Langerhans cell histiocytosis: an unusual cause of vesicular rashes in a neonate

2021 ◽  
Vol 14 (12) ◽  
pp. e247213
Author(s):  
Liang Yi Justin Wee ◽  
Alvin Ngeow ◽  
Mark Koh
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Third Trimester ◽  
Multisystem Disease ◽  
Maternal History ◽  
Systemic Involvement ◽  
The Third ◽  
History Of ◽  
Uncommon Case ◽  
Simplex Virus

The skin manifestations of Langerhans cell histiocytosis (LCH) in the neonate have a heterogenous presentation and can mimic other causes of neonatal rashes. We report an uncommon case of LCH in a term female neonate presenting with non-specific papules and vesicles from the first day of life. There was a maternal history of genital herpes simplex virus (HSV) infection in the third trimester. Blood, cerebrospinal fluid, surface swabs and vesicular fluid were negative for HSV by PCR, and a skin biopsy confirmed the diagnosis of LCH. Further investigations for systemic involvement returned negative. Our case emphasises the variable and non-specific presentation of neonatal cutaneous LCH, which can progress to or be part of multisystem disease.

Self-Healing Congenital Langerhans Cell Histiocytosis Presenting as Neonatal Papulovesicular Eruption

2001 ◽  
Vol 5 (6) ◽  
pp. 486-489 ◽  
Author(s):  
Kelli W. Morgan ◽  
Jeffrey P. Callen
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Rare Condition ◽  
Signs And Symptoms ◽  
Male Infant ◽  
Skin Lesions ◽  
Langerhans Cell ◽  
Self Healing ◽  
Systemic Involvement ◽  
S 100 ◽  
Simplex Virus

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.

scholarly journals Case series of cutaneous Langerhans cell histiocytosis in Indonesian children; The clinicopathological spectrum

2020 ◽  
Vol 12 (2) ◽  
Author(s):  
Irianiwati Widodo ◽  
Nita Sahara ◽  
Ery Kus Dwianingsih ◽  
Paranita Ferronika
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Case Series ◽  
Langerhans Cell ◽  
Multisystem Disease ◽  
Systemic Involvement ◽  
Clonal Proliferation ◽  
S 100 ◽  
Microscopic Features ◽  
Skin Biopsies ◽  
Histological Reactions

Langerhans Cell Histiocytosis (LCH) is a rare disease characterized by the clonal proliferation of Langerhans cells, which are immunoreactive to S-100 and CD-1a/ CD207 (Langerin). Cutaneous involvement is the most common presentation of LCH in children. It is suggested that the patients with single-system LCH limited to the skin have a better prognosis than those with systemic involvement. Three histologic reactions of cutaneous LCH have been reported and are associated with the clinical types of LCH. These histological reactions include: proliferative, granulomatous, and xanthomatous. This study presents the clinicopathological features of ten cutaneous LCH cases collected from Dr. Sardjito General Hospital Yogyakarta Indonesia between 2014-2018. The ten cases showed various clinical features, in which some features mimic other diseases. The microscopic features of skin biopsies showed granulomatous reaction in 80% of cases and proliferative reaction in the other 20%. Five patients (50% of cases) who died had systemic manifestation of thrombocytopenia, anemia, icterus, hepatosplenomegaly, and revealed the granulomatous type from their skin biopsy specimens. The clinical recognition of LCH and subsequent histological reaction determination are important since some cases may develop multisystem disease and have a poor prognosis.

scholarly journals Skin-limited Langerhans cell histiocytosis in an adult: a case report

2021 ◽  
Author(s):  
Neringa Guobytė ◽  
Emilija Šerpytienė ◽  
Monika Macejevska ◽  
Milda Krivickaitė ◽  
Jūratė Grigaitienė
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Skin Lesions ◽  
Langerhans Cell ◽  
Braf V600e ◽  
Multisystem Disease ◽  
Small Nodule ◽  
Systemic Spread ◽  
Life Threatening ◽  
History Of ◽  
Skin Surgery

Langerhans cell histiocytosis (LCH) is an idiopathic group of disorders characterized histologically by proliferation and infiltration of tissue by Langerhans cell-type histiocytes. This disease can affect various organs. Patients with single system lesion should be followed carefully. Detection of somatic BRAF-V600E mutation in circulating blood cells or in lesional biopsies has been associated with high-risk clinical characteristics. A 38-year old male presented to our Dermatology Centre with a 3 months history of small nodule on his right leg skin. Surgery to remove the lesion was performed. The diagnosis of skin-limited Langerhans cell histiocytosis was established. Due to possible systemic spread the patient was referred to a haematologist for further evaluation. Full body CT scan did not show any infiltrates in other organs. Bone marrow aspirate and biopsy was performed, no Langerhans cells were detected. Sometimes skin lesions may represent the most clinically evident manifestation of potentially life-threatening multisystem disease.

Langerhans Cell Histiocytosis Involving the Thymus

2003 ◽  
Vol 127 (7) ◽  
pp. e294-e297 ◽  
Author(s):  
Benjamin H. Lee ◽  
Suzanne George ◽  
Jeffery L. Kutok
Keyword(s):  
Myasthenia Gravis ◽  
Langerhans Cell Histiocytosis ◽  
Mediastinal Mass ◽  
Langerhans Cell ◽  
Anterior Mediastinal Mass ◽  
Multisystem Disease ◽  
Computed Tomographic ◽  
Aged Woman ◽  
Middle Aged Woman ◽  
History Of

Abstract Langerhans cell histiocytosis (LCH) is a neoplastic proliferation of Langerhans cells that occurs in a range of nodal and extranodal sites. Scattered reports of LCH within the thymus exist, typically among children within the setting of multifocal, multisystem disease. Rare cases of isolated LCH involving the thymus have occurred in adult patients with myasthenia gravis. We report a case of unifocal LCH involving the thymus in a middle-aged woman with a history of a resected leiomyosarcoma but no evidence of myasthenia gravis. Computed tomographic scans revealed an anterior mediastinal mass, which was excised and measured 9.0 cm. Histologic and immunophenotypic findings (CD1a, S100, and Fascin positive and CD68 negative) were consistent with LCH. To our knowledge, this is the first example of LCH occurring in a patient with a history of soft tissue sarcoma and one of the rare reported examples of LCH presenting as a large isolated lesion in the thymus of a nonmyasthenic adult.

scholarly journals Paediatric Langerhans Cell Histiocytosis Disease: Long-Term Sequelae in the Hypothalamic Endocrine System

2021 ◽  
pp. 1-9
Author(s):  
Elisa Vaiani ◽  
Guido Felizzia ◽  
Fabiana Lubieniecki ◽  
Jorge Braier ◽  
Alicia Belgorosky
Keyword(s):  
Anterior Pituitary ◽  
Langerhans Cell Histiocytosis ◽  
Endocrine System ◽  
Langerhans Cell ◽  
Hormone Deficiency ◽  
Bone Lesions ◽  
Pituitary Hormone ◽  
Multisystem Disease ◽  
Anterior Pituitary Hormone

Langerhans cell histiocytosis (LCH) is a disorder of the mononuclear phagocyte system that can affect almost any organ and system. The most common central nervous system (CNS) manifestation in LCH is the infiltration of the hypothalamic-pituitary region leading to destruction and neurodegeneration of CNS tissue. The latter causes the most frequent endocrinological manifestation, that is, central diabetes insipidus (CDI), and less often anterior pituitary hormone deficiency (APD). The reported incidence of CDI is estimated between 11.5 and 24% and is considered a risk factor for neurodegenerative disease and APD. Three risk factors for development of CDI are recognized in the majority of the studies: (1) multisystem disease, (2) the occurrence of reactivations or active disease for a prolonged period, and (3) the presence of craniofacial bone lesions. Since CDI may occur as the first manifestation of LCH, differential diagnosis of malignant diseases like germ cell tumours must be made. APD is almost always associated with CDI and can appear several years after the diagnosis of CDI. Growth hormone is the most commonly affected anterior pituitary hormone. Despite significant advances in the knowledge of LCH in recent years, little progress has been made in preventing long-term sequelae such as those affecting the hypothalamic-pituitary system.

Progressive Mucinous Histiocytosis: Importance of Electron Microscopy to Confirm Diagnosis

2010 ◽  
Vol 14 (5) ◽  
pp. 245-248 ◽  
Author(s):  
Iman Hemmati ◽  
W. Alastair McLeod ◽  
Richard I. Crawford
Keyword(s):  
Electron Microscopy ◽  
Light Microscopy ◽  
Clinical Examination ◽  
Crucial Role ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
History Of ◽  
Characteristic Features ◽  
Immunohistochemical Studies

Background: Progressive mucinous histiocytosis (PMH) is a benign, non-Langerhans cell histiocytosis with characteristic ultrastructural features that can be used for diagnosis. Once an important tool in dermatologic diagnosis, electron microscopy has been largely replaced by immunohistochemistry and immunofluorescence techniques today. However, electron microscopy occasionally still plays a crucial role in the diagnosis of dermatologic conditions. We report a case of PMH as an example of a dermatologic disorder that requires electron microscopy for its diagnosis. Methods: A 60-year-old woman presented to our clinic with a history of small, sharply demarcated, skin-colored papules ranging from 2 to 5 mm in diameter distributed over the arms, forearms, and dorsal hands. The results of light microscopy, immunohistochemical studies, and clinical examination were inconclusive. Another biopsy for electron microscopy showed the characteristic features of PMH. Conclusion: This case demonstrates that a dermatopathology service still needs to have access to electron microscopy for diagnostic purposes to successfully diagnose a small number of rare conditions.

Herpes Simplex Virus Infections

1996 ◽  
Vol 17 (12) ◽  
pp. 415-423
Author(s):  
Paula W. Annunziato
Keyword(s):  
Herpes Simplex Virus ◽  
Herpes Simplex ◽  
Hepatitis B Surface Antigen ◽  
Liver Function Tests ◽  
Virus Infections ◽  
Sexually Transmitted ◽  
The Third ◽  
Intravenous Acyclovir ◽  
History Of ◽  
Simplex Virus

Case Report A term female developed fever and tachypnea and had a transient episode of poor color and tone on the third day of life. She was delivered by cesarean section 5 hours after rupture of membranes to a 19-year-old, gravida 4, para 0 mother whose serology was negative for syphilis, human immunodeficiency virus (HIV), and hepatitis B surface antigen. The mother had no history of sexually transmitted diseases. Apgar scores were 8 at 1 minute and 9 at 10 minutes. When the infant was admitted to the nursery, scalp abrasions were noticed by the nurse. On the third day of life, a sepsis evaluation was initiated, and the infant received ampicillin and gentamicin intravenously: no bacterial infection was found. On the fifth day of life, vesicular lesions were noticed on her scalp. Bilateral interstitial infiltrates were present On chest radiograph and laboratory studies revealed mild elevations in liver function tests. There were no cerebrospinal fluid (CSF) abnormalities. She was started on intravenous acyclovir, and herpes simplex virus (HSV)-2 subsequently was isolated from both her pharynx and scalp lesions. After receiving intravenous acyclovir for 3 weeks, she was discharged with no evidence of residual sequelae. Four days after acyclovir was discontinued, new scalp vesicles appeared and the infant developed a temperature of 38.8°C (102°F).

Adult-onset Neurological Langerhans Cell histiocytosis and Response to Treatment

2021 ◽  
Vol 8 (1) ◽  
Author(s):  
Al-Hader R ◽  
◽  
Suneja A ◽  
Memon AB ◽  
Mukherje A ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Brain Magnetic Resonance Imaging ◽  
Langerhans Cell ◽  
Response To Therapy ◽  
Response To Treatment ◽  
Adult Onset ◽  
Multisystem Disease ◽  
Clonal Proliferation ◽  
Magnetic Resonance Imaging Mri ◽  
Mass Lesions

Introduction: Langerhans Cell Histiocytosis (LCH) is a rare form of cancer that mostly affects children and rarely adults. LCH involves an abnormal clonal proliferation of Langerhans cells in the bone marrow. These cells are capable of migrating from the skin to lymph nodes. Therefore, it is characterized as a multisystem disease. Neurological manifestations are not common, and often patients’ present with endocrine dysfunction with neuroimaging findings of hypothalamic and pituitary masses can mimic pituitary adenoma. Here, we discuss two instances of unusual adult-onset, primary neurological LCH in patients with a positive response to therapy-these two patients presented with mass lesion and neurodegenerative form of LCH, respectively. LCH can manifest features of mass lesions or neurodegeneration on brain Magnetic Resonance Imaging (MRI). Since it is rare in adults, it is crucial to identify this condition as timely treatment can have a better prognosis.

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