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

2021 ◽  
Vol 47 (1) ◽  
Author(s):  
Alessandra Rizzoli ◽  
Simona Giancristoforo ◽  
Cristina Haass ◽  
Rita De Vito ◽  
Stefania Gaspari ◽  
...  
Keyword(s):  
Oral Mucosa ◽  
Langerhans Cell Histiocytosis ◽  
Skin Lesions ◽  
Langerhans Cell ◽  
Self Healing ◽  
Review Of The Literature ◽  
Cutaneous Lesions ◽  
Cutaneous Manifestations ◽  
Case Presentation

Abstract 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. Patients affected by CSHRH require a close follow-up until the first years of life, due to the unpredictable course of Langerhans cell histiocytosis, in order to avoid missing diagnosis of more aggressive types of this disorder.

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.

scholarly journals LANGERHANS CELL HISTIOCYTOSIS OF THE SCAPULA - DIAGNOSIS & TREATMENT OPTIONS

2017 ◽  
Vol 16 (3) ◽  
pp. 240-243 ◽  
Author(s):  
ROHIT PANDEY ◽  
HIMANSHU BHAYANA ◽  
RAJESH KUMAR RAJNEESH ◽  
ISH KUMAR DHAMMI ◽  
REHAN UL-HAQ ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Treatment Options ◽  
Langerhans Cell ◽  
Single System ◽  
Diagnostic Dilemma ◽  
Systemic Involvement ◽  
System Disease ◽  
S 100 ◽  
Specific Finding

ABSTRACT Langerhans cell histiocytosis (LCH) is characterised by an abnormal histiocytic accumulation in tissues such as the lung, spleen, bone marrow, skin, central nervous system, liver and lymph nodes, causing focal or systemic effects. No specific clinical & radiographic presentation of LCH is described in literature. This poses a diagnostic dilemma for surgeons. The scapula is the site of 3% of bone tumours, while for LCH it is the least common site. In a 10-year-old boy with isolated lesion of the scapula with no other systemic involvement, and no specific finding in MRI or CT scan of scapula, diagnosis was confirmed on biopsy. Division into single and multi-system disease is paramount in treatment, given that it is a single system disease. The patient improved clinically on follow-up of 2 years. The scapula is one of the rarest site of LCH, and because various lesions mimic each other, a biopsy is always required, with immunohistochemistry for CD68 & S-100. This was only a single system disease, so conservative management was performed, and the patent improved clinically.

Occult Langerhans Cell Histiocytosis in Clear Cell Renal Cell Carcinoma

2017 ◽  
Vol 26 (2) ◽  
pp. 194-196 ◽  
Author(s):  
Hee Jung Kwon ◽  
Phil Hyun Song ◽  
Mi Jin Gu
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Langerhans Cell Histiocytosis ◽  
Mononuclear Cells ◽  
Clear Cell ◽  
Wide Spectrum ◽  
Langerhans Cell ◽  
Cell Renal Cell Carcinoma ◽  
S 100

Langerhans cell histiocytosis is a rare disease that is characterized by a localized or systemic proliferation of Langerhans dendritic cells and a wide spectrum of clinical presentations. We experienced an unusual case of occult Langerhans cell histiocytosis associated with clear cell renal cell carcinoma. A 62-year-old man underwent a partial nephrectomy for left renal mass. Histologic examination showed nests of clear cells with surrounding thin-walled vessel that were suggestive of clear cell renal cell carcinoma. A well-defined nodule composed of atypical mononuclear cells and plentiful eosinophils presented within clear cell renal cell carcinoma. The mononuclear cells were Langerhans cell histiocytes with S-100 and CD1a immunoreactivity. On follow-up comprehensive physical, laboratory, and radiologic examination, there were no other lesions of Langerhans cell histiocytosis.

Pulmonary Langerhans Cell Histiocytosis

2020 ◽  
Vol 41 (02) ◽  
pp. 269-279
Author(s):  
Brian Shaw ◽  
Michael Borchers ◽  
Dani Zander ◽  
Nishant Gupta
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Treatment Options ◽  
Skin Lesions ◽  
Langerhans Cell ◽  
Mitogen Activated Protein Kinase ◽  
Myeloid Neoplasm ◽  
Cystic Lung ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Mitogen Activated Protein ◽  
The Central Nervous System

AbstractPulmonary Langerhans cell histiocytosis (PLCH) is a diffuse cystic lung disease that is strongly associated with exposure to cigarette smoke. Recently, activating pathogenic mutations in the mitogen-activated protein kinase pathway have been described in the dendritic cells in patients with PLCH and have firmly established PLCH to be an inflammatory myeloid neoplasm. Disease course and prognosis in PLCH are highly variable among individual patients, ranging from spontaneous resolution to development of pulmonary hypertension and progression to terminal respiratory failure. A subset of patients with PLCH may have extrapulmonary involvement, typically involving the skeletal system in the form of lytic lesions, skin lesions, or the central nervous system most commonly manifesting in the form of diabetes insipidus. Smoking cessation is the cornerstone of treatment in patients with PLCH and can lead to disease regression or stabilization in a substantial proportion of patients. Further insight into the underlying molecular pathogenesis of PLCH has paved the way for the future development of disease-specific biomarkers and targeted treatment options directed against the central disease-driving mutations.

Laryngeal Langerhans Cell Histiocytosis: A Case Report and Literature Review

2020 ◽  
pp. 000348942095488
Author(s):  
Allen S. Zhou ◽  
Lei Li ◽  
Thomas L. Carroll
Keyword(s):  
Literature Review ◽  
Langerhans Cell Histiocytosis ◽  
Medical Center ◽  
Tertiary Care ◽  
Rare Condition ◽  
Surgical Excision ◽  
Langerhans Cell ◽  
Histopathological Analysis ◽  
Current Case ◽  
Tertiary Care Medical Center

Objective: To describe a case of laryngeal Langerhans cell histiocytosis, discuss its characteristic features and management, and provide a review of the available literature. Methods: A patient presenting to a tertiary care medical center with dyspnea and hoarseness is described. A literature review of laryngeal Langerhans cell histiocytosis cases was performed through a search of articles indexed in the National Institutes of Health PubMed system. Results: We report a case of a 69-year old male, who presented with a laryngeal mass highly suspicious for laryngeal squamous cell carcinoma, was treated with laser excision, and was subsequently found to have laryngeal Langerhans cell histiocytosis upon histological analysis. Including our current case, we found six prior reported cases of laryngeal Langerhans cell histiocytosis in the literature. Of the six cases, four were in adults, while two were in children. Dyspnea is a common presenting complaint present in all cases. Smoking may be a potential risk factor. Conclusions: Laryngeal Langerhans cell histiocytosis is a rare condition and an important consideration in the differential diagnosis of patients presenting with a laryngeal mass and symptoms of dyspnea or hoarseness. Biopsy and histopathological analysis are key to the diagnosis. Surgical excision and radiotherapy are successful treatments used in clinical practice.

scholarly journals Isolated Langerhans Cell Histiocytosis of Orbit: A Case Report and Review of the Literature

2018 ◽  
Vol 2018 ◽  
pp. 1-6
Author(s):  
Mayuresh Naik ◽  
Anuj Mehta ◽  
Neha Mehrotra ◽  
Anil Solanki
Keyword(s):  
Body Weight ◽  
Langerhans Cell Histiocytosis ◽  
Bone Erosion ◽  
Histopathological Examination ◽  
Oral Prednisolone ◽  
Langerhans Cell ◽  
The Body ◽  
S 100 ◽  
Immunohistochemical Studies ◽  
Incision Biopsy

A 2-year-old male child presented with a painless progressive mass in the inferolateral aspect of right orbit of three-month duration. Differential leucocyte count revealed raised eosinophil count (13%). On radiological examination, CT scan showed 25 × 27 mm round well-defined smooth-outlined homogenously enhancing extraconal mass arising from the zygomatic bone at the inferotemporal periorbital area of right orbit with bone erosion. Histopathological examination of the incision biopsy revealed characteristic Langerhans cells and immunohistochemical studies were positive for S-100 protein and adenosine deaminase. A diagnosis of Langerhans Cell Histiocytosis (LCH) was made and PET-CT revealed no other foci of uptake anywhere else in the body. The patient received 12 cycles of vinblastine, 0.2 mg/kg body weight, along with oral prednisolone, 1 mg/kg body weight. On completion of three cycles of chemotherapy, a reduction in size of the mass was noticed. A repeat PET scan was done 3 months after completion of chemotherapy did not reveal any activity noted previously.

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