Diagnosis Of Langerhans Cell Histiocytosis Research Articles

Langerhans cell histiocytosis of thyroid-a diagnostic dilemma.

Langerhans cell histiocytosis (LCH) is a rare disease of antigen presenting cells and involvement of thyroid is really uncommon. The thyroid if involved is usually seen in multisystem disease but isolated thyroid involvement is very rare. The diagnosis of Langerhans cell histiocytosis of the thyroid is very challenging due to its rarity and is usually misdiagnosed as benign goiters, undifferentiated carcinoma, lymphoma, etc. Management of Langerhans cell histiocytosis of the thyroid also remains controversial. Prognosis in an isolated Langerhans cell histiocytosis of the thyroid is usually good but as it may precede a multisystem involvement, prolonged follow-up is required. We present a rare case of Langerhans cell histiocytosis of the thyroid, with variable diagnoses on fine needle aspiration cytology.

Cutaneous Langerhans cell histiocytosis in elderly with chronic myelomonocytic leukemia

Langerhans cell histiocytosis (LCH) is a rare histiocytic neoplasm characterized by clonal proliferation of Langerhans cells in multi-organ systems including skin, bone, pituitary gland, liver and spleen. Skin-limited involvement of LCH usually indicates an indolent clinical course; however, in rare cases, LCH is accompanied by other myeloproliferative disorders, which may determine the prognosis. An 82-year old Japanese man presented with numerous asymptomatic facial papules clinically simulating rhinophyma. Although findings of histopathology and general examination including bone marrow biopsy led to the diagnosis of cutaneous LCH, he died from chronic myelomonocytic leukemia, which emerged 10 months after the initial diagnosis of LCH. The previously reported cases of LCH concomitant with other hematological disorders are also summarized and described compared with the present case.

Langerhans Cell Histiocytosis: Case Report With Cytogenetic Approach

Langerhans cell histiocytosis is a rare disease that usually affects children, without gender predilection. Boy, 3, was referred to the Oral Medicine service with granulomatous and ulcerous lesions in the hard palate and alveolar gingiva, as well as swollen areas on the head. Specimens obtained through incisional biopsies of the intraoral lesions underwent histopathological and immunohistochemical examination, yielding the diagnosis of Langerhans cell histiocytosis. Furthermore, classical cytogenetic analysis revealed karyotypic alteration 46,XY del(22)(q11.2). Treatment consisted of chemotherapy with the topoisomerase inhibitor Etoposide. At 1-year follow-up the patient evidenced a good response to the treatment. Current clinical prognosis is favorable.

Langerhans Cell Histiocytosis of the Clavicle in a 13-Year-Old Boy

Langerhans Cell Histiocytosis (LCH) is a rare neoplasm characterized by abnormal proliferation of histiocytic cells. In this case report, we describe a unique case of a 13-year-old boy who presented to the clinic with an insidious onset of mid-clavicular pain. The provisional radiologic diagnosis of Langerhans Cell Histiocytosis of the clavicle was confirmed by an incisional biopsy of the left mid-clavicle lesion. The patient's lesion was treated by curettage, bone grafting, and internal fixation, due to the presence of pathologic fracture. At the 2-year followup, the patient was asymptomatic, and the lesion showed complete resolution without recurrence. The case report highlights the characteristic features of Langerhans Cell Histiocytosis in an unusual location, the knowledge of which would help avoid delayed or missed diagnosis in the future.

Langerhans cell histiocytosis of the atlas in a female adult.

Langerhans cell histiocytosis (LCH) is an infrequent disorder complex. It is a disease of myeloid dendritic cells, lymphocytes, and macrophages mixed with eosinophils and neutrophils [1]. The accumulation of these cells causes the classic lytic bone lesions, skin rashes, lymphadenopathy, and organ dysfunction. Langerhaus cell histocytosis occurs at all ages from infancy up to senility with the peak incidence between 2 and 4 years of age. The male/female ratio is about 3.7: 1 [2]. We have recently treated a case of LCH of the atlas in a female adult, which may be the first case of atlas LCH in a female adult reported in the English literature. A 26-year-old woman complained of headache for 10 years, with aggravation for 3 months. No neurological deficits were observed, and no significant medical history or family history was reported. Physical examination demonstrated obvious restriction of the neck motion without neurological deficit or torticollis. Computed tomography (CT) examination confirmed a sharply osteolytic lesion of the right posterior lacerate foramen area. The atlas right lateral mass, anterior arch and squamous part of occipital bone had been eroded with attenuation. The gap between the atlas and dentate was enlarged. The tumor mass had a similar density as soft tissue such as muscles in a plain CT scan; however, after enhancement, the tumor mass density overall increased obviously, but not very uniformly. On magnetic resonsnce imaging MRI, the soft tissue mass was isointense on T1, but it enhanced homogeneously after contrast administration (Fig. 1A–D). The patient underwent surgical treatment. Under general anesthesia a grey-red tumor of approximately 3.5 × 5.5 × 6 cm was completely removed from the atlas. The tumor had a tough texture with clear border and abundant blood supply. Fig. 1 A) CT scan shows an osteolytic lesion of the right posterior lacerate foramen area, and the lesion's radiological density was similar to muscles nearby; B) after enhancement, the tumor density overall increased obviously, but not very uniformly; C) shows ... Histopathologically, it was proven to be LCH. At higher magnification, the proliferating cells had indented and folded or grooved nuclei resembling coffee beans. Multinuclear giant cells were frequently seen. Eosinophils and neutrophils were admixed with the proliferating cells. The main cells were positive for S-100 protein (Fig. 1E and ​andFF). Skull and mandible are common regions of LCH involvement, and there have been very few published cases describing Langerhans cell histiocytosis of the atlas. On radiological examination, sharply demarcated punch-out osteolytic lesions in bones are characteristic. Final LCH diagnosis is possible based only on histopathology. They are usually 10–15 µm in size with characteristically folded or multilobular nuclei with typical grooves. Langerhans cells are mixed with inflammatory cells, predominantly acidophilic granulocytes. A reliable immunological marker is S-100 protein, while CD1α is usually positive too. Treatment options include surgical excision and curettage of isolated or limited bone disease. External beam radiation with local doses ranging from 2.5 Gy to 120 Gy (usually 5–20 Gy) has also been used. Chemotherapy has been used with good success for disseminated or recalcitrant disease. Overall prognosis is good with survival rates greater than 90% in patients having limited organ involvement [3]. In our case, the delitescence of the course of disease was more than 10 years, and it showed a slow process of growth of LCH and a benign nature. After one year of follow-up, there is no evidence of recurrence.

Eosinophilic granuloma of skull in a 7 year child: Diagnosed on fine needle aspiration cytology

Eosinophilic granuloma is a rare bone tumor, representing less than 1% of all bone tumors. Eosinophilic granuloma is the benign form of three clinical variants of Langerhans cell histiocytosis (LCH). It commonly affects children and young adults, particularly males. The characteristic cytologic features of eosinophilic granuloma include langerhans cells in a characteristic milieu which includes histiocytes, eosinophils, neutrophils and lymphocytes. We are reporting a case of eosinophilic granuloma of skull in a 7 years child diagnosed on fine needle aspiration cytology (FNAC). We here by emphasize the importance of rapid and reliable diagnosis of LCH by FNAC and the need to consider the possibility of LCH in the differential diagnosis of a slide showing a large number of histiocytes and eosinophils.

Rare, Isolated, Langerhans Cell Histiocytosis in a Colonic Polyp

Purpose: Langerhans cell histiocytosis (LCH) is a rare disorder of unknown pathogenesis. It is characterized by abnormal proliferation and dissemination of Langerhans cells, which are derived from the bone marrow. The disease most commonly affects the bones and the skin. Involvement of the GI tract is extremely rare. We report a case of isolated LCH in a colonic polyp. A 49-year-old woman presented with complaints of constipation, abdominal bloating, and mild change in bowel habits. She denied abdominal pain, early satiety, or weight loss. Family history was positive for colon cancer in her mother and maternal aunt. Physical exam was non-revealing. She underwent a diagnostic colonoscopy, which showed three diminutive polyps at the splenic flexure. These were removed by cold biopsy polypectomy. The rest of the colon exam was unremarkable. On histology, one of the polyps showed an ovoid collection of atypical cells within the submucosa. The cells were intermediate in size with eosinophilic cytoplasm and ovoid nuclei. The nuclei were convoluted. No cytoplasmic inclusions were identified. The atypical cells extended into the lamina propria. Immunohistochemical staining showed the cells to be strongly positive for CD1a and S100, which confirmed the diagnosis of LCH. The other polyps were hyperplastic. Subsequent extensive clinical work-up showed no evidence of disseminated LCH. Only mild eosinophilia (10.3% eosinophils) was evident. Liver function tests and urinalysis were normal. Chest x-ray showed no acute disease. CT chest showed no pulmonary lesions suggestive of Langerhans cell histiocytosis. There was no mediastinal lymphadenopathy. Review of published literature shows only a handful of cases of colonic involvement with LCH in adults. Most patients are either asymptomatic or have nonspecific GI symptoms. Polyps have usually been found incidentally on screening colonoscopy. The polyps are reported as small (less than 1 cm) and solitary, as in our patient. Only two patients have been reported to have progressed to multisystem disease with skin involvement.Figure: Langerhans cell histiocytosis in a colonic polyp.

VEGF and p53 Biomarkers in Pediatric Patients With Langerhans Cell Histiocytosis

Langerhans cell histiocytosis (LCH) is a rare disease with abnormal accumulation of the dendritic Langerhans cells. In the localized form (single system), the disease is self-limiting but in the cases of multisystem disease, one third of the patients develop organ dysfunction with poor prognosis. The aim of this study was to examine the role of p53 and vascular endothelial growth factor (VEGF) in the pathogenesis of LCH and look for association of them with the extent of the disease. Biopsy specimens obtained from 26 patients with definitive diagnosis of LCH were stained immunohistochemically for p53 and VEGF. There were 13 male and 13 female cases. The mean age of patients at presentation was 41.9 months (range, 2 mo to 18 y). Multisystem disease was presented by 61% of the patients (8 boys and 8 girls). Patients with multisystem disease were on average older than those with single system disease. p53 protein could be detected in 92% of cases and 61.5% of patients expressed VEGF, mostly from multisystem group. These findings highlight the role of angiogenic factors in the clinical behavior of LCH and might be of prognostic or therapeutic importance. However, further studies, with larger sample sizes are warranted.

Surgical treatment based on pedicle screw instrumentation for thoracic or lumbar spinal Langerhans cell histiocytosis complicated with neurologic deficit in children

Background contextSurgical indications and procedures for spinal Langerhans cell histiocytosis (LCH) in children are still controversial. Reports containing large samples of surgically treated patients are few in the currently available literature, and the reported operative procedures were also somewhat obsolete. So, further investigation based on large-sample cases and using improved surgical techniques is beneficial and helpful to refine the treatment strategy. PurposeTo recommend a reasonable treatment strategy for thoracic or lumbar spine LCH in children complicated with neurologic deficit. Study design/settingRetrospective/academic medical center. Patient sampleTwelve children aged from 2 to 16 years old with the diagnosis of thoracic or lumbar spinal LCH accompanied by neurologic deficit received surgical treatment from January 2005 to January 2010. Outcome measuresFrankel scale for neurologic function, fusion of the mass, and recurrence of the lesion. MethodsAll 12 patients presented initially with local pain and progressive neurologic detriment. Neurologic evaluation revealed two patients with Frankel Grade B, eight with Grade C, and two with Grade D. Radiographic features were positive for typical vertebra plana, a space-occupying mass in the spinal canal compressing neural elements, and a spinal canal encroachment rate more than 50%. Posterior instrumentation with pedicle screw combined with anterior corpectomy, decompression, and support bone graft was performed in the first seven patients as a one-stage procedure. In the remaining five patients, posterior pedicle screw fixation, laminectomy for decompression (via excision of the tumor-like mass), and repair of laminae with allograft bone block were performed. The collapsed vertebral body was left untouched. No chemotherapy or radiotherapy was administrated postoperatively in any of the cases. ResultsThe mean follow-up duration was 43.3 months. The mean operation time was 330 minutes with combined procedure and 142 minutes with single posterior approach (p=.000). The average blood loss was 933 mL with combined procedure and 497 mL with single posterior approach (p=.039). Three of seven patients who received combined surgery encountered approach-related complications, that is, one with intercostal neuralgia and two with pleural effusion. No severe neurologic deteriorate, instrumentation failure, or disease recurrence was detected at follow-up. Neurologic function completely recovered in all 12 patients from 2 to 12 weeks after surgery. The anterior bone graft fused and shaped well in all seven patients, and allograft bone block for lamina repair also achieved complete fusion in the remaining five patients. The internal fixator was removed at 3 to 5 years (average 4.1 years) after initial operation in six patients. No deformity, including scoliosis and kyphosis, has been identified during follow-up period in both procedures. ConclusionsFor spinal LCH patients, neurologic deficit is a main indication for operative treatment to prevent permanent and serious consequences. Surgery provides an opportunity for rapid recovery of neurologic function. Both combined and single-stage posterior approaches based on pedicle screw instrumentation techniques are similarly effective in relieving neurologic compression. However, single-stage posterior approach is more favorable with less complications, and preserving involved vertebral body is not a latent hazard of recurrence.

A Rare Case of Langerhans Cell Histiocytosis of the Skull in an Adult: a Systematic Review

We report a 41-year old male who presented to the Emergency Department after falling while water-skiing. He had a previous medical history included chronic headaches, which had persisted for the last 2-3 months prior to presentation. Computed tomography of the head showed a small hypersensitivity with a small extra axial collection with a maximum thickness of 1mm. Differential diagnoses included an arachnoid cyst, haemangioma, meningioma or a secondary lesion. A diagnosis of Langerhans Cell Histiocytosis was made based on the histopathology examination and the immunoperoxidase staining.

Thymus and mediastinal node involvement in childhood langerhans cell histiocytosis: Long‐term follow‐up from the French national cohort

BackgroundMediastinal involvement (MI) in Langerhans cell histiocytosis (LCH) has been rarely reported. Here, we describe the clinical, radiological, and biological presentation, and the outcome of childhood LCH with MI.MethodFrom the French LCH register, which includes 1,423 patients aged less than 18 years, we retrieved the medical charts of patients with mediastinal enlargement detected on chest X-rays.ResultsThirty-seven patients were retrieved, including 18 males; median age of diagnosis was 0.7 years, and median follow-up time was 6.2 years. The prevalence of MI varied with the age at diagnosis, ranging from 7% below 1 year old to less than 1% at >5 years. Thirteen cases (35%) were diagnosed because of MI-related symptoms, including respiratory distress (N = 4), superior venous cava syndrome (N = 2), and/or cough and polypnea (N = 10). CT scans performed in 32 cases at diagnosis showed tracheal compression (N = 5), cava thrombosis (N = 2), and/or calcification (N = 16). All patients presented multi-system disease at LCH diagnosis, and 35/37 were initially treated with vinblastine and corticosteroids. Death occurred in five cases, due to MI (N = 1) or hematological refractory involvement (N = 4). The overall 5-year survival was 87.1%, and immunodeficiency was not detected as a sequel.ConclusionsMI in LCH mainly occurs in young children, and diagnosis was based on CT showing thymus enlargement and calcifications. Pediatr Blood Cancer 2013;60:1759–1765. © 2013 Wiley Periodicals, Inc.

Approach to the patient with Langerhans cell histiocytosis manifested as central diabetes insipidus and cervical lesions

[Summary] A 19-year-old male patient with central diabetes insipidus complained of polydipsia and polyuria and was found to be accompanied by cervical vertebra lesions.The diagnosis of Langerhans cell histiocytosis was confirmed by laboratory and pathological results.Therefore,it should be alert to consider the possibility of Langerhans cell histiocytosis in a patient with central diabetes insipidus coexisting with the signs of multisystem lesions such as bone disease. Key words: Central diabetes insipidus; Cervical vertebra lesions; Langerhans cell histiocytosis

Histiocytose langerhansienne : place actuelle de l’imagerie

Langerhans cell histiocytosis (LCH) is a rare disease of the reticuloendothelial system which consists in various clinical manifestations from a single lytic bone lesion to multisystemic lesions with organ dysfunction. One single bone lesion, the most frequent presentation, has a good prognosis. We relate here the case of a 4-year-old child who had a pain of his right hip with lameness and fever. The bone scintigraphy (BS) highlighted an increased uptake of the right iliac wing. Biopsy of the lesion led to the diagnosis of LCH. The progression was spontaneously favourable with relief of pain after the biopsy. This case report allows us to highlight the most recent guidelines regarding the classification of the disease and the indication of imaging. Staging is currently based on chest radiograph, abdominal ultrasound and skeletal radiograph survey. The role of BS, MRI and FDG PET/CT for staging remains to be clarified.

Multiple Elevated Red Spots in a Woman With Nausea, Altered Stools, and Weight Loss

A69-year-old woman presented with nausea, diminished appetite, 9 kilograms of weight loss in 6 months, and altered stools. Her stools were loose without loss of blood or mucus. She had multiple skin lesions on the trunk, vulva, andvagina.Becauseof gastrointestinal complaints, ourpatient underwent a gastroduodenoscopy. Along the duodenum, multiple small mucosal nodules with subtle central ulcerationwere seen; in the stomach, somecomparable lesions were visible. Colonoscopy and videocapsuleendoscopy showed the same elevated light red lesions spread throughout the colon, most marked in the rectosigmoid, and along the entire small intestine (Figures A and B). On pathologic examination, a mixed infiltration with an accumulation of histiocytes with many neutrophils and a few eosinophils was seen (Figure C). Immunohistochemically, the cells stained strongly positive for CD1a (Figure D) and CD4, and weakly positive for S-100 and CD68. Histopathologic findings of the skin biopsy specimen showed an identical infiltration with histiocytes and neutrophils. The diagnosis of Langerhans cell histiocytosis (LCH) was made based on the spreading infiltration of histiocytes, immunohistochemically positive for CD1a and S100. LCH is a rare disease, seen in both children and adults. It affects various organs and has a widespread pattern of signs and symptoms. In adults, the estimated incidence is 1 or 2 cases per million. The etiology of LCH still is unknown. The Langerhans cells are believed to originate from hematopoietic stem cells, which express CD1a and/or S100. Because the literature reports fewer then 20 adult cases, it is assumed that gastrointestinal LCH is underestimated. Notably, most reported adult cases present as solitary polyps. In adults, skin lesions associated with extensive involvement of the small and large intestine is seldom seen. Our patient was treated with prednisolone and vinblastine according to the LCH-A1 multicentre study program, complicated by polyneuropathy. Because there was multi-organ involvement, the patient was maintained on therapy with daily 6-mercaptopurine and prednisolone for 5 days once every 3 weeks. Three months later, her symptoms improved, and the skin and intestinal lesions diminished. However, complete remission was not achieved. In conclusion, we report an interesting case of LCH in an adult with involvement of the skin and intestinal tract that was responsive to prednisolone, vinblastine, and 6mercaptopurine.

Langerhans Cell Histiocytosis: a Case Report

Abstract Langerhans cell histiocytosis is a disease which results from accumulation or proliferation of a clonal population of cells with the phenotype of Langerhans cells arrested at an early stage of activation that are functionally deficient. The etiology and pathogenesis of the disorder are still unknown. There are ongoing investigations to determine whether it is a reactive or a neoplastic disease. The fact is that neoplastic and reactive processes may have many clinical and pathological similarities. Some emphasize the role of “cytokine storm” in Langerhans cells. Further studies are necessary in all areas, from the etiology and pathogenesis to diagnosis and therapy. Langerhans cell histiocytosis primarily affects bones, but less commonly it may involve other organ systems, or present as a multisystem disease. The clinical course is variable, from benign forms with spontaneous resolution, to chronic disseminated forms with fatal outcome. This is a report of a 29-year-old man with Langerhans cell histiocytosis with an onset at the age of 8, which later progressed to a multisystem disease. Apart from lesions on the skin and exposed mucous membranes, the patient also presented with: diabetes insipidus, granuloma of the right femur and slight bulbar protrusion of the right eye. The patient experienced spontaneous pneumothorax on two occasions. The diagnosis of Langerhans cell histiocytosis was histologically confirmed using electron microscopy by presence of Birbeck granules in the histiocytes. A favorable therapeutic response was obtained after systemic corticosteroid therapy.

Diabetes Insipidus and Sclerosing Cholangitis in a Child May Be a Clue to the Diagnosis of Langerhans' Cell Histiocytosis : A Case Report

Langerhans cell histiocytosis (LCH) is a rare disease that usually affects children and young adults. Sclerosing cholangitis (SC) can occur in 10-15% of patients with disseminated form of the disease. Central diabetes insipidus (CDI) is a rare disorder that may be caused by a variety of diseases mainly LCH and germinoma especially in children. In this case report, a- 4-year-old girl who is a known case of CDI and a single bone lesion in the left humerus, presented with jaundice, abdominal distention and itching. The diagnosis of SC was made by histopathology on liver biopsy. In this case, we found a link between CDI and SC through LCH, the diagnosis of which was made by histopathology of the explanted liver. The combination of CDI, liver involvement with SC and a single bone lesion is remarkable, since the histological diagnosis of LCH was made outside the biliary tract in the liver parenchyma.

Langerhans Cell Histiocytosis Presenting as Uncontrolled Asthma

Langerhans cell histiocytosis (LCH) is an uncommon disorder affecting primarily young adult smokers. It is characterized by abnormal proliferation of Langerhans cells, specialized monocyte-macrophage lineage antigen-presenting cells. LCH can affect the lungs in isolation or as part of a systemic disease. Most commonly, the disease presents in the third or fourth decade without gender predominance. Symptoms typically include dyspnea and cough. Commonly, physical examination is unremarkable but cor pulmonale may be observed in advanced disease. The chest radiograph is typically abnormal with nodular or interstitial infiltrates and cystic changes. High-resolution computed tomography of the chest with these findings in the middle and upper lobes of an adult smoker is virtually diagnostic of LCH. Pulmonary function assessment is variable. Asthma has rarely been reported in association with this disorder. There are only three reported cases of the diagnosis of concomitant asthma which have been made in association with the diagnosis of LCH. We present a case in which our patient presented with signs and symptoms of asthma to include confirmatory findings of airway hyperresponsiveness. The diagnosis of LCH was established after the patient failed to respond to conventional treatment for asthma, and further evaluation was completed.

Cytological diagnosis of Langerhans cell histiocytosis with cutaneous involvement

Langerhans cell histiocytosis (LCH) is a rare disease affecting predominantly children. The course of the disease varies, from spontaneous resolution to a progressive multisystem disorder with organ dysfunction and potential life-threatening complications. Diagnosis of LCH is often difficult and may be delayed because of its rarity and especially so if it occurs with unusual presentation. Fine needle aspiration cytology of a 4 year old male child, a case of LCH is presented with a purpose of highlighting the characteristic cytological features. A high index of suspicion, awareness of characteristic cytological features of LCH and its differential diagnoses is necessary. This can obviate the need of biopsy and electron microscopy. Immunohistochemistry if available can be performed on cytology smear and cell block.

Primary Intramedullary Langerhans Cell Histiocytosis of the Thoracic Spinal Cord

A 28-year-old male presented with a rare case of primary intramedullary spinal Langerhans cell histiocytosis (LCH) manifesting as the chief complaint of a 6-month history of gait disturbance and back pain, and difficulty with sphincter control. Serial T2-weighted magnetic resonance imaging of the thoracic spine revealed enlargement and intramedullary hyperintensity of the spinal cord at T2 to T4. Biopsy of the lesion was performed. Histological examination of the biopsy specimens verified vascular proliferation and remarkable infiltration of histiocytes that were positive for CD1a, suggesting a diagnosis compatible with LCH. The patient was treated successfully by steroid pulse therapy. LCH is a rare disease that occurs mainly in children and may cause a broad range of manifestations, from a single osseous lesion to multiple lesions involving more than one organ or system. The present case illustrates the unexpected occurrence and important differential diagnosis of primary intramedullary spinal LCH of the thoracic spine in adult patients presenting with progressive paraparesis and back pain.

A Case of Langerhans Cell Histiocytosis Presenting with Bilateral Otorrhea

Langerhans cell histiocytosis (LCH) involves the proliferation and accumulation of Langerhans cells at various sites and presents with various symptoms, depending on the organ or organs invaded. A 2-year-old boy with LCH was treated at our hospital. Fever, head rash, bilateral chronic otorrhea unresponsive to antibiotics, and left exophthalmos were observed at our first examination. The CT scan showed destruction of the skull bone, zygomatic bone and vertebrae, and masses within these lesions. No bony defect was detectable in the middle ear, but a mass was present in the external auditory meatus. Skull X-rays revealed characteristic “punched-out” lesions. We confirmed the diagnosis of LCH with a biopsy of the head mass and the external auditory meatus mass. Immunohistology revealed positive staining of the lesional cells with CD1a and Langerin (CD207). The final diagnosis was multisystem LCH (MS-LCH). The child was placed on the chemotherapeutic regimen recommended by the Histiocyte Society.