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

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.

2001 ◽  
Vol 5 (6) ◽  
pp. 486-489 ◽  
Author(s):  
Kelli W. Morgan ◽  
Jeffrey P. Callen

Background: Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare condition which may present at birth or during the neonatal period. It is usually characterized by the eruption of multiple, disseminated, red-brown papules and nodules which may increase in size and number during the first few weeks of life. Systemic signs are usually absent except for occasional mild hepatomegaly. Objective: We present a 3.5-kg male infant who presented at birth with numerous diffuse, erythematous, crusted erosions. He was presumed to have congenital herpes simplex virus (HSV) and was started on IV acyclovir. Histopathologic examination revealed a mixed inflammatory infiltrate with numerous histiocytes which were S-100 and peanut agglutin positive consistent with CSHLCH. Further workup did not reveal any signs of systemic involvement. Conclusion: CSHLCH has rarely been reported to present as a papulovesicular eruption at birth. In these cases, a viral etiology is commonly entertained in the differential diagnosis. Despite the spontaneous regression of skin lesions in CSHLCH, close followup is required to evaluate for systemic signs and symptoms associated with latent Letterer–Siwe disease.


2021 ◽  
pp. 77-81
Author(s):  
Hoon Choi ◽  
Dong Hyun Shim ◽  
Min Sung Kim ◽  
Bong Seok Shin ◽  
Chan Ho Na

Cutaneous cryptococcosis is classified either as localized cutaneous cryptococcosis, in which the lesions are confined to one area of the skin, or as disseminated cryptococcosis, in which cutaneous manifestations are more widespread. We report a case of fatal disseminated cryptococcosis with characteristic cutaneous manifestations. An 84-year-old woman with diabetes presented with crusted plaques and ulcers that were painful, diffuse, and erythematous to crusted and on only the left side of her face, neck, and upper chest. She was referred to our hospital from a local clinic, where herpes zoster had been suspected. She had no specific systemic symptoms. Histological examination of the skin lesion revealed granulomatous reactions and purple to reddish encapsulated spores. Cryptococcus neoformans was identified in fungal culture, and hospitalization was recommended. Oral fluconazole was prescribed, and she was admitted to another hospital. After 2 weeks, the patient's condition deteriorated, and she was transferred to our hospital. C. neoformans antigen was detected in the blood and urine during the evaluation for systemic involvement. The patient was treated with intravenous amphotericin B and fluconazole; however, she died 10 days after admission. Cutaneous manifestations of disseminated cryptococcosis can appear in various forms and mimic molluscum contagiosum, Kaposi's sarcoma, and cellulitis. In this case, the skin lesions occurred on only the left side of the face, neck, and chest, as in herpes zoster. Cutaneous cryptococcosis can occur before the onset of symptoms of systemic involvement; therefore, diagnosis is important. Systemic evaluation may reveal early markers of disseminated cryptococcosis.


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 ◽  
...  

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.


2017 ◽  
Author(s):  
Alexei A. Grom ◽  
Michael B Jordan ◽  
Jun Qin Mo

The mononuclear phagocytic system consists of dendritic cells (DCs) and monocytes/macrophages, historically referred to as histiocytes. The macrophages exhibit varying degrees of phagocytic activity. DCs typically have dendritic morphology. Their phagocytic activity is limited. Instead, they play a key role in antigen presentation to lymphocytes. The various populations of macrophages and DCs (such as Langerhans cells [LCs] and dermal dendrocytes) are usually distinguished based on characteristic morphology and patterns of expression of specific cell surface and intracellular markers. Abnormal accumulation and behavior of these cells may lead to the development of a spectrum of diseases collectively known as the histiocytoses. Clinically, histiocytic disorders comprise a wide variety of conditions that affect both children and adults and range from benign skin lesions to rapidly progressive life-threatening systemic disorders. LCs play a pivotal role in the development of Langerhans cell histiocytosis, dermal dendrocytes are the predominant cell population in the lesions of juvenile xanthogranuloma, and macrophages are central to the pathogenesis of hemophagocytic lymphohistiocytosis and related disorders. This review contains 5 figures, 3 tables, and 77 references. Key words: dendritic cells, dermal dendrocytes, hemophagocytic lymphohistiocytosis, histiocytes, juvenile xanthogranuloma, Langerhans cell histiocytosis, Langerhans cells, macrophage activation syndrome, macrophages 


2020 ◽  
Vol 26 (4) ◽  
pp. 449-453
Author(s):  
Jacob A. Kahn ◽  
Jeffrey T. Waltz ◽  
Ramin M. Eskandari ◽  
Cynthia T. Welsh ◽  
Michael U. Antonucci

The authors report an unusual presentation of juvenile xanthogranuloma (JXG), a non–Langerhans cell histiocytosis of infancy and early childhood. This entity typically presents as a cutaneous head or neck nodule but can manifest with more systemic involvement including in the central nervous system. However, currently there is limited information regarding specific imaging features differentiating JXG from other neuropathological entities, with diagnosis typically made only after tissue sampling. The authors reviewed the initial images of a young patient with shunt-treated hydrocephalus and enlarging, chronic, extraaxial processes presumed to reflect subdural collections from overshunting, and they examine the operative discovery of a mass lesion that was pathologically proven to be JXG. Their results incorporate the important associated histological and advanced imaging features, including previously unreported metabolic activity on FDG PET. Ultimately, the case underscores the need to consider JXG in differential diagnoses of pediatric intracranial masses and highlights the potential role of PET in the initial diagnosis and response to treatment.


2018 ◽  
Vol 2018 ◽  
pp. 1-8 ◽  
Author(s):  
Juan Pablo Sáenz ◽  
Mónica Paola Novoa ◽  
Darío Correal ◽  
Bell Raj Eapen

Background. The use of mobile applications in dermatology to support remote diagnosis is gaining acceptance, particularly in rural areas, where dermatology services are commonly managed by healthcare personnel with no specialty training. Moreover, ontologies—sets of concepts that represent knowledge in a given domain—are increasingly being used to support medical diagnosis. A specific case is ONTODerm: an ontology to aid dermatological diagnosis. However, there is little information on the combined use of mobile applications and ontologies as support solutions in dermatology. Objective. Assessing the reliability of ONTODerm as a tool to support remote dermatological diagnosis when used together with a mobile dermatological application in underprivileged areas. Methods. A mobile application that allows characterization of skin lesions was developed, and the information about the lesions was sent to ONTODerm. An exploratory study was conducted in a remote area without access to a dermatologist. A total of 64 dermatological queries were recorded in the application and consulted with ONTODerm. Later, an experienced dermatologist evaluated the characterization and diagnosis of each query to determine the accuracy of the system. Results. The results showed that the probability of obtaining a correct diagnosis was between 64.4% and 85.6% with a confidence interval of 95%. A higher accuracy rate was obtained when the skin lesion occurred on the face or when its border was categorized as poorly demarcated. Conclusions. This study demonstrates the implementation of a teledermatology strategy based on mobile applications and domain ontology-driven knowledge base to provide timely assistance to healthcare professionals. This approach was found to be pertinent in the Colombian rural context, particularly in forest regions, where dermatology specialists are not available. The results of this article do not represent a final validation of the proposed approach; they suggest how the ontology can be improved to effectively support medical staff in marginalized regions.


1999 ◽  
Vol 14 (12) ◽  
pp. 2947-2949 ◽  
Author(s):  
Minh-Truc Vo-cong ◽  
Alexandre Persu ◽  
Liliane Marot ◽  
Eric Goffin

2019 ◽  
Vol 7 (5) ◽  
pp. 782-785 ◽  
Author(s):  
Ivanka Temelkova ◽  
James Patterson ◽  
Georgi Tchernev

BACKGROUND: Scleromyxedema, also referred to as the Arndt-Gottron (S-AG) syndrome or the systemic form of Lichen myxedematosus (LM), is a cutaneous mucinosis with a chronic course and high lethality from systemic involvement of other organs and systems. Interesting in several aspects is the association between scleromyxedema and viral hepatitis about: 1) hepatitis virus infection as a possible etiological factor for the development of scleromyxedema, 2) antiretroviral therapy for the treatment of hepatitis as a method of reversing scleromyxedema and 3) antiviral drugs as inducers of scleromyxedema. CASE REPORT: We present a 53-year old patient who for nine months had been on tenofovir disoproxil 245 mg (0-0-1) therapy for chronic hepatitis B. Three months after the start of antiviral therapy (i.e. for a period of 6 months), the patient observed swelling, itching and hardening of the skin on the face, ears and hands, which subsequently spread throughout the trunk. Subsequent histological study of a skin biopsy revealed changes of scleromyxedema at an advanced stage, though immunoelectrophoresis of serum and urine excluded the presence of paraproteinaemia or para proteinuria. Systemic antihistamine and topical corticosteroid therapy were instituted. Bone involvement with possible plasmacytoma was excluded, and a myelogram showed evidence of an erythroblastic reaction of bone marrow. CONCLUSION: We believe that drug-induced scleromyxedema is a rare but possible phenomenon. We describe the first case of tenofovir-induced scleromyxedema within the framework of chronic hepatitis B treatment.


2008 ◽  
Vol 35 ◽  
pp. 50-54 ◽  
Author(s):  
Don T. Tran ◽  
Gregory M. Wolgamot ◽  
John Olerud ◽  
Stan Hurst ◽  
Zsolt Argenyi

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