Solitary cutaneous dendritic cell tumor in a child: Role of dendritic cell markers for the diagnosis of skin Langerhans cell histiocytosis

Weiss, Th.; Weber, Lutz; Scharffetter‐­Kochanek, Karin; Weiss, Johannes M.

doi:10.1016/j.jaad.2005.07.011

Cited by 36 publications

(28 citation statements)

References 42 publications

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“…In rare cases, downmodulation of Langerin in activated LCs makes the classical view less certain that the clinical diagnosis of LCH can be based solely on Langerin expression. 49 Recently, the specificity of Langerin expression for LCs was further challenged in mice. Using GFP knockin BALB/c mice in the Langerin locus, Langerin expression was found in LN, spleen, thymus, and liver, whereas similar findings in humans have been reported (a first description of such cells in human spleen is found in Figure 2G).…”

Section: Discussionmentioning

confidence: 99%

In vivo transformation of mouse conventional CD8α+ dendritic cells leads to progressive multisystem histiocytosis

Steiner

Otten

Hicks

et al. 2008

Blood

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IntroductionDendritic cells (DCs) are the antigen-presenting cells (APCs) capable of inducing adaptive immune responses and tolerance. 1 DCs act as sentinels in peripheral tissues and as APCs in secondary lymphoid organs, filtering their environment and detecting environmental changes that modulate the balance between tolerance and immune response. Following infection and inflammation, DCs induce costimulatory molecules, secrete cytokines, and can change their migration properties. 2,3 Several DC subtypes with unique and overlapping functions have been described. Plasmacytoid DCs (pDCs) are the main interferon (IFN) type I-producing cells, whereas several subtypes of conventional DCs (cDCs) are widely distributed. 4,5 In skin, 2 DC populations, namely Langerhans cells (LCs) and dermal DCs, localize to the epidermis and dermis, respectively. Upon pathogen encounter, they migrate to the draining LN. 6 In skin and spleen, DCs are derived either from blood or from immediate local precursors. [7][8][9][10][11][12][13] DCs are dividing cells rather than terminally differentiated cells. [12][13][14][15] The importance of DC cycling on their homeostasis is currently debated, and DC proliferation has been recently proposed to extend the duration of antigen presentation. 15 The human histiocytic diseases are characterized by abnormal accumulation or proliferation of macrophages or DCs. 16 They are divided into LC or non-LC histiocytosis. In the former category, LC histiocytosis (LCH) is best described. LCH can range from a spontaneously regressing single organ disease to a life-threatening or disabling relapsing multisystem disease. 16 In the latter category, a genetic deficiency in cytotoxic activity, familial hemophagocytic lymphohistiocytosis (FHL), is the principal type implicating macrophages and lymphocytes. In contrast to LCH and FHL, lesions with proliferative DCs with or without cytologic atypia are less well classified and their prevalence remains controversial. 16 Unfortunately, a confusing terminology describing these cases of proliferating DCs persists in the literature as malignant histiocytosis, histiocytic sarcoma, and DC sarcoma.The diagnosis of histiocytic diseases relies on the morphologic, ultrastructural, and immunohistochemical histiocyte properties. The macrophage, DC, or LC lineage markers allow LC to be distinguished from non-LC histiocytosis. For example, diagnostic criteria for LCH include expression of Langerin or detection of Birbeck granules that are pathognomonic, as well as CD1a and S100, which are less specific. According to the International Lymphoma Study Group, histiocytic sarcoma, LC tumor, and LC sarcoma (LCS) are the terms used to separate cases based on the degree of cytologic atypia and on lineage markers. 17 For example, diagnosis of LCS requires the same markers as LCH and the additional presence of cellular and mitotic atypia.While LCS, LC, and DC tumors are clearly neoplastic, it is vigorously debated whether LCH is a reactive or a neoplastic The online version of this article ...

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Section: Discussionmentioning

confidence: 99%

In vivo transformation of mouse conventional CD8α+ dendritic cells leads to progressive multisystem histiocytosis

Steiner

Otten

Hicks

et al. 2008

Blood

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“…In addition, CD1a may be superior to Langerin and S100 protein in identifying Langerhans cells in liver biopsies. This theory is supported by a study that demonstrated that Langerhans cells may lose Langerin expression after reaching maturity 23 and by another study that demonstrated that the periductal dendritic cells in PBC are mostly mature dendritic cells. 11 Finally, S100 protein is not a specific marker for Langerhans cells, and it may be positive in macrophages and histiocytes in the liver.…”

Section: Discussionmentioning

confidence: 82%

Evaluation of Langerhans Cell Infiltrate by CD1a Immunostain in Liver Biopsy for the Diagnosis of Primary Biliary Cirrhosis

Graham

Smyrk

Zhang

2012

American Journal of Surgical Pathology

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Primary biliary cirrhosis (PBC) is characterized by chronic nonsuppurative destructive cholangitis, which is considered to be a cell-mediated immune reaction. Antigen-presenting cells, including Langerhans cells and dendritic cells, have been found in portal tracts and in bile duct epithelium and may play a role in the pathogenesis of PBC, but the importance of identifying these cells for diagnosing PBC has not been studied yet. In this study, we sought to evaluate the importance of identifying Langerhans cells using a CD1a immunostain in the diagnosis of PBC. Liver biopsies from adult patients diagnosed with PBC (n=60), primary sclerosing cholangitis (n=29), obstructive cholangitis (n=13), chronic viral hepatitis B or C (n=19), autoimmune hepatitis (AIH, n=15), acute cellular rejection (n=11), and chronic rejection (n=10) at our institution were retrospectively reviewed. An immunohistochemical stain for CD1a was used to detect Langerhans cells, and the distribution of CD1a-positive Langerhans cell infiltrate was recorded as lobular, portal with bile duct sparing, and intraepithelial. Intraepithelial Langerhans cells were identified in 58% of PBC including antimitochondrial antibody-negative PBC and PBC-AIH overlap cases, 14% of primary sclerosing cholangitis, 15% of obstructive cholangitis, 9% of acute cellular rejection, 6% of AIH, and no cases of chronic viral hepatitis or chronic rejection. The number of intraepithelial Langerhans cells was significantly higher in PBC than in other conditions, with the mean number of CD1a-positive intraepithelial Langerhans cells per bile duct in PBC calculated as 2.2 compared with <1 per duct for the other control cases. Thirty-three of 60 (55%) PBC cases showed at least one bile duct containing ≥2 CD1a-positive Langerhans cells. This was statistically significant (P<0.01) when compared with control groups. The overall sensitivity and specificity of using ≥2 CD1a-positive Langerhans cells per bile duct as the diagnostic criteria for PBC were 55% and 96%. Given the heterogenous nature of liver involvement by PBC, a review of cases with morphologic features of duct damage yielded an increased sensitivity (79%) with no reduction in specificity. In conclusion, the detection of a Langerhans cell infiltrate of ≥2 cells by CD1a in a given bile duct on needle biopsy may be a valuable tool in the diagnosis of PBC.

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“…First, juvenile cutaneous xanthogranuloma can appear as of birth or during the neonatal period and can initially resemble Langerhans histiocytosis; it only takes on its characteristic yellow tint later. Routine histology cannot differentiate; immunohistochemical labeling (negative protein S100) and electron microscopy (absence of Birbeck granules) are needed to confirm the diagnosis [22]. Ulcerated hemangioma may also resemble an ulcerated crusted nodule and, indeed, was the diagnosis initially proposed for our patient 2, before he was seen in the pediatric dermatology clinic.…”

Section: Discussionmentioning

confidence: 99%

“…For some authors, the association of Birbeck granules and dense lamellar bodies is highly suggestive of HPH [28]. Immunohistochemical labeling with monoclonal antibodies to langerin, reported to be a new specific marker of Birbeck granules [22], might eventually eliminate the need for electron microscopy.…”

Section: Discussionmentioning

confidence: 99%

Congenital Solitary Histiocytoma: A Variant of Hashimoto-Pritzker Histiocytosis

Zunino-Goutorbe

Eschard²,

Durlach³

et al. 2008

Dermatology

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Background: Self-healing solitary-lesion Hashimoto-Pritzker histiocytosis (HPH), a rare, congenital, purely cutaneous Langerhans histiocytosis (only 30 cases reported), carries a good prognosis. Objective: To describe the clinical and histopathological characteristics of solitary HPH. Methods: To conduct a retrospective, observational study on 8 affected newborns. Results: For these infants, with otherwise normal physical examinations, the unique nodule or papule (5–15-mm diameter) was congenital. Systematic routine histological examination of the lesions found dermal infiltrates constituted predominantly of histiocytes with lymphocytes and eosinophils. Protein S100 and CD1a immunolabelings, done for 7 patients, were positive. Electron microscopy (n = 4) observed Birbeck granules. No visceral involvement or recurrence has ever been observed after 2–12 years of follow-up. Conclusions: Because of its self-healing nature, congenital solitary HPH frequency has probably been underestimated. In the absence of systemic involvement, regular physical examination for at least 2 years seems a valid approach.

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Solitary cutaneous dendritic cell tumor in a child: Role of dendritic cell markers for the diagnosis of skin Langerhans cell histiocytosis

Cited by 36 publications

References 42 publications

In vivo transformation of mouse conventional CD8α+ dendritic cells leads to progressive multisystem histiocytosis

In vivo transformation of mouse conventional CD8α+ dendritic cells leads to progressive multisystem histiocytosis

Evaluation of Langerhans Cell Infiltrate by CD1a Immunostain in Liver Biopsy for the Diagnosis of Primary Biliary Cirrhosis

Congenital Solitary Histiocytoma: A Variant of Hashimoto-Pritzker Histiocytosis

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