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Histiocytosis is a set of distinct proliferative illnesses defined by the proliferation and infiltration of varied numbers of dendritic cells, macrophages, and monocytes in the afflicted tissues. The skin and other organs may be impacted by the inflammatory infiltration. It can occur at any age. The severity of the symptoms can range from mild to severe, depending on the degree and type of organ involvement. Although certain forms of histiocytosis can be fatal, others can be treated successfully without sequelae. Langerhans cell histiocytosis manifests itself clinically in both children and adults. A combination of clinical, histological, and radiological tests is required to achieve a diagnosis. A severe, multisystemic, acute form of Langerhans cell histiocytosis is called Letterer-Siewe illness, which usually affects infants in their first year of life. In this article, we provide a brief literature review and a case study of a 9-month-old girl who presented with recurring gastrointestinal problems as the first sign of Letterer-Siwe disease.

Langerhans cell histiocytosis is a rare clonal disease of monocyte-macrophage system characterized with uncontrolled proliferation and accumulation of immature dendritic cells. Acute disseminated form of this disease known as Letterer-Siwe disease is life threatening. Here, we report such a case who presented as multisystem inflammatory syndrome following COVID-19 infection.

Langerhans cell histiocytosis is a rare clonal disease of monocyte-macrophage system characterized with uncontrolled proliferation and accumulation of immature dendritic cells. Acute disseminated form of this disease known as Letterer-Siwe disease is life threatening. Here, we report such a case who presented as multisystem inflammatory syndrome following COVID-19 infection. [ABSTRACT FROM AUTHOR]

Background: Langerhans cell histiocytosis (LCH) is a rare disorder characterized by abnormal proliferation and infiltration of histiocytic cells. Methods: This review focused on the main aspects associated with LCH. Results: LCH can involve single or multiple organs and systems, with bone and skin being the most commonly affected sites. Regarding skeletal manifestations, the jawbones are involved in about 20%–30% of all cases. Such lesions may present as unilocular or multilocular images mainly affecting the posterior mandible. Oral soft tissue lesions may also occur, with the gingiva and hard palate being the most frequently affected sites. Conclusion: The diagnosis and management of LCH are challenging, requiring a multidisciplinary approach, with dentists playing a central role since oral manifestations can be the first sign of the condition. [ABSTRACT FROM AUTHOR]

Langerhans histiocytosis is the recent term used for the complex syndrome consisting of three sub types 1. Eosinophilic granuloma, 2. Hand-Schuller Christian disease and 3. Letterer-siwe disease. The clinical manifestations vary with each entity. Although rare, radiological findings present a typical pattern. Advanced Imaging adds to the plain film diagnosis. The Imaging findings are illustrated in detail.

Background: Letterer-Siwe Disease (LSD) is one of the variants of Langerhans cell histiocytosis (LCH), which is considered as a rare disease that affects many systems in the body; it is characterized by monoclonal migration and proliferation of specific dendritic cells. The disease affects the bones and skin primarily, but can involve other organs as well, or appear as a multi-system disease leading to different clinical manifestations and eventually death. Summary: The authors present a case report of LSD in a two-year-old child from western Sudan, Messeria tribe, who is presented with one and a half-month history of fever, cutaneous ulcers, purprae, scaly crusted scalp, and pallor. His full blood count showed very low Hb with marked reduction of platelets. TWBC was normal. US showed hepatosplenomegaly with lymphadenopathy. A suspicion of sickle cell anemia and leukemia was suggested. He received treatment in his area in the form of antibiotics, skin care, blood transfusion and platelets aggregate without improvement. Patient was referred to Khartoum for further investigations and management. On presentation, a diagnosis of histiocytosis x was suggested depending on the clinical presentation of a general ill health in a child with purpurae, skin ulcers, and a scaly crusted scalp. A skin biopsy, bone marrow aspirate, and a skull x-ray were requested. Bone marrow aspiration showed hyper cellular BM with marked hemophagocytosis. Patient was admitted in a pediatric ward for further general investigations and blood transfusion, but he passed few days later before starting chemotherapy. Usually this is the prognosis of this rare and fatal aggressive form of histiocytosis x. Conclusion: A sick child with fever, anemia, hepatosplenomegaly, scaly scalp, and skin lesions should be investigated for LSD. [ABSTRACT FROM AUTHOR]

Langerhans cell histiocytosis (LCH), a rare proliferative disorder of cells that share phenotypic characteristics with dermal Langerhans cells, has long been considered an "orphan," or neglected disease. Prior to its current designation, LCH was known as Hand-Schuller-Christian disease, Letterer-Siwe disease, or eosinophilic granuloma, which was combined under the umbrella designation of Histiocytosis X. The pathogenesis of LCH has remained unclear since the initial reports approximately 100 years earlier, and gene expression studies have demonstrated that these tumors do not originate from dermal Langerhans cells. It remains unclear whether LCH is reactive or neoplastic, although these tumors manifest a unique self-healing phenomenon that is rare among malignancies. The clinical presentation of LCH varies according to the involved organ/system, including common manifestations such as otitis media or pneumothorax or unusual manifestations such as diabetes insipidus. Accordingly, LCH cases are classified according to the number of involved organs/systems and sites, as well as the involvement of so-called "risk organs". This review aimed to highlight recent insights as a better understanding of LCH will lead to more effective therapies. [ABSTRACT FROM AUTHOR]

Introduction Langerhans cell histiocytosis (LCH) is a rare uni or multisystem disorder associated with extreme production of immunoreactive Langerhans cells. Although this disorder has been reported in all age groups, spinal involvement especially thoracolumbar spine is seldom reported in toddlers. Case presentation We present a one (1) year, four (4) months female child with a history of recurrent fever, irritability and severe anemia of one (1) month duration. CT-scan and MRI revealed a collapsed twelfth thoracic vertebra (T12) body height greater than 95% with a huge intradural soft tissue mass. T12 cortectomy via posterior thoracolumbar approach was used to decompress the soft tissue part followed by interbody fusion with titanium cage filled with autologous iliac crest bone graft, fixation using plates and screws. Conclusion We are of the view that surgical decompression of spinal LHC lesions with interbody fusion with titanium cage filled with autologous iliac crest bone graft and fixation using plates and screws is very crucial in relieving neurological deficits. However, the patient will need repeated surgeries as she advances with age in a timely manner to avert any neurological deficit that may occur. [ABSTRACT FROM AUTHOR]

Langerhans cell histiocytosis (LCH) is a rare idiopathic disorder that results in tissue destruction due to the intense and abnormal proliferation of histiocytes. It affects numerous organs, but isolated mandibular osteolysis has not been previously reported as an initial manifestation of this disease. This paper aims to describe an unusual presentation of LCH in the mandible of a 10-year-old girl initially assessed to have Gorham’s disease based on clinical and imaging findings. Computerized tomography (CT) scan and MRI showed extensive lysis and resorption of the mandible, with note of a contrast-enhancing soft-tissue mass in the right maxillary sinus. Incisional biopsy and histopathologic examination were performed, and the diagnosis of LCH was established and confirmed by S100 and CD1-a immunostaining positivity. The patient underwent chemotherapy with vinblastine and prednisone which eventually resulted in reconstitution of the patient’s mandible. LCH should be considered as a differential in a patient presenting with signs and symptoms of Gorham’s disease. [ABSTRACT FROM AUTHOR]

Introduction: Langerhans cell histiocytosis (LCH) is a rare condition, and even rare is cervical spine involvement. Case report: A 9-year-old girl had neck pain, neck tilt and left upper limb weakness, occasional fever and positive family history of tuberculosis. Imaging showed C5 vertebral body collapse with epidural and prevertebral soft tissue collection causing cord and nerve root compression. The patient underwent C5 corpectomy and fusion. Histopathological was suggestive of LCH. She underwent radiotherapy and was asymptomatic at 1-year follow-up. Conclusion: Despite the rarity of the condition, the possibility of LCH should be considered in such cases. When neurologic deficits are present, operative treatment should be considered. [ABSTRACT FROM AUTHOR]

Histiocytosis of Langerhans cells (class 1 histiocytosis) consists of a range of clinical manifestations, including bone eosinophilic granuloma, Hand-Schüller-Christian syndrome, and Letterer-Siwe disease. These syndromes represent a spectrum of severity and prognosis of an underlying disorder which is usually sporadic. This report describes three cases in one family, who developed the disease a few years after their brother was discovered to have histiocytosis. All three patients had the same clinical manifestations. They had hyperthermia, eczematic rash, swelling in skull, hand and foot. Radiological data included lytic areas in the skull and fourth metacarpal. Serology for Epstein-Barr infection was negative. Infiltration of abnormal Langerhans cell histiocytes was demonstrated upon bone biopsy. Chemotherapy was administered. One case (male) died after a year of chemotherapy. In another case (female) chemotherapy was unsuccessful, but T-cell suppressor (cyclosporin) induced remission. In the third case (female), chemotherapy was successful.

The histiocytoses are diseases caused by the proliferation of histiocytes in various organs, including the skin; they have a very variable clinical spectrum and prognosis ranging from an often fatal multisystem involvement to a self-healing single lesion in a single organ. The classification of histiocytosis, which is based on the origin cell and malignancy potential, divides them into Langerhans cell histiocytosis (Class I), non-Langerhans histiocytosis (Class II) and malignant histiocytosis (Class III). In each of these classes numerous clinical forms have been described, but these forms according to some Authors only represent different developmental stages of the same disease. Letterer-Siwe disease is the most frequent form among Class I histiocytoses and juvenile xanthogranuloma and Rosai-Dorfman disease are the most frequent forms among Class II Histiocytoses. In Class I histiocytoses the histologic examination is not able to distinguish between severe and mild forms; the observation of the skin lesions can help in this differentiation. [ABSTRACT FROM AUTHOR]

Brief Background: Langerhans cell histiocytosis (LCH) is a disease complex which includes eosinophilic granuloma, Lettere-Siwe diseaseand Hand-Schuller-Christian disease,1. LCH can present in a variety of ways from a spontaneously regressing solitary lesion of bone to a multisystem life-threatening disorder. In all cases, the unifying histological finding is proliferation of the Langerhans cell. Materials and Methods: A four year old girl reported to the OPD of department of pediatrics at PGIMS, Rohtak with a complaint of increased water intake with up to 20-25 glasses of water per day, corresponding increase in urinary output and loose teeth with swelling of gums. radiographs showed floating teeth appearance. Incisional biopsy of gingivae was performed with S-100 and CD-1a immunohistochemistry confirmed Langerhans cell histiocytes. Discussion: The definitive diagnosis of Langerhans cell histiocytosis complies of history, physical examination, cytology and histopathology and radiographic screening, and taking various differential diagnosis in consideration. Summary and Conclusions: Langerhans cell histiocytosis is a rare lytic disease of bone commonly affecting facial skeleton and correct diagnosis of the disease and its subtype is key to correct management. [ABSTRACT FROM AUTHOR]

Congenital Langerhans cell histiocytosis (LCH) (formerly called Letterer-Siwe disease) is characterized by a clonal proliferation of Langerhans cells occurring in children at birth and manifests typically with multifocal cutaneous lesions, hepatosplenomegaly, lymphadenopathy, pulmonary lesions, and destructive osteolytic bone lesions. We present a case of LCH involving multiple systems high-risk organs (LCH MS-RO+), in a 32-week stillborn from a 20-year-old G2A1. The fetus was mildly hydropic and pale. Apart from maceration, the skin showed multiple targetoid lesions over the face, trunk, and limbs. There was hepatosplenomegaly and a pale brain. The placenta was large and bulky. Despite severe autolysis, histological examination showed disseminated histiocytes with multinucleated giant cells in the skin, lungs, thymus, mesenteric lymph nodes, spleen, and brain. By immunohistochemistry, the histiocytes were positive for S100, CD1a, and Langerin (CD207), confirming the diagnosis of LCH. There was extramedullary hematopoiesis in the spleen, brain, and placenta. Targeted next-generation sequencing performed on thymic DNA did not show the BRAF p.V600E variant but did show the MAP2K1 p.F53_Q58delinsL. Infants with LCH pose a diagnostic challenge due to their heterogeneous presentations. Our case is unusual in that the newborn presented with severe multiorgan involvement including brain and intrauterine death. LCH is still poorly understood requiring further genetic and molecular studies.

Langerhans cell histiocytosis, disease that most commonly occurring in children, demonstrates a broad spectrum of clinical and neuroimaging abnormalities. This article presents the clinical case of histiocytosis, it's literature review - etiologic factors, radio logical CT and MRI findings, differential diagnoses. [ABSTRACT FROM AUTHOR]

Abstract: Langerhans’ cell histiocytosis is a collective term used to describe a group of enigmatic, proliferative disorders. The natural history of the disease varies from a slow, benign, localized, symptomatic bony or soft-tissue lesion, to a rapidly, progressive, widespread, multiple-organ disorder which is often fatal. Eosinophilic granuloma (EG) accounts for 60–70% of all cases of Langerhans’ cell histiocytosis and can present as solitary (50–75%) or multifocal defects in bone. It occasionally presents as a localized soft-tissue lesion. Multiple treatment options are available but the response is unpredictable. However, no studies show complete resolution following a conservative modality of treatment in the management of EG of condyle and ramus. We describe a case, treated with intralesional injections of triamcinolone acetonide (Tricort 40mg/ml, 6 injections at weekly intervals over 6 weeks), leading to complete resolution of the lesion, along with a brief mention of the review of literature. [Copyright &y& Elsevier]

Langerhans cell histiocytosis (LCH) may be a single-system disease or a multisystem disease with organ dysfunction. Cytotoxic drugs and corticosteroids, alone or in combination, are the treatments for multisystem LCH. We report the case of a young girl who had multiple lentigines localized in periflexural areas, arisen after chemotherapy for Letterer-Siwe disease with skin and bone involvement. The time of lesions onset induces to hypothesize that immune suppression caused by chemotherapy may favor melanocyte proliferation. We hypothesize that the unusual periflexural localization of the lentigines may be due to a greater concentration of melanocytic cells in periflexural areas or a greater concentration of the drugs in the folds due to vasodilation. [ABSTRACT FROM AUTHOR]

Background: Patients with Langerhans cell histiocytosis are at particularly high risk for central diabetes insipidus (CDI) due to hypothalamic-pituitary disease. Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the proliferation of specialized bone marrow-derived Langerhans cells and mature eosinophils. Three disease variants were included in this description, eosinophilic granuloma, Letterer-Siwe disease and Hand-Schuller-Christian syndrome. Case Reprort: We report a patient of Langerhans cell histiocytosis-solitary eosinophilic granuloma of the mandible that presented with diabetes insipitus after biopsy of mass. [ABSTRACT FROM AUTHOR]

Langerhans cell histiocytosis (LCH) is a rare clonal disorder of unknown etiology and characterized by the proliferation of dendritic cells. LCH most commonly involves the bone followed by the skin and the lymph nodes. Recently, only a few cases of LCH with predominant lymph node involvement have been diagnosed by fine-needle aspiration cytology (FNAC). A 2-year-old boy presented with generalized lymphadenopathy, fever, and cough. The patient had hepatosplenomegaly, anemia, and lytic lesions in the skull. FNAC from the largest submandibular lymph node showed features of LCH. The large cells of LCH showed positive immunostaining for S-100 protein on FNAC smears. Later, lymph node biopsy and immunohistochemistry against S-100 protein and CD1a confirmed the diagnosis of LCH. The patient was treated with chemotherapy and he is under regular follow-up. This case report highlights the importance of FNAC as a rapid and accurate investigation in the diagnosis of lymph node predominant LCH. [ABSTRACT FROM AUTHOR]

Solitary eosinophilic granuloma is a part of the spectrum of Langerhans cell histiocytoses (LCH) which are rare diseases with varied clinical presentations. They comprise of three major overlapping syndromes: solitary eosinophilic granuloma (unifocal disease), Hand-Schueller-Christian disease (multifocal, unisystem), and Letterer-Siwe disease (multifocal, multisystem). A case of solitary eosinophilic granuloma of the ulna is presented here for its relatively uncommon incidence and the difficulty in making an accurate diagnosis based on the radiological picture alone. We hereby, emphasize the importance of a quick and reliable diagnosis based on fine needle aspiration cytology, and the need to consider the possibility of LCH in the differential diagnoses of a slide showing a large number of histiocytes and eosinophils, as in tuberculosis, Hodgkin's disease, and other primary and metastatic bony lesions. Characteristic nuclear grooves and a large number of eosinophils help in clinching the cytological diagnosis. [ABSTRACT FROM AUTHOR]

Abstract??Langerhans cell histiocytosis (LCH) is a complex disease entity comprised of three distinct clinical syndromes that demonstrate indistinguishable histology. These syndromes are: eosinophilic granuloma, which is predominantly osseous or pulmonary; Hand-Sch?ller-Christian?s disease, which involves multiple organ systems and, most typically, the skull base; and Letterer-Siwe?s disease, the most severe disease manifestation, which typically involves the abdominal viscera. This article reviews our current understanding of Langerhans cell histiocytosis by discussing the history, histology, etiology, and treatment of the disease. It focuses on the radiographic findings and imaging modalities that are the most useful in disease diagnosis and management. [ABSTRACT FROM AUTHOR]

Abstract Langerhans cell histiocytosis (LCH) has diverse presentations which can bring it before all physicians regardless of specialty. A retrospective audit was undertaken at Westmead Hospital, Sydney, Australia, to ascertain the incidence, epidemiology and clinical features of patients with LCH over a 10-year period (1994–2004). A total of 12 patients was identified (six male, six female). Eleven patients had involvement of the skeletal system, three of the patients had pulmonary LCH and only one patient presented with soft tissue involvement (nose and antrum). Three patients had diabetes insipidus. Our results are consistent with that noted in the published literature and confirm the low incidence, diverse nature of presentation and the differing treatment strategies available for this rare and yet interesting condition. (Intern Med J 2005; 35: 622–625) [ABSTRACT FROM AUTHOR]

Histiocytosis of Langerhans cells (class 1 histiocytosis) includes a range of clinical manifestations that have been described as bone eosinophilic granuloma, Hand-Schüller-Christian syndrome, and Letterer-Siwe diseases. This syndrome represents a spectrum of severity and prognosis of some underlying disorder which is usually sporadic. This report discribes three cases in one family, who developed the disease a few years after their brother was found to be suffering from histiocytosis. All 3 patients had the same clinical manifestations: hyperthermia, eczematic rash, and swelling in skull, hand, and foot. X rays showed lytic areas in the skull and metacarp of fourth finger. Serology for EBV infection was negative. Infiltratration of abnormal Langerhans cells histiocytes were demonstrated in bone biopsies. These patients were given chemotherapy. Case 1 (brother) died 1 year after chemotherapy, case 2 (girl) was given chemotherapy without success. She was given T-cell suppresor (cyclosporine), which induced remission, and case 3 was given chemotherapy, which was successful. [ABSTRACT FROM AUTHOR]

Unlabelled: Congenital self-healing reticulohistiocytosis (CSHRH), a rare disorder initially seen at birth or in the newborn period, is characterized by spontaneously involuting skin lesions. We present the case of a 12-d-old female who was born with many red-brownish nodules scattered over her entire body, and which regressed spontaneously.Conclusion: Although there are no clues to differentiate CSHRH from Letterer-Siwe disease, some signs and symptoms might enable us to reach an accurate diagnosis. A long-term follow-up to detect evidence of relapse or progression of the disease would be well advised. [ABSTRACT FROM AUTHOR]

Eosinophilic granuloma has traditionally been designated as the least severe component of the inter-related triad of diseases, Histiocytosis X (Lichtenstein 1953, 1964). In 1969, Lieberman and his group questioned the use of this time-honored term and proposed instead that eosinophilic granuloma be regarded as a separate and distinct entity which could present either as a singular (unifocal) lesion or as a multifocal process. The term, "multifocal eosinophilic granuloma" was, thus, suggested as a replacement for the designation formerly known as Hand-Schuller-Christian Disease. The remaining component of Histiocytosis X termed Letterer-Siwe Disease was felt to be a totally separate disorder unrelated to eosinophilic granuloma and best classified as a relatively aggressive reticuloendothelial malignancy. In this presentation, we wish to report a case of multifocal eosinophilic granuloma which has run a very interesting course over a total of almost 10 years from the time of initial symptoms to the present. The more recent classification of Lieberman will be employed throughout this presentation. We will also propose the hypothesis that in this case, multifocal eosinophilic granuloma may represent a sequela of acquired immune deficiency perhaps initiated by T-cell viral infection. The possibility that, in a susceptible host, development of eosinophilic granuloma may be triggered by contact with environment agents also be discussed. [ABSTRACT FROM AUTHOR]

A mandibular lesion from an infant with Letterer-Siwe disease was studied by electron microscopy and immunohistochemistry. Ultraslructurally, two types of cells were identified; clear and dark cells. Clear cells had indented nuclei and Langerhans cell granules, while dark cells were characterized by the occasional presence of a large number of lysosomes. Lysozyme was usually not detectable in proliferating cells with indented nuclei, but, it was strongly positive in cells without indented nuclei. The latter also usually contained IgG, although the occurrence of cytoplasmic IgM, A, D and E seemed to be negative in both types of cells. Thus clear cells showed a close resemblance to Langerhans cells and dark cells had features of ordinary histiocytes. Although the intimate relationship between these two types of cells remains obscure, our findings suggest that Letterer-Siwe disease may be a reactive or tumorous condition of a cellular subpopulation of Langerhans ceils with a proliferation of differentiated histiocytes. [ABSTRACT FROM AUTHOR]

The morphological, ultrastructural and immunophenotypic properties of Histiocytosis-X (H-X) cells were investigated in a lymph node involved by Letterer-Siwe (L-S) disease. H-X cells were T6+ (CD1a), S-100+, T4+ (CD4) and HLA-DR+; in addition they were consistently T11+ (CD2) and were stained by antibodies directed against receptors for transferrin (T9), C3bi (OKM-1/CD11b), IgG-Fc (Leu-11/CD16) and Interleukin-2 (IL-2R/CD25). On immunostained cytosmears, T6+ cells were highly polymorphic and a prominent fraction (45%) showed immature morphology, characterized by lymphoid appearance. Cells expressing macrophage markers (ANAE, AACT, Leu-M3/CD14, PAM-1) were 10-fold fewer than T6+ cells and did not show a lymphoid morphology. At TEM level, H-X cells were characterized by poor content of LC granules and by the presence of myelin-like laminated bodies and of lysosome-like dense bodies. The immunophenotypic properties of H-X cells were compared to those of epidermal Langerhans cells (LCs) and of LCs present in lymph nodes of three cases of dermatopathic lymphadenitis. Epidermal LCs were T6+/HLA-DR+, and sometimes faintly T4+. Lymph node LCs were T6+, S-100+, T4+, HLA-DR+, and showed the same variety of surface receptors detected in H-X cells; furthermore, in a case with massive infiltration of the paracortex by T6+ cells, lymph node LCs were faintly T11+ and some of the T6+ cells had lymphoid aspect. Our findings suggest that the H-X cell population of L-S disease is not homogeneous, but is composed of discrete cell subsets with distinctive antigenic and morphological traits closely resembling those of cells of LC lineage at different maturational stages. [ABSTRACT FROM AUTHOR]

Fresh, not chemically fixed, glycerol-cryoprotected material from skin lesions of histiocytosis-X (Letterer-Siwe disease) was examined by the freeze-fracture technique. Fracture appearances of the cytoplasmic X-granules characteristic of cells of histiocytosis-X were established, and the accuracy of previous three-dimensional reconstructions of variations, in overall shape of granules based upon stained thin sections was confirmed. These variations, therefore, arc not artifacts of conventional tissue processing. Little or nothing of the internal structure of X-granules is seen in replicas, and the technique provides no new information concerning their origin or mode of formation. [ABSTRACT FROM AUTHOR]

Large globular histiocytes infiltrated the papillary region of the dermis with little tendency to extend deeper than the subpapillary vascular plexus. Histiocytes penetrated the basement membrane and colonized the epidermis causing disruption with formation of haemorrhagic crusts. Groups of histiocytes within the epidermis give an appearance similar to that of Paget's disease. The histiocytes were large and rounded with "shaggy" margins and fairly distinct copius cytoplasm. Both Langerhans' cells and histiocytosis X cells fail to become strongly phagocytic under ordinary conditions in lymph nodes and in the skin. None the less, these cells show some of the characteristics of phagocytes with migratory ability, adhesion to glass, apposition to Lymphocytes and a variable hydrolytic enzyme content.

A 91-year--old woman presented with an itchy, erosive and scaly eruption of about 2 years duration. Clinical, histological and ultrastructural examination of the skin lesions showed typical changes due to Letterer-Siwe disease. Large mononuclear cells with deeply indented or convoluted nuclei and with Langerhans' cell granules in the cytoplasm, were found to invade the superficial dermis, epidermis and dermo-epidermal junction. [ABSTRACT FROM AUTHOR]

This article presents the clinical details of seven patients who were over 50 years of age at the onset of Letterer-Siwe disease. There were four females and three males. The skin eruption is fairly distinctive and is due to infiltration of the epidermis and dermis by histiocytes. In all cases the lesions involved the trunk and major flexures. The small papules tended to have a lichenoid crusted appearance and often appeared pigmented or purpuric. The distribution was usually seborrhoeic in type and initially in some cases was diagnosed as a refractory seborrhoeic dermatitis. Where the lesions show follicular prominence, Letterer-Siwe disease in the adult may resemble Darier's disease. There is a distinctive skin eruption but the prognosis of the disease depends on the extent of systemic rather than the degree of cutaneous involvement.