Langerhans Cell Histiocytosis Presenting Research Articles

Langerhans cell histiocytosis presenting as isolated central diabetes insipidus in a 2-year-old child: a rare manifestation of rare disease

Central diabetes insipidus, though uncommon in children, has varied causes, the commonest ones being genetic mutations, infiltrative disorders, infections. Isolated central diabetes insipidus is not one of the often encountered conditions in the pediatric practice. Here we report a case of 2 ½ years old female child who presented to us following history of polyuria for 2 months, who was confirmed to have central diabetes insipidus which was later evaluated to be secondary to Langerhans cell histiocytosis. Magnetic resonance imaging (MRI) and histopathological studies further helped in confirmation of the diagnosis. Langerhans cell histiocytosis is a rare, multifarious, and underdiagnosed hematologic disease in which isolated diabetes insipidus can be the sole presenting feature before other manifestations. Hence, this diagnosis could strongly be considered in the work up of central diabetes insipidus in children.

Langerhans cell histiocytosis presenting as eosinophilic granuloma of the bilateral forearms in an 8-year-old girl: a case report

BackgroundLangerhans cell histiocytosis previously known as histiocytosis X is a rare disease of children and young adults with a very broad clinical spectrum. In children, its annual incidence is estimated between 0.2–0.5 per 100,000.Case representationAn 8-year-old Moroccan girl with no known personal or family history presented to our institution with painful swelling of both forearms. An X-ray and magnetic resonance imaging were inconclusive. We then performed a biopsy curettage (of her left forearm). Microscopic analysis followed by immunohistochemical analysis disclosed a diagnosis of Langerhans cell histiocytosis. No chemotherapy was necessary. Clinical and radiological improvement was achieved after 6 months.ConclusionThe particularity of this observation is the bilaterality of the lesion on both forearms and it has not previously been reported. Langerhans cell histiocytosis should be included in the differential diagnosis of osteomyelitis and Ewing’s sarcoma.

A 5 Years Retrospective Study of Various Presentation of Langerhans Cell Histiocytosis in Children : In A Tertiary Care Hospital

A 5 Years Retrospective Study of Various Presentation of Langerhans Cell Histiocytosis in Children : In A Tertiary Care Hospital

High-Risk Langerhans Cell Histiocytosis in Infants Exhibits Malignant Features in a Xenograft Model but Responds Durably to Targeted Therapy

Background: Langerhans cell histiocytosis (LCH) is a rare hematologic neoplasm primarily of childhood, characterized by a neoplastic proliferation of Langerhans-like cells. The clinical presentation of LCH is highly variable, and while some children are cured with combination chemotherapy, many will relapse and experience irreversible morbidity. Genomic profiling of LCH has identified recurrent somatic activating mutations in BRAF and MAP2K1, all of which culminate in activation of the mitogen-activated protein kinase pathway. However, key mechanistic and clinical questions such as the curative potential of targeted therapy and the cell of origin remain unanswered.Methods: Four infants with risk-organ positive (RO+) multisystem BRAF V600E-mutant LCH and secondary hemophagocytosis were treated at our institution with the BRAF V600E-inhibitor dabrafenib after failing chemotherapy, or up front at the discretion of the treating physician. Response assessment was performed as indicated for good clinical care. Excess bone marrow obtained during routine clinical evaluation was collected under an IRB-approved protocol, and transplanted into busulfan-conditioned NOD/LtSz-SCID interleukin-2(IL2)RG-/- (NSG) or NSG-SGM3 (NSGS) mice. Donor chimerism was assessed by flow cytometry for mouse and human CD45 and lineage reconstitution was determined with standard human markers. BRAF V600E detection was performed on extracted DNA from xenografts using a TaqMan Mutation Detection RTPCR assay.Results: At presentation, all patients exhibited features of HLH (hemophagocytic lymphohistiocytosis) with fever, splenomegaly, pancytopenia, elevated markers of inflammation, hypofibrinogenemia, and hemophagocytosis on bone marrow examination. All patients displayed marked and rapid improvement following initiation of dabrafenib. With a median follow up of 21 months, all patients remain in complete clinical remission, and are thriving. None of the patients experienced significant adverse effects classically associated with dabrafenib. In two patients, despite clinical disease resolution, BRAF V600E was still detectable in bone marrow by RTPCR at 23 and 26 months respectively post initiation of dabrafenib. Remarkably, the other two patients who were treated upfront with dabrafenib achieved molecular remissions (defined as undetectable BRAF V600E allele in a previously positive patient) after 11 and 12 months respectively of therapy.Among mice transplanted with patient bone marrow cells, the median survival was 9.6 weeks. A comparison arm consisting of mice transplanted with bone marrow from a low-risk LCH patient did not develop evidence of disease during the observation period. At necropsy, animals exhibited splenomegaly, thrombocytopenia and anemia. Flow cytometry of both bone marrow and spleens from mice demonstrated robust engraftment of human hematopoietic cells, and the BRAF V600E allele was detected from marrow and splenocytes in all mice. Frequent hemophagocytic forms were visible on bone marrow cytospins, and on histologic examination, bone marrow exhibited an abundance of CD1a-negative, CD163+ histiocytes, similar to findings observed in human disease.Bone marrow and splenocytes from primary NSG xenograft recipients were transplanted into secondary NSGS mice, and two out of three animals examined similarly became ill with comparable disease latency, and achieved engraftment of human cells with detectable BRAF V600E in bone marrow and splenocytes.Conclusions: For patients with LCH with hematologic involvement and secondary HLH, disease-initiating cells are present in the bone marrow and can engraft and recapitulate disease following primary and secondary transplant into immunodeficient mice. Targeted therapy with the BRAF V600E-specific inhibitor dabrafenib leads to durable, sustained remissions. Furthermore, we demonstrate that certain patients can achieve molecular remission with BRAF V600E-specific monotherapy alone. However, in at least a subset of patients with RO+ LCH, the persistence of mutated BRAF despite prolonged targeted therapy suggests that while quiescent, transformed disease-initiating cells are not eliminated by BRAF inhibition alone. Based on these results, we hypothesize that first-line therapy with BRAF inhibitors may be more likely to induce molecular remissions. DisclosuresNo relevant conflicts of interest to declare.

Treatment Modality Choices and Outcome in Langerhans Cell Histiocytosis: Radiation Therapy Versus Other Modalities

Langerhans cell histiocytosis (LCH) is a rare disease characterized by histiocytic proliferation with varied clinical presentations in both children and adults. Although treatment guidelines exist for systemic therapy, there is limited evidence to guide selection of treatment modality (radiation, systemic therapy, excision, or observation) among the many presentations of LCH, particularly in limited, single-system LCH involvement. Here we report treatment modality selection and outcome in a large single-institution database of this rare disease, and the role of radiation therapy (RT) in the treatment of single-system LCH. We hypothesize that RT provides good local control in single-system LCH disease. We identified 184 patients with histologically confirmed LCH who presented to our center from 2000-2015. Clinical and treatment characteristics were examined, and disease was classified by single or multisystem organ LCH involvement. The pediatric group was defined by age ≤18 years at time of LCH diagnosis. Response assessment per lesion was assessed using the RECIST 1.1 and PERCIST 1.0 criteria. For patients with multiple lesions, treatment and response for each lesion was assessed individually. Adverse events were evaluated using CTCAE v4.0. Of 184 patients, 108 were adults and 76 children with median age 31 (0.1-89). The most common sites of LCH were as follows: adults: bone, lung, skin, and brain, and children: bone, brain, and skin. Physician choice of RT versus other modalities, primarily excision, was guided by proximity to critical structures, extent of disease, or patient preference. Of 42 adult bone lesions, 28 (67%) underwent excision, 10 (24%) RT, 3 (7%) observed, and 1 (2%) received systemic therapy. Of the 10 RT patients, none required additional therapy for LCH disease. Of 73 bone lesions in children, 32 (44%) underwent excision, 19 (26%) had systemic treatment, 15 (21%) received RT, and 7 (10%) were observed. No patients required additional therapy post-excision. Of the 15 RT patients, only 1 (7%) required a second-line treatment. Median age of RT patients was 35 years (1.5 – 67). The median RT dose was 11.4 Gy (7.5-50.4). There were no acute Grade 3 events and only 1 late Grade 3 event of chronic headache noted. Bone lesions are the most common site of LCH involvement in single-system disease in both pediatric and adult populations. Radiotherapy provides good local control with low likelihood of local failure and is well tolerated in the treatment of select LCH bone lesions for which resection is sub-optimal. Radiotherapy is a safe and effective modality that should be considered for local control of single-system LCH bone lesions among both adult and pediatric patients. Further characterization of the role of RT in site-specific single-system LCH may guide future management decisions.

A case report of orbital Langerhans cell histiocytosis presenting as a orbital cellulitis

Abstract Clinical case A 10-year-old girl was seen with a 3-week history of right upper lid swelling and with no other symptoms or fever. There was no recent history of sinusitis, trauma, or previous infection involving the periorbital area, or response to oral antibiotic treatment. Orbital computed tomography showed a lesion involving the upper margin of the orbit, and bone destruction at the orbital roof. Biopsy performed revealed the presence of Langerhans cell histiocytosis. The lesion was surgically debulked and corticosteroids were used intra-operatively. The lesion responded to treatment. Discussion The orbital involvement of Langerhans cell histiocytosis, despite its low incidence, should be considered in the examination of acute peri-orbital swelling. It usually presents as an osteolytic lesion, and it is confirmed with a histological examination and immunohistochemical techniques for CD1a and S100. An interdisciplinary approach is recommended to rule out multifocal or multisystemic diseases, as well as to develop an appropriate treatment strategy.

Pediatric Langerhans cell histiocytosis of the temporal bone: clinical and imaging studies of 27 cases

BackgroundWe aimed to evaluate the clinical and imaging presentations of Langerhans cell histiocytosis (LCH) in the pediatric temporal bone.MethodsThis retrospective study included 27 pediatric cases with pathological confirmed LCH of the temporal bone. The clinical and imaging features of the cases were analyzed. The involvement of ossicular chain and otic capsule was also evaluated.ResultsA total of 38 lesions (27 cases) with 11 bilateral involvement were identified. For the 27 cases, the most common complaint was periauricular swelling (12/27, 44.4%), followed by otorrhea (9/27, 33.3%) and otalgia (5/27, 18.2%). The mastoid process was the most common involved subsite (31/38, 81.6%) among the 38 lesions. Ten (26.3%, 10/38) lesions belonged to the group of the diffuse involvement, 22 (57.9%, 22/38) were divided into the group of partial involvement and six (15.8%,6/38) localized lesions with punched-out appearance. Erosion of ossicular chains and otic capsule were found in three and seven lesions respectively.ConclusionThe results indicate that the most common subsite for LCH of the pediatric temporal bone was the mastoid process. The location and extent of pediatric LCH of the temporal bone varied a lot between each other. The ossicular chains usually remain intact and the erosion of otic capsule can occur in some lesions.

A rare case of langerhans cell histiocytosis presenting in the gastrointestinal tract

We report a case of an 11-month-old boy who presented with gastrointestinal symptoms associated with unexplained anaemia. Upper gastrointestinal biopsies confirmed the presence of a subtle infiltrate consistent with Langerhans cell histiocytosis (LCH).

Adult Langerhans cell histiocytosis with pulmonary and colorectoanal involvement: a case report

BackgroundLangerhans cell histiocytosis is a rare systemic disease characterized by the abnormal overproduction of histiocytes that tend to infiltrate single or multiple organ systems leading to significant tissue damage. It mainly affects – by order of decreasing frequency – the bone, the skin, the lymph nodes, the liver, and lungs. Gastrointestinal tract involvement is extremely rare in adults.Case presentationWe describe the case of a 32-year-old Middle Eastern man with Langerhans cell histiocytosis involving his lungs and the colorectoanal part of his gastrointestinal tract, with complete resolution of gastrointestinal tract lesions following a non-standardized chemotherapy regimen.ConclusionsGastrointestinal tract lesions are a rare manifestation of Langerhans cell histiocytosis, especially when associated with extraintestinal involvement, such as the lungs. Chemotherapy protocols have not been well established for the treatment of the disease. The clinical impact of the effective chemotherapy regimen used to treat this uncommon presentation of Langerhans cell histiocytosis will be viewed in this case report.

LANGERHANS CELL HISTIOCYTOSIS OF THE SCAPULA - DIAGNOSIS & TREATMENT OPTIONS

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.

Langerhans Cell Histiocytosis Mimicking Periapical Pathology in a 39-year-old Man

Langerhans cell histiocytosis (LCH) is a clonal neoplastic proliferation of Langerhans-type dendritic cells, with more than 50% of cases of LCH seen in children younger than 15 years of age. The most common clinical presentation of LCH is solitary or multiple bony lesions. The jaws are affected in approximately 10%–20% of cases, with a strong predilection for the mandible. The maxilla is involved in only 1% of head and neck cases. When the jaws are involved, lesions of LCH may mimic periapical pathology as seen in patients requiring endodontic therapy or bone loss as seen in periodontal disease. We report the case of a 39-year-old man with LCH involving the posterior maxilla. This is a rare presentation of LCH with respect to both location and patient age. Clinicians should consider LCH when developing a differential diagnosis of an apical radiolucency of vital teeth or teeth that fail to respond to endodontic therapy and be aware of its clinical and radiographic mimics.

Langerhans cell histiocytosis presenting as a blueberry muffin rash

Langerhans cell histiocytosis presenting as a blueberry muffin rash

Image Gallery: A rare abscess-like presentation of Langerhans cell histiocytosis

Image Gallery: A rare abscess-like presentation of Langerhans cell histiocytosis

Orbital cellulitis as the initial presentation of Langerhans cell histiocytosis in an adult patient

A 28-year-old woman presented with 3 weeks of right-sided blurry vision, proptosis, orbital pain, and upper eye lid swelling. She also complained of progressively worsening intermittent headaches. She had been evaluated in the emergency department multiple times for the same complaint, and had received multiple courses of intravenous antibiotics with minimal improvement. Physical examination revealed significant proptosis of the right eye although visual acuity was preserved in both eyes. Initial laboratory studies including complete blood count, basic metabolic panel, and thyroid functioning tests were within normal limits. An infectious workup including blood and fungal cultures were negative. A non-contrast computed tomography (CT) scan of the head demonstrated a mass on the right orbital rim which was concerning for right periorbital and orbital cellulitis, with the post septal component present along the superior and lateral aspects of the right orbit (Figure 1A). Broad spectrum antibiotics were initiated and a repeat magnetic resonance imaging scan performed ten days later showed partial improvement of the inflammatory changes associated with orbital cellulitis. However, there was an interval increase in the size of a more discrete mass-appearing lesion which was now eroding into the adjacent bony structures (Figure 1B). A biopsy of the lesion showed an infiltrate which stained strongly positive for CD1a and S100 (Figure 1C-F). A diagnosis of Langerhans cell histiocytosis (LCH) was considered. CT scans of the chest and abdomen-pelvis and a bone marrow biopsy did not show evidence of systemic LCH disease. She declined therapy and her prognosis is unknown. CNS imaging findings for Langerhans cell histiocytosis (LCH). A: CT head without contrast showed right periorbital edema with underlying, focal cortical destruction of the right superior orbital rim consistent with periorbital cellulitis. B: A MR of the orbits performed 2 weeks later showed an enlargement of the mass with invasion into the orbital rim. The mass is delineated by a yellow star. Subsequent pathology obtained from biopsy of the mass revealed Langerhans's histiocytosis (eosinophilic granuloma). C: Low power view; (E) High power view (cells are medium to large size, with folded/convoluted nuclei, fine chromatin, abundant eosinophilic cytoplasm in a background of eosinophils, neutrophils and small lymphocytes; (D) CD1a positive; (F) S100 positive LCH, previously known as histiocytosis X, describes a spectrum of proliferation of Langerhans cells that may manifest as a single system or multiple system disease. Although solitary ocular manifestations of LCH without evidence of systemic LCH are documented in children, they are rare in adults. The location of the infiltrate within the orbit largely determines the patient's presenting complaints.1 Most orbital manifestations of LCH in adults have been in the context of systemic LCH disease with only a handful of cases of solitary orbital LCH presenting as orbital cellulitis documented. The diagnosis is primarily determined by a biopsy of the presenting lesion, where immunohistochemical staining with S100 and CD1a is characteristic of Langerhans cells.2 Treatment strategies for orbital LCH depend on “case-by-case basis” and surgical resection and radiation is reserved for solitary lesions in children. Given its rarity, ideal management strategies are unclear, however solitary orbital LCH can spread to the central nervous system, and these patients can develop diabetes insipidus and systemic LCH. Aggressive prolonged multi-drug regimens including combinations of vincristine, prednisolone, etoposide, and methotrexate are used for treatment, with routine surveillance for recurrence with history, examination, and imaging. None. Not Applicable Obtained AD drafted and revised the manuscript. AG provided care for the patient and conceptualized the report. AG and HN revised and edited the manuscript for intellectual content.

Clinical Profile of Langerhand Cell Histiocytosis- Single Center Experience

Background/Objectives:Dubai hospital is one of the three main centers which carters for the management of children presenting with benign and malignant blood disorders in the United Arab Emirates. This study is to demonstrate the varied clinical profile of children with Langerhans cell histiocytosis (LCH) who presented to Dubai hospital since 2012.Methods/Design:A Prospective analysis of children with LCH treated at our center was done. Complete history, physical examination, relevant laboratory and radiological investigations were studied for all children suspicious to have LCH. Diagnosis was confirmed by obtaining tissue from biopsy of involved areas. Once histopathology confirmed the diagnosis, extent of disease was evaluated and chemotherapy started based on the LCH-IV protocol.Results:A total of 10 children presented to Dubai hospital with LCH from 2012 to 2016. The age at diagnosis ranged from 5 months to 5 years. Diagnosis was confirmed by positive CD1a and S100 immuno-markers of involved tissue (100%). These children had varied presentations like respiratory distress, recurrent chest infections, chronic diarrhea, abdominal pain, back pain, inability to walk, lymphadenopathy and buccal mucosa swelling. Median time of diagnosis once the children reached to our center was between 15 days to 1 month. Five children (50%) had multisystem LCH with risk organ involvement. Four children (40 %) had single system involvement and one child had single system multifocal involvement. Osteolytic lesions were present in six children (60%) leading to an early diagnosis.Good response to Induction I therapy (Prednisolone, Vinblastinefor 6 weeks) of Stratum I, LCH-IV protocol was seen in sevenchildren (70%). Two children were upgraded to Stratum II chemotherapy, LCH-IV protocol at the end of Induction II of stratum I chemotherapy (total 12 weeks of prednisone and Vinblastine) because of either poor response or progressive disease. One child was started on salvage chemotherapy, (Cladiribine, Cytarabine) during Induction therapy because of no response. There has been no mortality in our cohort.Conclusion:There should be high index of suspicion for LCH. One needs to be well acquainted to varied presentations of LCH for quickerdiagnosis. Induction therapy with Prednisolone and Vinblastine is a good prognostic marker for children with LCH in our study. It should still be considered the first line therapy for all children with LCH requiring chemotherapy because of lower toxicity profile and good response in the majority. Poor response to Initial Induction of 6 weeks with Vinblastine and Prednisolonewas salvageable with second line therapy in our cohort. DisclosuresNo relevant conflicts of interest to declare.

A Case of Neonatal Langerhans Cell Histiocytosis Presenting with Generalized Skin Involvement at Birth Evolving to Hepatic Involvement

A Case of Neonatal Langerhans Cell Histiocytosis Presenting with Generalized Skin Involvement at Birth Evolving to Hepatic Involvement

Langerhans Cell Histiocytosis with Atypical Intervertebral Disc and Sacroiliac Joint Involvement Mimicking Osteoarticular Tuberculosis in an Adult.

Langerhans cell histiocytosis (LCH), typically found in children, is a rare single or multisystem disorder with a wide range of clinical and radiological manifestations. Unusual presentations of LCH are occasionally encountered and it may be difficult to distinguish LCH from an infection or a benign or malignant tumor. A 35-year-old female presented with pain in her back and left buttock, malaise, and weight loss, with a duration of several months. Her laboratory test results were within the normal ranges except for the levels of acute phase reactants, which were elevated. Magnetic resonance imaging and computed tomography revealed a unilateral destructive sacroiliac lesion, and multiple vertebral lesions with adjacent discal involvement and extensive soft tissue extensions. She was initially misdiagnosed with multifocal osteoarticular tuberculosis. An open biopsy and joint curettage was performed. Histopathological examination showed that she had LCH. To the best of our knowledge, this is the first case of LCH associated with a destructive unilateral sacroiliac lesion, discal involvement, and involvement of the adjacent vertebrae, in an adult patient; the LCH mimicked osteoarticular tuberculosis. Disease onset in adulthood is rare, and this can potentially delay diagnosis. Familiarity with the imaging features of unusual LCH manifestations is necessary to ensure accurate diagnosis and appropriate treatment.

Langerhans cell histiocytosis presenting as worsening migraine. (P6.092)

Langerhans cell histiocytosis presenting as worsening migraine. (P6.092)

Multisystem langerhans cell histiocytosis with thymic involvement diagnosed with anterior mediastinal mass in a 2-month-old boy

Thymus gland involvement in Langerhans cell histiocytosis (LCH) is usually part of multi-system disease and may be more common than previously recognized. However, thymic involvement causing an anterior mediastinal mass is an extremely rare presentation of multisystem LCH. Here we report a 2-month-old-boy admitted to hospital with a giant anterior mediastinal mass with multisystem LCH involving the thymus, lungs, liver and skin. The differential diagnosis of mediastinal mass in children should also include LCH, especially multisystem disease. LCH should also be kept in mind in the differential diagnosis of skin lesions in infants, even if spontaneous regression occurs.

A Case of Adult Generalized Cutaneous Langerhans Cell Histiocytosis.

Dear Editor: Langerhans cell histiocytosis (LCH) is a rare disease and generally affects infants and children. Adult-onset form and generalized cutaneous lesions at presentation without extracutaneous involvement are uncommon. We report an unusual case of generalized adult cutaneous LCH without extracutaneous involvement. The patient was an 81-year-old man who complained of rapidly growing eruptions on the perianal area, groins, axillae, face, and ankles. He stated that the lesions had been present for about 3 months—they first appeared on the perianal area, and then extended rapidly to the groins, axillae, face, and ankles. Physical examination revealed disseminated violaceous papules and plaques on the perianal area, groins, axillae, face, and ankles; the lesions on the perianal area and groins were confluent and ulcerated (Fig. 1). He had no weight loss and no palpable lymphadenopathy or organomegaly. The laboratory results, including complete blood count with differential, liver, and renal function tests, were within the normal range. Bone marrow biopsy examination revealed no evidence of tumor involvement. In addition, a whole body positron emission tomography/computed tomography study showed no involvement of any organ. Histopathologic findings of the lesions showed diffused infiltration of large tumor cells in the dermis, and epidermotropism was appreciated. The tumor cells had a moderate amount of eosinophilic cytoplasm and grooved nuclei consistent with Langerhans cells. These cells were mixed with small lymphocytes, neutrophils, and eosinophils (Fig. 1). The immunohistochemistry staining showed that the tumor cells were diffusely positive for S-100, CD1a, langerin, leukocyte common antigen (LCA), CD4, and CD68, but negative for CD3, CD5, CD20, CD34, CD79a, human melanoma black (HMB)-45, and melan-A (Fig. 2). The Ki-67 proliferating index was approximately 60%. Taken together, these morphological and immunological data were consistent with a cutaneous LCH according to the World Health Organization diagnostic criteria. Fig. 1 (A~C) Multiple, erythematous, rust-colored papules and plaques with erosions distributed on the perianal area, groins, and axillae; (D~F) multiple, erythematous macules and papules on the face and ankle. Fig. 2 (A) Under low-power microscopic examination, the lesion shows diffused infiltration of tumor cells in the dermis (H&E, ×25). (B) The lesion shows infiltration of large tumor cells in the dermis, and epidermotropism was appreciated (H&E, ... After diagnosis of LCH, the patient was referred to an oncologist and received chemotherapy. However, the patient died of myocardial infarction 10 days after being diagnosed with the disease. LCH is a clonal proliferative disorder of Langerhans cells, typically seen in infants and children. Occurrences of LCH in the adult population are rare, but even rarer with the disease limited to the skin1. Compared with LCH in infants and children, adult LCH occurs predominantly in bone and presents mainly as single system disease, with a more chronic clinical course and a better prognosis. The clinical presentation of cutaneous LCH in adults is variable. It can present as papules, plaques, or nodules localized to a single anatomic site or in a generalized distribution. Pruritic papules or nodules of cutaneous LCH may appear similar to prurigo nodularis, and other (non-LCH) papules or ulcers of the external genitalia1,2. Sites most frequently involved include the scalp, trunk, flexural and intertriginous areas, glabrous skin, and external genitalia1. Treatment of cutaneous LCH is controversial. Several therapeutic approaches have been reported in the literature, according to the extent of disease: topical and systemic corticosteroids, topical imiquimod, psoralen plus ultraviolet A (PUVA), thalidomide, interferon-α, vinblastine, and surgery have been used for treating this disease3,4,5.