110 results match your criteria Congenital Self-Healing Reticulohistiocytosis


Congenital self-healing reticulohistiocytosis in a newborn: unusual oral and cutaneous manifestations.

Ital J Pediatr 2021 Jun 10;47(1):135. Epub 2021 Jun 10.

Dermatology Unit, Bambino Gesù Children's Hospital, IRCCS, Piazza S. Onofrio, 4, 00165, Rome, Italy.

Background: Congenital self-healing reticulohistiocytosis (CSHRH), also called Hashimoto-Pritzker disease, is a rare and benign variant of Langerhans cell histiocytosis, characterized by cutaneous lesions without extracutaneous involvement.

Case Presentation: We present a case of CSHRH with diffuse skin lesions and erosions in the oral mucosa, present since birth and lasting for 2 months, and we perform a review of the literature on Pubmed in the last 10 years.

Conclusions: Our case confirm that lesions on oral mucosa, actually underestimated, may be present in patients with CSHRH. Read More

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Congenital self-healing reticulohistiocytosis in a neonate.

Arch Dis Child Fetal Neonatal Ed 2021 May 10. Epub 2021 May 10.

Department of Dermatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, China, Beijing, China

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BRAF V600E-Positive Congenital Multisite Langerhans Cell Histiocytosis.

Cureus 2020 Sep 2;12(9):e10200. Epub 2020 Sep 2.

Pediatric Oncology, Instituto Nacional de Cancerología, Bogotá, COL.

Congenital Langerhans cell histiocytosis (LCH) usually manifests as a disease limited to the skin, with self-healing characteristics; however, in some cases, it may be a more severe entity, with multisystemic expression and poor prognosis. We present the case of a patient diagnosed with multisystemic congenital LCH, with the presence of the BRAF V600E mutation, with a severe form of the disease, with risk organ compromise, and manifestations of resistance to chemotherapy. This case is a challenge due to the disease's biologically aggressive behavior in this patient. Read More

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September 2020

Solitary congenital Langerhans cell histiocytoma: A pattern of benign, spontaneous regression in patients with single lesion disease.

Pediatr Dermatol 2020 Nov 14;37(6):1009-1013. Epub 2020 Sep 14.

Weill Cornell Medicine Department of Dermatology, New York, NY, USA.

Langerhans cell histiocytosis (LCH) is the neoplastic proliferation of dendritic langerin-positive histiocytes manifesting as either single system unifocal, single system multifocal, or multisystem disease. The designation Hashimoto-Pritzker, or self-healing LCH, has fallen out of favor since it is impossible to predict at time of diagnosis whether the disease is truly self-remitting or capable of spreading to other organ systems. We review the English literature on solitary congenital Langerhans cell histiocytoma, draw novel conclusions from the data provided by 81 cases in the literature, and illustrate a typical presentation of the diagnosis with a previously unreported patient. Read More

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November 2020

Congenital self-healing langerhans cell histiocytosis: clinical and pathological characteristics.

Int J Clin Exp Pathol 2019 1;12(6):2275-2278. Epub 2019 Jun 1.

Department of Pathology, Southern Medical University Affiliated Maternal and Child Health Hospital of Foshan Foshan 528000, Guangdong, P. R. China.

Introduction: Congenital Self-Healing Langerhans Cell Histiocytosis (CSHLCH) is rare, characterized by cutaneous lesions at birth or in the neonatal period, and absence of systemic lesions.

Materials And Methods: Skin biopsy was performed and the histologic examination of the skin section, routinely stained with hematoxylin-eosin. Paraffin sections were stained by immunohistochemical study which were carried out according to the manufacturer's protocols. Read More

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Hyperpigmented nodule overlying the right Achilles tendon.

JAAD Case Rep 2019 Nov 28;5(11):985-987. Epub 2019 Oct 28.

Department of Dermatology University Hospitals Cleveland Medical Center, Cleveland, Ohio.

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November 2019

Extracellular signal-regulated kinase activation of self-healing Langerhans cell histiocytosis: A case report.

J Dermatol 2019 Sep 4;46(9):812-815. Epub 2019 Jul 4.

Department of Dermatology, Graduate School of Medicine, University of the Ryukyus, Nishihara, Japan.

A 3-month-old boy developed small papules on his trunk. After the papules increased in number, the patient was diagnosed with Langerhans cell histiocytosis based on the pathological findings. He was referred to our department for further examination. Read More

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September 2019

Congenital Self-Healing Langerhans Cell Histiocytosis: A Rare Presentation of Blueberry Muffin Baby "Spectrum".

Dermatopathology (Basel) 2019 Apr-Jun;6(2):37-40. Epub 2019 May 7.

Section of Dermatology, Department of Medicine, University of Perugia, Perugia, Italy.

A case of congenital self-healing Langerhans cell histiocytosis (CSHLCH), also known as Hashimoto-Pritzker disease, is reported. The newborn presented as blueberry muffin baby at birth, showing numerous non-blanching blue-purplish and dark-red papular, nodular lesions without documented infections and systemic involvement. Histopathological and immunohistochemical findings were suggestive for Langerhans cell histiocytosis. Read More

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Congenital self-healing reticulohistiocytosis with BRAF V600E mutation in an infant.

Clin Exp Dermatol 2019 Aug 4;44(6):647-650. Epub 2019 Jan 4.

Department of Pediatrics, The Seventh Affiliated Hospital, Sun Yat-Sen University, Shenzhen, China.

Congenital self-healing reticulohistiocytosis (CSHR) is a rare disorder characterized by benign skin lesions with a tendency to self-heal. Multiple skin lesions are usually present in CSHR. It is very difficult to distinguish between CSHR and an invasive Langerhans cell histiocytosis. Read More

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Langerhans Cell Histiocytoma: A Benign Histiocytic Neoplasm of Diverse Lines of Terminal Differentiation.

Am J Dermatopathol 2019 Jan;41(1):29-36

Pathology Department, Necker-Enfants Malades Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.

Langerhans cell (LC) histiocytoma is a neonatal tumor that often consists of a single, ulcerated nodule. Systemic involvement is rare, and LC histiocytoma is considered to be a variant of congenital, self-healing LC histiocytosis (also referred to as Hashimoto-Pritzker disease). In view of its low prevalence, LC histiocytoma is not always diagnosed in a clinical examination and requires histological confirmation. Read More

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January 2019

A case of congenital self-healing Langerhans cell histiocytosis with two lesions.

Eur J Dermatol 2018 Aug;28(4):548-549

Department of Pathology, Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing 210042, China.

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Case of Congenital Self-Healing Reticulohistiocytosis Expanding the Spectrum of Blueberry Muffin Baby.

Am J Dermatopathol 2018 Feb;40(2):136-138

Dermatology Department, Hospital Universitario del Valle, Universidad del Valle, Cali, Colombia.

Congenital self-healing reticulohistiocytosis (CSHRH) is a benign, rare variant of histiocytosis. This condition can present phenotypically as blueberry muffin baby. This is the case of a male neonate with skin involvement of papulonodular violaceous lesions, which resolved spontaneously. Read More

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February 2018

Congenital self-healing reticulohistiocytosis with spontaneous regression.

An Bras Dermatol 2017 Jul-Aug;92(4):553-555

Department of Dermatology, Shandong Provincial Hospital of Dermatology and Venereology, Shandong University, Jinan, Shandong, China.

Congenital self-healing reticulohistiocytosis is a rare, benign, self-limiting variant of Langerhans cell histiocytosis (LCH). LCH encompasses a group of idiopathic disorders characterized by the clonal proliferation of Langerhans cells. Congenital self-healing reticulohistiocytosis typically appears at birth or in the neonatal period as isolated cutaneous lesions, often appearing as multiple crusted papules with no systemic findings. Read More

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October 2017

Congenital Self Healing Langerhans Cell Histiocytosis.

Indian J Pediatr 2018 04 19;85(4):316-317. Epub 2017 Sep 19.

Department of Neonatology, Sawai Man Singh Medical College, Jaipur, Rajasthan, India.

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Congenital Self-Healing Langerhans Cell Histiocytosis.

J Pediatr 2017 05 21;184:232-232.e1. Epub 2017 Feb 21.

Section of Pediatric Dermatology Children's Hospital of Philadelphia; Department of Pediatrics and Dermatology University of PennsylvaniaPhiladelphia, Pennsylvania.

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Congenital self-healing reticulohistiocytosis: an underreported entity.

Cutis 2016 Apr;97(4):296-300

Western University of Health Sciences, Pomona, California, and the Department of Dermatology, Pacific Hospital of Long Beach, California, USA.

Langerhans cell histiocytosis (LCH), also known as histiocytosis X, is a group of rare disorders characterized by the continuous replication of a particular white blood cell called Langerhans cells. These cells are derived from the bone marrow and are found in the epidermis, playing a large role in immune surveillance and the elimination of foreign substances from the body. Additionally, Langerhans cells are capable of migrating from the skin to lymph nodes, and in LCH, these cells begin to congregate on the bone, particularly in the head and neck region, causing a multitude of problems. Read More

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Congenital self-healing reticulohistiocytosis: the need for investigation.

Australas J Dermatol 2016 Feb;57(1):76-7

Paediatric Oncology, Starship Hospital, Auckland, New Zealand.

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February 2016

Diverse Cutaneous Presentations of Langerhans Cell Histiocytosis in Children: A Retrospective Cohort Study.

Pediatr Blood Cancer 2016 Mar 19;63(3):486-92. Epub 2015 Nov 19.

Laboratory of Pediatric Immunology, KU Leuven, Leuven, Belgium.

Background: Langerhans cell histiocytosis (LCH) is a rare disease, frequently affecting young children.

Procedure: We performed a retrospective study in patients younger than 16 years old manifesting with skin symptoms, and documented their different cutaneous lesions and systemic symptoms. We compared subgroups of children with single-system, skin-only, and multisystem disease and sought signs predictive for multisystem disease. Read More

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Congenital self-healing Langerhans cell histiocytosis in a male neonate.

Indian J Dermatol Venereol Leprol 2015 Jan-Feb;81(1):75-7

Department of Dermatology, Children's Hospital of Chong Qing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, China.

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September 2015

Congenital self-healing reticulohistiocytosis presented with multiple hypopigmented flat-topped papules: a case report and review of literatures.

J Med Assoc Thai 2014 Sep;97(9):993-7

Congenital self-healing reticulohistiocytosis, also known as Hashimoto-Pritzker disease, is a single system Langerhans cell histiocytosis that typically presents in healthy newborns and spontaneously regresses. In the present report, we described a 2-month-old Thai female newborn with multiple hypopigmented flat-topped papules without any internal organ involvement including normal blood cell count, urinary examination, liver and renal functions, bone scan, chest X-ray, abdominal ultrasound, and bone marrow biopsy. The histopathology revealed typical findings of Langerhans cell histiocytosis, which was confirmed by the immunohistochemical staining CDla and S100. Read More

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September 2014

Congenital "self-healing" Langerhans cell histiocytosis (Hashimoto-Pritzker disease): a report of two cases with the same cutaneous manifestations but different clinical course.

J Dermatol 2014 Dec 30;41(12):1098-101. Epub 2014 Oct 30.

Department of Dermatology, University of Modena and Reggio Emilia, Modena, Italy.

Congenital self-healing Langerhans cell histiocytosis or Hashimoto-Pritzker disease is a rare condition present at birth or in the neonatal period characterized by small reddish-brown crusted papulonodular lesions. In most cases these lesions are not accompanied by systemic findings and tend to involute spontaneously within weeks or months, but in other cases there may be extracutaneous involvement and/or recurrence of the disease. This emphasizes that the clinical course is variable and a long-term follow-up is mandatory in order to reveal possible systemic involvement. Read More

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December 2014

Solitary congenital erosion in a newborn: report of a solitary congenital self-healing reticulohistiocytosis.

Ann Dermatol 2014 Apr 30;26(2):250-3. Epub 2014 Apr 30.

Department of Dermatology, St. Paul's Hospital, School of Medicine, The Catholic University of Korea, Seoul, Korea.

Congenital self-healing reticulohistiocytosis (CSHRH) is a rare, cutaneous, self-limited form of Langerhans cell histiocytosis. Whereas multiple lesions are common, a solitary lesion is rare. A 14-day-old neonate presented with a solitary, 5-mm, oval, reddish, and eroded papule with crust on the left thigh that had existed since birth. Read More

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Multisystemic Langerhans cell histiocytosis mimicking diffuse neonatal hemangiomatosis.

Pediatr Dermatol 2014 May-Jun;31(3):e87-9. Epub 2014 Mar 3.

Department of Dermatology, Hospital Universitario 12 de Octubre, Madrid, Spain.

We report the first case of multisystemic Langerhans cell histiocytes mimicking diffuse neonatal hemangiomatosis clinically. This has been described in patients with congenital self-healing reticulohistiocytosis but not in patients with acute, disseminated, and multisystemic disease. In our experience, dermoscopic findings did not help to diagnose the condition. Read More

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December 2014

Congenital self-healing reticulohistiocytosis.

J Clin Aesthet Dermatol 2014 Feb;7(2):49-53

The authors are from Penn State/Hershey Medical Center, Hershey, Pennsylvania.

Congenital self-healing reticulohistiocytosis, also known as congenital self-healing Langerhans cell histiocytosis or Hashimoto-Pritzker disease, is a Langerhans cell histiocytosis. It is characterized by skin lesions in the newborn period in an otherwise healthy infant that show a Langerhans cell infiltrate in the skin on histological analysis. These findings subsequently spontaneously involute. Read More

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February 2014

Solitary congenital self-healing Langerhans cell histiocytosis: a benign variant of Langerhans cell histiocytosis?

Dermatol Online J 2013 Jan 15;19(1). Epub 2013 Jan 15.

University of British Columbia, Vancouver, BC, Canada.

Langerhans cell histiocytosis (LCH) represents a diverse group of diseases with various different clinical presentations and outcomes. We present two cases of solitary CSH-LCH (sCSH-LCH) and highlight certain unique characteristics including the favorable prognosis and lack of documented progression to systemic involvement in reported cases. Read More

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January 2013

Congenital self-healing reticulohistiocytosis: concern for a poor prognosis.

Dermatol Online J 2012 Oct 15;18(10). Epub 2012 Oct 15.

School of Medicine, University of California Davis, Sacramento, California, USA.

Congenital self-healing reticulohistiocytosis (CSHRH) is a rare type of Langerhans cell histiocytosis with potential for relapse and systemic involvement. Whereas CSHRH was traditionally considered a benign disease, there is an approximately 3 percent risk of mortality and a 10 percent chance of relapse. This article, using an extensive review of cases since Hashimoto and Pritzker first described the condition in 1973, highlights the various presentations of CSHRH and reveals high rates of relapse and systemic involvement in cases that specifically address features of CSHRH occurring within the first year of life. Read More

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October 2012

Congenital self-healing histiocytosis presenting as blueberry muffin baby: a case report and literature review.

Indian J Dermatol Venereol Leprol 2012 May-Jun;78(3):407

Clinic of Dermatovenereology, Clinical Center of Serbia, Department of Dermatovenereology, Faculty of Medicine, University of Belgrade, Belgrade, Serbia.

Congenital self-healing Langerhans cell histiocytosis (CSHLCH), also called as Hashimoto-Pritzker disease, is a rare, benign variant of histiocytosis. Despite the initial dramatic clinical presentation, affected infants are otherwise healthy and skin lesions disappear spontaneously within several weeks to months. We present a case of CSHLCH presenting as blueberry muffin baby. Read More

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September 2012

[Disseminated juvenile xanthogranuloma expressing protein S100].

Ann Dermatol Venereol 2012 Feb 6;139(2):128-31. Epub 2011 Dec 6.

Service de dermatologie, hôpital Hédi-Chaker, route El Ain km 1, 3029 Sfax, Tunisie.

Background: Juvenile xanthogranuloma (JXG) is a form of self-healing non-Langerhans histiocytosis. We report a new case of disseminated JXG without visceral involvement but with positive immunostaining of the majority of histiocytes for protein S100.

Patients And Methods: A 6-month-old male infant was admitted to our department in January 2010 for congenital nodular lesions of varying size, shape and appearance. Read More

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February 2012

[Congenital self-healing Langerhans cell histiocytosis].

Hautarzt 2011 Nov;62(11):804-7

Klinik für Dermatologie und Allergologie, Universitätsklinikum RWTH Aachen, Pauwelsstr. 30, 52074 Aachen.

A male infant, born at term after an uncomplicated pregnancy, presented with multiple red-brown papules and nodules spread over his body. The infant's general condition was not affected and there was no systemic involvement. After only 6 weeks the lesions had healed completely without recurrence. Read More

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November 2011