may not cause disease. In 1944 Gellerstedt reported seven cases of tuberculoid skin lesions due to atypical acid-fast bacilli. He considered these to be due to exogenous infection with acid-fast bacilli differing from the organisms found in tuberculosis, and he considered them as possibly saprophytic organisms. In 1948 MacCallum and co-workers reported six cases of skin lesions due to acid-fast organisms that were not Mycobacterium tuberculosis. The lesions did not contain tubercle follicles, giant cells or caseous material. The name "Mycobacterium ulcerans" was applied to these organisms by Australian workers. In 1954 Linnell and Nordén described skin lesions that occurred in 80 boys and girls who used a certain swimming pool in a Swedish town. The etiologic organisms were acid fast, differed from those described by MacCallum and associates and were not lepra bacilli. Guinea pigs injected with these organisms presented no evidence of lesions after 7 weeks.

scholarly journals Rosai-Dorfman Disease: Rare Pulmonary Involvement Mimicking Pulmonary Langerhans Cell Histiocytosis and Review of the Literature

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Rashid AL Umairi ◽  
Danielle Blunt ◽  
Wedad Hana ◽  
Matthew Cheung ◽  
Anastasia Oikonomou
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Corticosteroid Treatment ◽  
Pulmonary Involvement ◽  
Skin Lesions ◽  
Langerhans Cell ◽  
Sinus Histiocytosis ◽  
Review Of The Literature ◽  
Massive Lymphadenopathy ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Rosai Dorfman Disease

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare nonmalignant lymphohistiocytic proliferative disorder. We report a patient with RDD who presented with multiple skin lesions, pulmonary involvement, and CT manifestations mimicking Langerhans cell histiocytosis, which improved after initiation of corticosteroid treatment.

scholarly journals Identification of lamb flocks susceptible and resistant against Brachiaria poisoning

2016 ◽  
Vol 36 (5) ◽  
pp. 383-388 ◽  
Author(s):  
Rayane C. Pupin ◽  
Gleice K.A. Melo ◽  
Rubiane F. Heckler ◽  
Tatiane C. Faccin ◽  
Camila C.B.F. Ítavo ◽  
...  
Keyword(s):  
Genetic Resistance ◽  
Clinical Signs ◽  
Giant Cells ◽  
Skin Lesions ◽  
Bile Duct Proliferation ◽  
Subcutaneous Edema ◽  
Lymphoplasmacytic Infiltrate ◽  
The Face ◽  
Resistant Group ◽  
Foamy Macrophages

Abstract: This study was designed to assess the influence of genetic resistance against brachiaria poisoning in sheep. Two groups of sheep, one identified as susceptible (formed by two ewes and one ram) and the other as resistant against brachiaria poisoning (formed by three ewes and one ram) were selected. Sheep considered susceptible were those that presented clinical signs of brachiaria poisoning at any time of their life; resistant sheep were those that even raised on Brachiaria spp. pastures, did not developed any sign of the poisoning during their life. The offspring of the two flocks (15 lambs from the sensitive flock and 9 lambs from the resistant flock) were placed into brachiaria pasture (initially Brachiaria decumbens and B. brizantha,and only B. decumbens after weaning) and followed up during two years (2013-2014). The determination of protodioscin levels in B. decumbens pasture was performed only in 2014 and revealed significant amounts of the toxic principle. Eleven lambs of the susceptible group were affected to some degree of brachiaria poisoning and six died; no lamb of the resistant group was affected. Clinical signs consisted of varying degrees of subcutaneous edema of the face and, erythema and loss of hair of the ears, crusts on the skin of ears, around the eyes and on planum nasale, scar deformation of the ears, and bilateral ocular discharge; affected lambs also sought for shadowy shelters and they were poor doers. Several sheep recovered from the condition and then relapsed. Necropsy findings in six lambs included pale mucous membranes, emaciation, dermatitis, scar deformation of the ears, large yellow livers with marked lobular pattern, and moderate infestation by Haemonchus contortus. Histologically the liver lesions were similar in all necropsied lambs but with varying degrees of severity; they were consistent with brachiaria poisoning and included architectural disruption of hepatocellular trabecula, clusters of foamy macrophages occasionally forming multinucleated giant cells, swollen and vacuolated hepatocytes, crystals or negative images of crystals in the biliary system, bilestasis, bile duct proliferation and lymphoplasmacytic infiltrate in portal triads. The skin lesions were those of photodermatitis and included epidermal necrosis, hyperkeratosis and dermal neutrophilic infiltrate. The results of this study allow to conclude that there is a genetic related resistance to brachiaria poisoning in sheep since the progeny of resistant sheep did not manifest the poisoning. The use of resistant flocks in brachiaria pastures is suggested as a valuable option for the prevention of brachiaria poisoning in sheep.

scholarly journals Terapi necrobiotic xanthogranuloma dengan siklofosfamid-metilprednisolon

2016 ◽  
Vol 46 (2) ◽  
pp. 202
Author(s):  
Bayu Lesmono ◽  
Yussy Afriani Dewi ◽  
Sinta Sari Ratunanda ◽  
Nur Akbar Aroeman
Keyword(s):  
Monoclonal Gammopathy ◽  
Progressive Disease ◽  
Subcutaneous Tissue ◽  
Giant Cells ◽  
Skin Lesions ◽  
Exclusion Criteria ◽  
Burning Pain ◽  
Patient Number ◽  
The Face ◽  
Ulcerative Lesions

Latar belakang: Necrobiotic xanthogranuloma (NXG) merupakan suatu penyakit yang langka, kronis, dan progresif. NXG ditandai dengan lesi kulit ulseratif pada daerah indurasi, dan berwarna kuning, atau berupa nodul yang mengenai sel histiosit non Langerhans. Daerah predileksi tersering ialah pada wajah, orbita, dan ekstremitas. Etiologi belum diketahui, tetapi sering dikaitkan dengan monoclonal gammopathy. Gambaran histopatologi NXG yaitu ditemukan makrofag dan terdapat sel busa pada dermis, jaringan subkutan, nekrobiosis luas, sel datia Touton, dan folikel limfoid. Penderita memiliki lesi yang bersifat asimtomatik, parestesi, rasa terbakar, dan terkadang timbul rasa nyeri. Tata laksana NXG sampai saat ini masih sangat bervariasi. Tujuan: Memaparkan hasil penatalaksanaan dua pasien NXG. Laporan kasus: Dilaporkan pasien pertama, laki-laki 44 tahun dengan lesi pada kedua pipi dan dahi sejak 5 bulan sebelumnya. Terapi diberikan metilprednisolon dengan dosis 0,8 mg/kgBB tappering off selama 1 bulan dengan hasil perbaikan. Pasien kedua, wanita 29 tahun dengan lesi pada kedua pipi dan telinga sejak 5 bulan sebelumnya. Terapi diberikan siklofosfamid 750 mg/m2 per 3 minggu dengan hasil membaik. Metode: Pencarian dilakukan melalui PubMed dan Dermatol. Setelah menyaring judul dan abstrak yang sesuai dengan kriteria inklusi dan eksklusi, didapatkan lima artikel utuh. Kemudian, tiga artikel terpilih untuk ditelaah secara konsensus, meliputi kesahihan (validity), kepentingan (importancy), dan aplikabilitas (applicability) pada pasien necrobiotic xanthogranuloma. Hasil: Telaah artikel-artikel tersebut menunjukkan bahwa semua artikel memiliki karakteristik yang serupa dalam hal kesahihan (validity), hasil atau kesimpulannya. Walau demikian, ada beberapa kekuatan dan kelemahan pada masing-masing artikel. Kesimpulan: Penatalaksanaan NXG masih diperlukan penelitian lebih lanjut dengan jumlah sampel yang banyak untuk mengetahui keefektifitasan penatalaksanaan NXG.Kata kunci: Siklofosfamid, metilprednisolon, necrobiotic xanthogranulomaABSTRACT Background: Necrobiotic Xanthogranuloma (NXG) is a rare, chronic, and progressive disease that provokes skin lesions, such as damage of the histiocytes of non-Langerhans cell, skin lesions (yellowish or noduled ulcerative lesions) in the induration skin. The most common predilection areas of this are on the face, orbital, and extremities. The etiology is still unknown, but sometimes associates with monoclonal gammopathy. NXG histopathologic contained macrophages and foam cells in the dermis, subcutaneous tissue, extensive necrobiosis, touton giant cells, and lymphoid follicles. Some patients who had lesions are asymptomatic, sometimes they will feel paresthesias, burning pain. Nowadays, this management is still vary widely. Objective: Sets forth the results of two patients NXG. Case: There were two patients. Patient number one, a 44-years-old man, with some lesions on both cheeks and forehead since 5 months ago. He was treated with methylprednisolon 0.8 mg/kg body weight, and tappered off for a month with improved results. Patient number two, a 29-years-old woman, with some lesions on both cheeks and ears since 5 months ago. She was treated with Cyclosphosphamide 750 mg/m2 with improved results within three weeks. Method: Searching for literature was conducted in PubMed and Dermatol. After screening titles and abstracts with inclusion and exclusion criteria, five articles were available as full texts. Three articles were selected to be reviewed for their validity, importancy and applicability in patient with necrobiotic xanthogranuloma. Results: The review showed that all those articles had a similar characteristic in validity, result, and conclusion. Nevertheless, each articles had its own strong points and weaknesses. Conclusion: NXG treatment still required further research by the number of samples that much to find out the efficiency management NXG.Keywords: Cyclophosphamide, methylprednisolon, necrobiotic xanthogranuloma

scholarly journals Langerhans cell histiocytosis: Skin diseases and visceral lesions

2017 ◽  
Vol 89 (4) ◽  
pp. 115-120
Author(s):  
V D Elkin ◽  
T G Sedova ◽  
E A Kopytova ◽  
E V Plotnikova
Keyword(s):  
Langerhans Cells ◽  
Langerhans Cell Histiocytosis ◽  
Skin Diseases ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Langerhans Cell ◽  
Literature Analysis ◽  
Trigger Factors ◽  
Statistical Variations ◽  
The World

Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder that belongs to class I histiocytoses and is characterized by infiltration of one or many organs by Langerhans cells to form granulomas. The literature analysis could identify a lot of etiological, pathogenetic, and trigger factors and mechanisms for LCH development, which determine the diversity of the clinical picture and course of the disease. The clinical manifestations of LCH are very variable and depend on the severity of lesions and the age of patients. In addition to skin lesions, there is involvement of one or more visceral organs. Difficulties in diagnosing the disease lead to statistical variations of LCH in different countries of the world and require more attention by physicians of all specialties.

scholarly journals Tinea incognito: Clinical perspectives of a new imitator

2020 ◽  
Vol 12 (1) ◽  
Author(s):  
Samer Dhaher
Keyword(s):  
Lupus Erythematosus ◽  
Skin Diseases ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Systemic Steroid ◽  
Discoid Lupus Erythematosus ◽  
Wide Range ◽  
The Face ◽  
Tinea Incognito ◽  
A Prospective Study

Tinea incognito is a variant of dermatophyte infection of the skin modified by an erroneously applied topical or systemic steroid. Aim of the study: to describe the various clinic-epidemiological aspects of tinea incognito found among our patients. A prospective study was carried out in the Department of Dermatology, Basra Teaching Hospital, Basra, Iraq. The clinical diagnosis was confirmed by mycological tests. Clinical data were described in more detail and the lesions were classified according to the site, shape, and extent. Ninety cases of tinea incognito were seen, median age was 34years. The initial inaccurate diagnoses were eczema in 60 patients (67%), intertrigo in 16 (18 %) and psoriasis in 14 (15%) patients. The type of topical drugs applied was topical corticosteroid (potent and fluorinated) in most cases (54.4%), fixed drug combination creams in 36.6% and a topical calcineurin inhibitor in 5.5%. Commonly presented as acute eczema-like, on hands and trunk, discoid lupus erythematosus-like lesions on the face and psoriasiform lesions on the scalp. Due to the wide range of clinical manifestations, tinea incognito imitates many skin diseases and should, therefore, be considered in any chronic, erythematous, scaly skin lesions not responding to topical treatment.

scholarly journals A Rare Case of Juvenile Xanthogranuloma in 8 Months Old Baby with Dyslipidemia

2021 ◽  
Vol 5 (4) ◽  
pp. 1212-1217
Author(s):  
Jessica Herlianez Saiful ◽  
Rina Gustia
Keyword(s):  
Lower Limb ◽  
Foam Cell ◽  
Laboratory Finding ◽  
Skin Lesions ◽  
Langerhans Cell ◽  
Juvenile Xanthogranuloma ◽  
Formula Milk ◽  
Systemic Involvement ◽  
The Face ◽  
Lipid Abnormalities

Background: Juvenile xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis disease of childhood. But due to rarity of non Langerhans cell hystiosis itself, the exact prevalence of juvenile xanthogranuloma remain unknown with only a few epidemiological journal ever published. Juvenile xanthogranuloma usually wihout lipid abnormality and systemic involvement. But association between JXG and lipid abnormalities is still not well understood. We describe a patient with multiple cutaneous JXG who also developed hyperlipidemia. Case: A case of a  8 months-old baby patient with juvenile xanthogranuloma is reported.  Patient parents noticed yellowish dots on child’s face since six months ago, and it was gradually increase in size and number, and spread to trunks, upper and lower limb since 2 months ago. Patients got formula milk since 7 months ago. Patients father has uncontrolled hypercholesterolemia, and grandparents  had controlled dyslipidemia. Dermatological state showed yellowish plaque and papule on the face, trunk, lower limb, and upper limb. Dermoscopy show yellowish papule with sun setting appearance and branched and linear vessel on orange yellow background. Laboratory finding showed  elevated lipid serum. Foam cell and Touton giant  cell is found on histopathology examination. Discussion: The presented case demonstrates that skin lesions in patients with diagnosed JXG may have a variable clinical presentation, ranging from single to diffuse skin lesions, also present from the birth to childhood. The diagnosis requires histopathological confirmation to avoid misdiagnosis of malignant disease. Association between JXG and lipid abnormalities remain unknown, with most of the patient show normal  lipid serum. Majority of patients presenting lesions limited to the skin requires only a strict dermatological observation.

Clinical Manifestations and Radiological Characteristics on Rosai-Dorfman Disease: A Retrospective Observational Study

2020 ◽  
Vol 5 (5) ◽  
Keyword(s):  
Blood Vessels ◽  
Abdominal Cavity ◽  
Clinical Manifestations ◽  
Ct Images ◽  
High Signal ◽  
Radiological Features ◽  
Imaging Characteristics ◽  
Rosai Dorfman Disease ◽  
Heterogeneous Enhancement ◽  
The Masses

Background and Objective: Rosai-Dorfman disease (RDD) are usually misdiagnosed because of rarity and nonspecific clinical and radiological features. The aim of our study is to explore the clinical and imaging characteristics of RDD to improve diagnostic accuracy. Methods: Clinical and imaging data in 10 patients with RDD were retrospectively analyzed. 7 patients were underwent CT scanning and 3 patients were underwent MR examination. Results: 8 (8/10) patients presented with painless enlarged lymph nodes (LNs) or mass. 3 cases were involved with LNs, 5 cases were involved with extra-nodal tissues, and the remaining 2 cases were involved with LNs and extra-nodal tissue simultaneously. In enhanced CT images, enlarged LNs displayed mild or moderate enhancement, and 2 cases showed heterogeneous ring-enhancement. MR features of 3 patients with extra-nodal RDD, 2 cases showed a mass located in the subcutaneous and anterior abdominal wall respectively, and 1 case showed an intracranial mass. Besides, all lesions showed high signal foci on DWI images, and were characterized by marked heterogeneous enhancement with blurred edge. The dural/fascia tail sign and dilated blood vessels could be seen around all the lesions on enhanced MRI. Radiological features of 2 cases with LN and extranodal tissue involved, one case presented with the swelling and thickening of pharyngeal lymphoid ring and nasopharynx, meanwhile with enlarged LNs in bilateral submandibular area, neck and abdominal cavity, and also companied with osteolytic lesion in right proximal humerus. All these LNs displayed mild and moderate enhancement on CT images. Another case showed enlarged LNs in bilateral neck accompanied with soft tissue mass in the sinuses. Conclusions: RDD occurred commonly in young and middle-aged men and presented with painless enlarged LNs or mass.RDD had a huge diversity of imaging findings, which varied with different location. The radiological features, such as small patches of high signal foci in the masses on DWI images, heterogeneous enhancement and blood vessels around the masses, are helpful in diagnosis of extranodal RDD.

Scabies: A Neglected Global Disease

2020 ◽  
Vol 16 (1) ◽  
pp. 33-42 ◽  
Author(s):  
Alexander K.C. Leung ◽  
Joseph M. Lam ◽  
Kin F. Leong
Keyword(s):  
English Language ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Sarcoptes Scabiei ◽  
Clinical Findings ◽  
Effective Control ◽  
Search Term ◽  
Skin Contact ◽  
Intense Pruritus ◽  
Meta Analyses

Background: Scabies is a skin disease caused by an obligate human parasite mite Sarcoptes scabiei var. hominis. Children under the age of two and elderly individuals are at the greatest risk. Knowledge of this condition is important for an early diagnosis to be made and treatment to be initiated. Objective: The review aimed to familiarize physicians with the clinical manifestations, diagnosis, evaluation, and management of scabies. Methods: A search was conducted using Pubmed with the built-in "Clinical Queries" tool. The search term "Scabies" was used. The categories of "epidemiology", "diagnosis", "therapy", "prevention" and "prognosis" had a limited scope for primary clinical studies. Meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews were included. Only papers published in the English language were included. A descriptive, narrative synthesis was provided of the retrieved articles. Results: Worldwide, scabies affects 200 to 300 million individuals annually. The average prevalence is estimated to be 5 to 10% in children of developing countries. Transmission usually occurs after close prolonged skin-to-skin contact. Classic scabies is characterized by an erythematous papular eruption, serpiginous burrows, and intense pruritus. Sites of predilection include the webs of the fingers, volar wrists, lateral aspects of fingers, extensor surfaces of elbows and knees, waist, navel, abdomen, buttocks, groins, and, genitals. A clinical diagnosis of classic scabies can be made on the basis of the history and clinical findings. Other clinical variants include crusted scabies, nodular scabies, and bullous scabies. Finding the mite, ova, or fecal pellets on microscopic examination of scrapings taken from skin lesions confirms the diagnosis of scabies infestation. For eradication of scabies mites, the drugs of choice are topical permethrin and oral ivermectin. Conclusion: Scabies is a highly contagious parasitic cutaneous disease that is stigmatising and debilitating. Increased awareness, accurate diagnosis, and prompt treatment are essential for the effective control of scabies and for the prevention of the spread of the disease.

scholarly journals Cervical lymph node enlargement as the initial manifestation of rectal cancer

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Tong-Hui Xie ◽  
Peng Su ◽  
Jian-Guo Hong ◽  
Hui Zhang
Keyword(s):  
Colorectal Cancer ◽  
Rectal Cancer ◽  
Lymph Node ◽  
Lymph Nodes ◽  
Cervical Lymph Node ◽  
Clinical Manifestations ◽  
Initial Symptom ◽  
Cervical Lymph Nodes ◽  
Supraclavicular Lymph Nodes ◽  
Lymph Node Enlargement

Abstract Background Colorectal cancer is a very common malignant tumor worldwide. The clinical manifestations of advanced colorectal cancer include the changes in bowel habits, hematochezia, diarrhea, local abdominal pain and other symptoms. However, the colorectal cancer with an initial symptom of cervical lymph node enlargement is extremely rare. In this article, we report a case of rectal cancer presenting with cervical lymph nodes enlargement as the initial symptom. Case presentation A 57-year-old woman was admitted to our hospital for cervical lymph node enlargement which was accidentally detected during physical examination. Computed tomography scan revealed multiple enlarged lymph nodes in the neck. Cervical ultrasound showed normal thyroid gland and multiple left supraclavicular lymph nodes enlargement. The patient underwent lymph nodes biopsy and pathologic results showed metastatic adenocarcinoma. The subsequent lower gastrointestinal endoscopy revealed a mucosal bulge lesion located at rectus and biopsy revealed adenocarcinoma. The patient underwent rectal cancer resection. She is alive with no evidence of recurrence or new tumors 2 years after surgery. Conclusions Cervical lymph node metastasis is a rare metastatic way in colorectal cancer. This is the first case of rectal cancer presenting with cervical lymph nodes metastases as the initial symptom. Surgical resection combined with postoperative chemotherapy improved long-term prognosis of the patient. This rare metastatic way of rectal cancer should be paid attention for clinicians.

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