Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P06126 (CD1a)
 2,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Papular xanthoma (PX) is a very rare skin disorder. We describe a typical case of PX in a 13-month-old Chinese boy who presented with numerous yellow-red papulonodules, 2-8 mm in diameter, mainly on the face, both upper extremities, and abdomen of 10 months duration. Histologic studies showed a diffuse monomorphous infiltrate of foamy cells in the upper dermis. The foamy cells stained positively with oil red O and CD68. The periodic acid Schiff (PAS) stain, S-100 protein, CD1a, CD56, lysozyme, alpha1-antitrypsin, and factor XIIIa were all negative in the foamy cells. The electron microscopic (EM) studies revealed the morphologic features of macrophages with electron-dense, membrane-limited lipid vacuoles in the cytoplasm. After 14 months, neither spontaneous regression nor anetoderma-like scars were noted. Our immunohistochemical and ultrastructural studies support the notion that the origin of the foamy cells is the macrophage rather than the factor XIIIa (+) dermal dendrocyte. There was no associated or underlying disease in this case. We suggest the term primary PX for cases such as this one.
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PMID:Primary papular xanthoma of children: a clinicopathologic, immunohistopathologic and ultrastructural study. 941 17

Dermatofibroma is composed largely of interlacing fascicles of slender spindle cells set within a loose collagenous stroma and of scattered foamy histiocytes and multinucleated giant cells. There is clear evidence indicating that factor XIIIa+ dermal dendritic cells (DDCs) are the cells constituting dermatofibromas. However, it is still unknown what stimulation is responsible for transforming DDCs into different cell types, producing different subtypes of dermatofibromas. Recently, it has become possible to obtain dendritic cells (DCs), that are identical with DDCs in their phenotypic and functional characteristics, from the culture of CD14+ peripheral blood monocytes to which IL-4 and GM-CSF were added. Using these monocyte-derived DCs, we examined the ability of various cytokines, such as IL-1beta , IL-3, IL-5, IL-6, IL-7, IL-8, IL-10, TNFalpha, TGFbeta, M-CSF, IFNalpha, and IFNgamma, and phorbol 12-myristate 13-acetate (PMA), to induce different cell types observed in DFs. Among them, only PMA could induce a variety of cell types such as histiocytic cells, fibroblastic spindle-shaped cells, and even multinucleated giant cells of Touton or foreign body type. Phenotypically, all the induced cell types expressed CD1a, CD80, CD86, HLA-DR, and CD68 in a magnitude similar to that of non-treated monocyte-derived DCs. The expression of factor XIIIa was strongest in histiocytic cells, moderate in fibroblastic cells, and weakest or negative in giant cells. These data suggest that dermatofibromas are a kind of neoplastic disease which is induced only by the effect of some tumor promoter on DDCs.
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PMID:Phorbol 12-myristate 13-acetate can transform monocyte-derived dendritic cells to different cell types similar to those found in dermatofibroma. A possible in vitro model of the histogenesis of dermatofibroma. 952 94

Dermal dendrocytes (DDs) are bone marrow-derived cells which are abundant in normal human and murine dermis, where they are closely associated with mast cells in the perivascular space. The biological role of DDs remains enigmatic. DDs express coagulation factor XIIIa and the recently described von Willebrand factor receptor, GPIb alpha, potentially indicating a function in tissue repair and haemostasis, although participation in antigen presentation is also speculated. In healing wounds and 'fibrohistiocytic' tumours, such as dermatofibromas, DDs are often associated with non-dendritic histiocytes, some of which also express factor XIIIa (FXIIIa). We have utilized human skin organ culture to examine the effects of various biological mediators on cytological characteristics of DDs. It was found that by 24 h in organ culture, immunoreactive DDs begin to lose their dendritic shape, assuming more rounded contours. This phenomenon was accentuated by mast cell degranulation; was independent of the nature of mast cell secretagogue; and could not be reproduced by recombinant tumour necrosis factor-alpha, a cytokine known to increase FXIIIa expression in DDs. Like their dendritic precursors, non-dendritic cells expressed variable FXIIIa, CD34 and CD68 and did not express CD1a or CD45. By ultrastructure, non-dendritic cells that develop in vitro resembled non-degenerating monocytes containing occasional primary lysosomes and lipid inclusions, and like DDs, expressed fibronexus-like plaques on the cell membrane. Transition of DDs from dendritic to non-dendritic cells as a consequence of specific microenvironmental influences may provide insight into the frequent concurrence of these two cytological types in fibrohistiocytic tissue reactions and neoplasia.
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PMID:Cytological alterations in dermal dendrocytes in vitro: evidence for transformation to a non-dendritic phenotype. 1088 40

We report three children who had multisystem Langerhans cell histiocytosis (LCH) with cutaneous involvement and subsequently developed juvenile xanthogranuloma (JXG). JXG appeared 3--6 years after the initial manifestation of LCH. JXG lesions, which presented as yellowish papules, revealed typical Touton giant cells and were factor XIIIa positive but S100 and CD1a negative. Non-LCH histiocyte disorders, such as JXG, are known to occur as a reaction to a variety of external stimuli such as infection and trauma. It is therefore conceivable that the inflammatory reaction associated with LCH may have precipitated the development of JXG in our patients. Alternatively, one could speculate that this association might be due to a common histogenetic precursor of the cell types involved.
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PMID:Juvenile xanthogranuloma as a sequel to Langerhans cell histiocytosis: a report of three cases. 1148 23

To elucidate the pathogenesis of T cell-mediated inflammatory skin diseases, we examined the exact sites where CD8(+) T cells proliferate, correlating them with the localization of antigen-presenting dendritic cells. We performed CD8/Ki-67 double immunohistochemical staining and single staining for CD1a, CD68, and factor XIIIa on sections of paraffin-embedded tissue samples of inflammatory dermatoses in which T lymphocytes are thought to play a crucial role. The dermatoses were lichen planus (12 samples), acute graft-versus host disease (GVHD) (12 samples), chronic GVHD (10 samples), spongiotic dermatitis (8 samples) and psoriasis (7 samples). Labelling for Ki-67 among CD8(+) T cells was predominantly observed in the subepidermal lymphoid infiltrate, and was scanty in the epidermis. This suggested that proliferation of CD8(+) T cells occurred preferentially in the dermis. The labelling index for Ki-67 among dermal and epidermal CD8(+) cells was quite different among the different diseases studied (P < 0.05). They were rich in the subepidermal portion of the dermis of spongiotic dermatitis, acute GVHD and chronic GVHD, but rare in the dermis of psoriasis and lichen planus. A moderate infiltrate was also observed in lesional epidermis of spongiotic dermatitis, acute GVHD and chronic GVHD, whereas they was almost none in the epidermis of psoriasis and lichen planus. CD1a(+) dermal dendritic cells were densely distributed within the lymphoid infiltrate in the affected dermis of spongiotic dermatitis, psoriasis and lichen planus, whereas they were minimal in GVHD. These dermal dendritic cells are candidates as stimulators on T cells in the dermis. In conclusion, the proliferative status of T cells could be an important clue in the elucidation of the pathophysiology of T cell-mediated inflammatory dermatoses.
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PMID:Proliferative activity of CD8(+) T cells as an important clue to analyze T cell-mediated inflammatory dermatoses. 1175 86

Distinguishing desmoplastic melanoma (DM) from scar tissue on routine microscopy can be difficult, especially in re-excision specimens, and S100 immunohistochemistry has been recommended as a useful adjunct. The purpose of this study is to evaluate the extent and nature of S100 positivity in scars. In this study, formalin-fixed paraffin archival tissues were evaluated with immunohistochemistry. Ten re-excision specimens of previously biopsied nonnevomelanocytic lesions were immunostained with the S100 and CD57 (Leu 7) antibodies. In 9 of the 10 cases, the scars contained S100-positive spindle cells, but there were no cases with CD57+ cells. Ten re-excised atypical nevi and 10 re-excised melanomas were also immunostained for the S100 protein, and all 20 cases contained S100-positive spindle cells within the scars. There was a trend toward quantitatively more S100-positive spindle cells in these nevomelanocytic re-excisions. To evaluate the nature of the spindle cells, scars from two of the nonnevomelanocytic re-excisions were further analyzed utilizing immunostains for glial fibrillary acidic protein, HMB-45, Melan-A, CD1a, factor XIIIa, and neuron specific enolase. In both scars, neuron specific enolase diffusely stained the fibroblast population, but the remaining immunostains were negative in the scar. The presence of S100-positive spindle cells in scars represents a potential diagnostic pitfall, particularly in the evaluation of re-excision specimens of DM.
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PMID:S100-positive spindle cells in scars: a diagnostic pitfall in the re-excision of desmoplastic melanoma. 1214 9

A 13-year-old girl developed a non-pruritic pityriasis rosea-like rash, which did not respond to topical corticosteroids or UV therapy but persisted for 2 years. The lymphohistiocytic infiltrate in the upper dermis showed mononuclear cells immunoreactive with S100, CD68, factor XIIIa and CD1a. Electron microscopic evaluation of these cells demonstrated lamellated dense bodies but no Birbeck granules, lipid vacuoles or cholesterol crystals. Two diagnoses were made: a primarily clinical diagnosis of generalized eruptive histiocytosis and a more cell-biology-based diagnosis of an indeterminate cell histiocytosis. Three years later, the lesions are showing spontaneous resolution, with loss of erythema and flattening. Our patient's indeterminate cells fulfil Rowden's classical definition (dendritically shaped epidermal non-keratinocytes without identifying cytoplasmic features), as well as Zelger's newer definition (cells with features of both macrophages and dendritic cells). A Christmas tree pattern has not been previously described in indeterminate cell histiocytosis. Development of indeterminate cell histiocytosis in the lesions of a healing pityriasis rosea might explain the unusual distribution pattern. The development of a skin disorder at the site of an unrelated, already healed skin disease is known as an isotopic response. Key
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PMID:Long-lasting "christmas tree rash" in an adolescent: isotopic response of indeterminate cell histiocytosis in pityriasis rosea? 1236 Nov 35

Antigen-presenting cells (APCs) participate in the initiation of the inflammatory process in various immune-mediated dermatoses through the activation of antigen-specific T lymphocytes. The skin contains several different subsets of APCs. To investigate the role of these APCs in T-cell immune-mediated inflammation, we examined the distribution and numbers of epidermal and dermal CD1a(+) dendritic cells (DCs), factor XIIIa(+) dermal DCs, and CD68(+) macrophages in five T-cell-mediated inflammatory skin diseases. Immunohistochemistry of CD1a, factor XIIIa, and CD68 was performed using paraffin-embedded tissue obtained from a total of 51 patients with eczematous dermatitis (histologically spongiotic dermatitis), psoriasis, lichen planus, acute graft-versus-host disease (GVHD), and chronic GVHD. The numbers of positive cells for each staining were compared with those in site-matched normal skin control specimens from aged-matched subjects. In spongiotic dermatitis and lichen planus, the numbers of epidermal and dermal CD1a(+) cells and factor XIIIa(+) cells were significantly greater than in normal control skin, while in psoriasis only factor XIIIa(+) cells were significantly increased in number. Acute and chronic GVHD showed a reduced number of dermal CD1a(+) cells. Interestingly, factor XIIIa(+) cells were decreased in acute GVHD while they were increased in chronic GVHD. There was a significant reduction in epidermal CD1a(+) cells in acute GVHD, but not in chronic GVHD. The differences in the numbers of APCs in lesional skin appeared to reflect differences in the pathophysiology of these inflammatory skin diseases.
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PMID:Comparison of the distribution and numbers of antigen-presenting cells among T-lymphocyte-mediated dermatoses: CD1a+, factor XIIIa+, and CD68+ cells in eczematous dermatitis, psoriasis, lichen planus and graft-versus-host disease. 1237 34

Juvenile xanthogranulomas (JXG) is a histiocytic disorder, primarily but not exclusively seen throughout the first two decades of life and principally as a solitary cutaneous lesion. This study is a retrospective clinical and pathologic review of 174 cases documenting the cutaneous and extracutaneous manifestations in patients presenting from the neonatal period to 20 years of age (mean 3.3 years; median 1 year). There was a male predominance (99 male:75 female) in all categories of clinical presentation, but especially notable in the group with multiple cutaneous lesions (12 male:1 female). A solitary cutaneous lesion accounted for 67% of all cases, followed by a solitary subcutaneous or deep soft tissue mass (28 cases, 16%), multiple cutaneous lesions (13 cases, 7%), a solitary extracutaneous, nonsoft tissue lesion (9 cases, 5%), and multiple cutaneous and visceral-systemic lesions (8 cases, 5%). The recorded deaths due to disease included two neonates with systemic JXG who developed hepatic failure and thrombocytopenia and at autopsy had giant cell-neonatal hepatitis in addition to JXG in the liver and other visceral sites. A third death in a 3-month-old boy with a retroperitoneal-pelvic JXG occurred after failure to control severe hypercalcemia. The characteristic Touton giant cell in variable numbers was a consistent feature of the cutaneous lesions; however, these cells were either absent or present in reduced numbers in the various extracutaneous lesions when compared with JXG in the skin. Spindle cells intermingled among the mononuclear cells or forming short fascicles were seen in both cutaneous and extracutaneous lesions. Immunohistochemistry was performed on all extracutaneous lesions, and the constituent cells, regardless of their individual morphologic features, were uniformly positive for vimentin, CD68, and factor XIIIa and negative for S-100 protein and CD1a. It is widely held that JXG is a proliferative disorder of dendrocytes, possibly dermal dendrocytes; thus, its clinical and pathologic similarities to Langerhans cell histiocytosis are not entirely unexpected in light of the most recently proposed international classification of histiocytic disorders, which includes JXG and Langerhans cell histiocytosis together as "dendritic cell-related" histiocytoses.
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PMID:Juvenile xanthogranulomas in the first two decades of life: a clinicopathologic study of 174 cases with cutaneous and extracutaneous manifestations. 1271 44

Sarcomas derived from immune accessory cells are uncommon malignancies, most of them occurring in lymph nodes while extra nodal sites are very rarely affected. Based on the immune profile, the cells that give origin to these neoplasms are currently divided in: follicular dendritic cell (FDC), interdigitating dendritic cell (IDC), indeterminate cell and Langerhans cell. A case of a dendritic cell sarcoma arising in the alveolar ridge mucosa in a 50-year-old female is reported here. The lesion presented as a nodular mass without defined borders and covered by reddish mucosa. Histologically, the tumour was composed of spindle-shaped cells with large nuclei and abundant cytoplasm, arranged in variable patterns as storiform and whorled and revealing interspersed lymphocytes. No capsule could be seen and the neoplasm extended up to the lining epithelium. Immunohistochemically, the spindle cells were positive for vimentin, S-100 protein, CD1a, factor XIIIa and focally to smooth-muscle actin, but were negative for CD21, CD35, CD23 and caldesmon--all markers of follicular dendritic cells. In conclusion, the present case has morphologic pattern of dendritic cell sarcoma and the immunophenotype is compatible with IDC cells or with intermediate dendritic cells and demonstrates the overlap of features among these entities.
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PMID:Dendritic cell sarcoma of the oral cavity. 1474 67


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