Langerhans cell histiocytosis (LCH) is a group of unexplainable abnormal proliferation and aggregation of Langerhans cell. LCH can be classified into four clinical variants: Letterer-Siwe disease, Hand-Schüller-Christian disease, eosinophilic granuloma, and congenital self-healing LCH. LCH is most prevalent in children. Lesions can be localized in a single system or multiple organs, and clinical manifestations vary depending on the affected organs. The skin and mucocutaneous tissues are the starting point of the affected tissue. This study presents a LCH case characterized by transient self-healing. This case can further provide references for the clinical diagnosis and treatment of LCH.
Child ; Humans ; Histiocytosis, Langerhans-Cell/therapy* ; Diagnosis, Differential

Adult ; Humans ; Stomach ; Histiocytosis, Langerhans-Cell/diagnosis*

Consensus ; Histiocytosis, Langerhans-Cell/diagnosis* ; Humans

Histiocytosis, Langerhans-Cell/diagnosis* ; Humans ; Periodontitis/diagnosis*

A girl, aged 22 months, attended the hospital due to recurrent vulvar rashes for more than half a year. Skin biopsy showed Langerhans cell histiocytosis, and evaluation of systemic conditions showed no systemic involvement. Therefore, the girl was diagnosed with Langerhans cell histiocytosis (skin type). In conclusion, for rashes on the vulva alone, if there are no specific clinical manifestations, the possibility of Langerhans cell histiocytosis should be considered after molluscum contagiosum, sexually transmitted diseases, and Fordyce disease are excluded.
Developmental Disabilities ; Exanthema/etiology* ; Female ; Histiocytosis, Langerhans-Cell ; Humans ; Infant ; Vulvar Diseases/diagnosis*

Lung cysts are commonly seen on computed tomography (CT), and cystic lung diseases show a wide disease spectrum. Thus, correct diagnosis of cystic lung diseases is a challenge for radiologists. As the first diagnostic step, cysts should be distinguished from cavities, bullae, pneumatocele, emphysema, honeycombing, and cystic bronchiectasis. Second, cysts can be categorized as single/localized versus multiple/diffuse. Solitary/localized cysts include incidental cysts and congenital cystic diseases. Multiple/diffuse cysts can be further categorized according to the presence or absence of associated radiologic findings. Multiple/diffuse cysts without associated findings include lymphangioleiomyomatosis and Birt-Hogg-Dubé syndrome. Multiple/diffuse cysts may be associated with ground-glass opacity or small nodules. Multiple/diffuse cysts with nodules include Langerhans cell histiocytosis, cystic metastasis, and amyloidosis. Multiple/diffuse cysts with ground-glass opacity include pneumocystis pneumonia, desquamative interstitial pneumonia, and lymphocytic interstitial pneumonia. This stepwise radiologic diagnostic approach can be helpful in reaching a correct diagnosis for various cystic lung diseases.
Amyloidosis ; Birt-Hogg-Dube Syndrome ; Bronchiectasis ; Diagnosis ; Emphysema ; Histiocytosis ; Histiocytosis, Langerhans-Cell ; Lung Diseases ; Lung Diseases, Interstitial ; Lung ; Lymphangioleiomyomatosis ; Neoplasm Metastasis ; Pneumonia, Pneumocystis

Germinoma is the most common type of intracranial germ cell tumors (GCTs). Pineal gland and suprasellar region are the most frequent sites of central nervous system (CNS) involvement. Intracranial masses caused by Langerhans cell histiocytosis (LCH) mimics features of CNS GCTs. LCH frequently involve spine and is the most common cause of vertebra plana in children. A 15-year-old boy presented with progressing symptoms of polydipsia, polyuria, general headache, nausea and severe back pain. Brain MRI showed brain tumor with simultaneous involvement of suprasellar region and pineal gland. An excisional biopsy of suprasellar mass was done. The pathologic assessment confirmed the diagnosis of germinoma. Patient's treatment continued accordingly. A spine MRI, done due to persistent backache, showed a vertebra plana. We reevaluated the primary diagnosis suspecting LCH. Germinoma of CNS was confirmed and a biopsy of vertebral lesion resulted in hemangioma. Thus we report a case of CNS germinoma with co-occurrence of vertebra plana. We emphasized the importance of histopathologic diagnosis of pineal/suprasellar masses and primary investigation of other CNS regions including spine for possible metastasis or comorbidities.
Adolescent ; Back Pain ; Biopsy ; Brain ; Brain Neoplasms ; Central Nervous System ; Child ; Comorbidity ; Diagnosis ; Germinoma* ; Headache ; Hemangioma ; Histiocytosis, Langerhans-Cell ; Humans ; Magnetic Resonance Imaging ; Male ; Nausea ; Neoplasm Metastasis ; Neoplasms, Germ Cell and Embryonal ; Pineal Gland ; Polydipsia ; Polyuria ; Spine*

BACKGROUND: Langerhans cell histiocytosis (LCH) frequently involves the head and neck and increases the risk of central nervous system (CNS) involvement of LCH, such as central diabetes insipidus (CDI), when the craniofacial bones are involved. We analyzed risk factors and clinical features of CDI among patients with LCH involving the head and neck. METHODS: From January 1, 2000 to May 1, 2018, 63 patients with histologically confirmed LCH in the Department of Pediatrics, Ajou University Hospital were retrospectively analyzed. RESULTS: Forty eight cases (76.2%) of patients had head and neck involvement, and 9 cases (14.3%) in craniofacial bones at the time of initial diagnosis of LCH. CDI was found in 6 cases (9.5%) among all LCH patients, 6 cases (12.2%) among patients with head and neck involvement, and 3 cases (33.3%) among patients with craniofacial bone involvement. Three cases of CDI occurred at the time of initial LCH diagnosis, and another 3 cases occurred at the time of 2, 4, and 8 years after initial LCH diagnosis. Of the 6 CDI patients, 3 had CNS risk lesions and 3 had no CNS risk lesions, but all had multi-system involvement of LCH. CONCLUSION: CDI can occur even in patients with head and neck LCH without CNS risk lesions, if there are multisystem involvement of LCH. Patients with head and neck LCH may develop CDI over time, so continuous observations should be done while considering the occurrence of CDI.
Central Nervous System ; Diabetes Insipidus, Neurogenic* ; Diagnosis ; Head* ; Histiocytosis, Langerhans-Cell* ; Humans ; Neck* ; Pediatrics ; Retrospective Studies ; Risk Factors

Langerhans cell histiocytosis (LCH) is characterized by clonal proliferation and accumulation of abnormal dendritic (Langerhans) cells in various organs. Pulmonary involvement, although rare in children, has been reported in 20%–50% of childhood cases of multisystem LCH. Isolated pulmonary LCH in children, especially in infants, is still rarer, but should be suspected in those with cystic lung disease. We report a case of a 10-month-old boy who presented with chronic dyspnea and whose chest computed tomography (CT) scan demonstrated cystic lesions. Lung biopsy established the diagnosis of LCH; microscopy revealed a background of lymphocytes and eosinophils with kidney-shaped abnormal cells. These abnormal cells were positive for S-100, CD207 (Langerin), and CD1a on immunohistochemical staining. Chemotherapy was administered using a cytotoxic agent (vinblastine) and a steroid. After 12 weeks of induction chemotherapy, although no significant change in cyst size was noted on chest CT, clinical symptoms improved. Consolidation chemotherapy was then administered for 1 year. Thereafter, chest CT findings demonstrated a significant decrease in cyst size and a significant increase in the volume of normal lung parenchyma. Therefore, aggressive treatment of isolated pulmonary LCH in infants with severe tissue destruction and symptoms seems warranted.
Biopsy ; Child ; Consolidation Chemotherapy ; Diagnosis ; Drug Therapy ; Dyspnea ; Eosinophils ; Histiocytosis, Langerhans-Cell* ; Humans ; Induction Chemotherapy ; Infant* ; Lung ; Lung Diseases ; Lymphocytes ; Male ; Microscopy ; Thorax ; Tomography, X-Ray Computed

Background: Pulmonary Langerhans cell histiocytosis (PLCH) is an interstitial primary pulmonary disease, characterized by Langerhans cell proliferation. It is easily misdiagnosed in children. This study aimed to characterize the clinical manifestations and features of PLCH by retrospective analysis. Methods: A retrospective analysis was performed in 117 PLCH patients out of 338 LCH patients who were admitted in our center from November 2006 to October 2013. Variables between two groups were compared by Mann-Whitney U-test and Chi-square test. Kaplan-Meier curves were constructed to compare the survival rates and Cox regression to evaluate the effect of risk factors. Results: The median age of PLCH group was significantly lower than that of non-PLCH group (18.63 months vs. 43.4 months, P < 0.001). All PLCH children had other organ involvement and only 11 cases (9.4%) had respiratory symptoms. The most common radiologic finding was cystic lesions (29 cases, 24.8%). Pulmonary function abnormalities were dominated by obstructive ventilatory dysfunction (63 cases, 82.9%). The 5-year overall survival (OS) of PLCH children was 93.6% ± 2.3% and the event-free survival (EFS) was 55.7% ± 5.2%. Among the 38 cases with progressed or relapsed disease, five cases (13.2%) were due to progression or recurrence of lung damage. The 5-year OS of PLCH children with "risk organ" involvement was significantly lower than those without "risk organ" involvement (86.0% ± 4.9% vs. 100%, χ = 8.793, P = 0.003). The difference of EFS between two groups was also significant (43.7% ± 7.7% vs. 66.3% ± 6.5%, χ = 5.399, P = 0.020). The "risk organ" involvement had a significant impact on survival (hazard ratio = 1.9, P = 0.039). Conclusions PLCH mainly occurs in young children, and only a small percentage of patients have respiratory symptoms. They generally have other organ involvement. Most of PLCH children have a good prognosis and most lung lesions could have improved or stabilized. Management of "risk organ" involvement is the key point to improving EFS.
Child ; Child, Preschool ; Female ; Histiocytosis, Langerhans-Cell ; complications ; diagnosis ; Humans ; Infant ; Langerhans Cells ; Lung ; physiopathology ; Lung Diseases ; etiology ; Male ; Retrospective Studies