Eventually, the ulcer was biopsied, revealing a typical LCH pathology, with dermal infiltrate of morphologically characteristic Langerhans cells extending into the epidermis, which were positive for S100, CD1a, and
CD207, with other inflammatory cells (Figure 2).
Silva et al., "Langerhans cells (CD1a and
CD207), dermal dendrocytes (FXIIIa) and plasmacytoid dendritic cells (CD123) in skin lesions of leprosy patients," Microbial Pathogenesis, vol.
LCs express C-type lectins on their plasma membrane langerin (
CD207) in mice [71] and CD1a in human [72] and Fc[gamma] and Fc[epsilon] receptors [73].
The characteristic immunophenotype of LCH includes expression of CD1a, S100 protein, and langerin (
CD207) in LCs.
In systemic histiocytic disorders, such as juvenile xanthogranuloma, xanthoma disseminatum, Erdheim-Chester disease, and dendrocytomas, FXIII-A, along with other markers (e.g., S100, CD1a, CD68, fascin,
CD207, and CD35), is also used as a key diagnostic tool [49-51].
Langerhans cells stain positive for CD1a, Langerin (
CD207), S-100 protein, and HLA-DR on immunohistochemistry.
Abbreviations, CD numeration, and alternate names: DEC-205 (CD205), B220 (CD45R), PDCA-1 (plasmacytoid DC Ag-1; CD317; Bst2), Siglec H (Sialic acid-binding immunoglobulin-like lectin H), Langerin (
CD207), CD141 (BDCA-3), CD1c (BDCA-1), and CD303 (BDCA-2).
Langerin (
CD207, Clec4K) is a type-II transmembrane cell surface receptor highly expressed on Langerhans cells, CD103+ DCs, and splenic CD8+ DCs (Table 1).
Histopathology of LCH shows a granulomatous infiltrate that is epidermotropic, and the presence of positive CD1a or
CD207 staining confirms the diagnosis, he said.
Histopathology of LCH shows a granulomatous infiltrate that is epidermotropic, and the presence of positive CDla or
CD207 staining confirms the diagnosis, he said.
We detected a wide heterozygous duplication at 2p13.3 that spanned about 735 kb and included 10 genes:
CD207 (
CD207 molecule, langerin), VAX2 (ventral anterior homeobox 2), ATP6V1B1 (ATPase, H+ transporting, lysosomal 56/58kDa, V1 subunitB1), ANKRD53 (ankyrin repeat domain 53), TEX261 (testis expressed 261), NAGK (N-acetylglucosamine kinase), MCEE (methylmalonyl CoA epimerase), MPHOSPH10 [M-phase phosphoprotein 10 (U3 small nucleolar ribonucleoprotein)], PAIP2B [poly(A) binding protein interacting protein 2B], and ZNF638 (zinc finger protein 638), as well as the first 20 exons of DYSF [dysferlin, limb girdle muscular dystrophy 2B (autosomal recessive)], which causes LGMD2B (33).
The presence of Birbeck granules can be more easily proven now by immunohistochemical staining of Langerin (
CD207).