elastin and Granuloma--Giant-Cell

Annular elastolytic giant cell granuloma (AEGCG) is a rare granulomatous skin condition. It belongs to a group of skin and elastic fiber disorders. When it affects sun-exposed skin, it is also called actinic granuloma. The etiology and pathogenesis are still debated. However, sun-induced actinic damage to elastic fibers is acknowledged as the primary triggering factor, though the pathogenesis of instances in sun-covered areas is unknown. The most commonly linked systemic illness is diabetes mellitus. Different case reports show an association of this disease with hematological conditions, infections, sarcoidosis, and protoporphyria. Multisystemic involvement was also reported in a case. The disease is clinically recognized by erythematous non-scaly annular patches and plaques with raised borders and hypopigmented or skin-colored centers, sometimes atrophic. It is usually asymptomatic or mildly itchy. The presence of an inflammatory infiltration with non-palisading granulomas, multinucleate large cells, elastin degradation, and elastophagocytosis, as well as the absence of necrobiosis and mucin, are histopathological characteristics. We report a 5-year history of annular elastolytic giant cell granuloma in a 66-year-old woman with a history of type two diabetes mellitus, hypertension, and fatty liver disease (steatosis). She presented with asymptomatic polymorphic erythematous skin lesions mainly in sun-exposed areas.

Topics: Aged; Diabetes Mellitus; Elastin; Female; Granuloma, Giant Cell; Humans; Metabolic Syndrome; Mucins; Photosensitivity Disorders

Annular elastolytic giant cell granuloma (AEGCG) is characterized by non-palisading granuloma and elastophagocytic giant cells. Granulomas consist of structured masses of macrophages, dendritic cells, and T lymphocytes which play an essential role in granuloma formation. Two lineage systems of dendritic cells and macrophages originated from peripheral blood monocytes have been established in vitro. To know how elastin fragments are involved in the granuloma formation in AEGCG, we tested in vitro whether elastin fragments potentially induce monocyte-derived macrophages or dendritic cells to form granuloma and multinucleated giant cells. Immunohistochemical studies of the lesional skins of AEGCG (n = 5) revealed that the 67-kDa elastin receptor was specifically expressed in the epithelioid or multinucleated giant cells. Proliferation of factor XIIIa(+) cells and CD68(+) cells was also seen in the lesional skins of AEGCG. Factor XIIIa(+) dendritic cells or CD68(+) macrophages were established by the treatment of granulocyte/macrophage-colony stimulating factor (GM-CSF)/interleukin-4 or M-CSF, respectively. Further treatments of these dendritic cells or macrophages with elastin peptide resulted in the formation of granuloma or multinucleated giant cells which were immunoreactive with anti-67-kDa elastin receptor antibody. These findings suggest that elastic tissue induces factor XIIIa(+) cells and CD68(+) macrophages to form granuloma or multinucleated giant cells and plays an essential role in the formation of granuloma in AEGCG.

Topics: Aged; Aged, 80 and over; Antigens, CD; Antigens, Differentiation, Myelomonocytic; Biopsy; Cells, Cultured; Dendritic Cells; Elastin; Factor XIIIa; Female; Granuloma, Giant Cell; HLA-DR Antigens; Humans; In Vitro Techniques; Macrophages; Male; Middle Aged; Monocytes; Receptors, Cell Surface; Skin Neoplasms

Eight specimens each of the following groups were investigated: gingival pyogenic granuloma, fibrous epulis, calcifying fibrous epulis, peripheral giant cell granuloma, giant cell fibroma (four gingival, four non-gingival), denture-irritation hyperplasia and fibroepithelial polyp. These lesions have diverse histopathological appearances but the composition of their connective tissue is poorly defined. The elastic system consists of a complex mixture of glycoproteins that in normal oral mucosa form three differentially distributed fibre types; oxytalan, elaunin and elastic. The elastic system was investigated by Verhoeff's haematoxylin stain, aldehyde fuchsin staining and an anti-elastin monoclonal antibody. Elastin was identified in all fibroepithelial polyps and denture-irritation hyperplasias, but in none of the other lesions. In particular, this identified a distinct difference in the extracellular matrix between the giant cell fibroma and fibroepithelial polyp. Many of the epulides included only oxytalan fibres, but the presence of oxytalan fibres did not follow any pattern within either a single lesion group, or between different lesions. However, the presence of oxytalan fibres in the absence of elastin does not necessarily support a periodontal ligament origin for reactive epulides.

Topics: Antibodies, Monoclonal; Diagnosis, Differential; Elastic Tissue; Elastin; Fibroma; Gingival Diseases; Gingival Hyperplasia; Gingival Neoplasms; Gingival Overgrowth; Granuloma, Giant Cell; Granuloma, Pyogenic; Hematoxylin; Humans; Immunohistochemistry; Mouth Mucosa; Polyps; Rosaniline Dyes; Stomatitis, Denture

Heterologous elastin particles were injected into the deep dermis or below the panniculus carnosus of hairless mice. A granulomatous tissue reaction with concomitant giant cell formation occurred around the implanted elastin in the early stages and, subsequently, calcium deposition with disappearance of the histiocytes and giant cells occurred in the later stages. Ultrastructural study revealed that some elastin particles were engulfed by the giant cells; others underwent calcification but remained intact. The heterologous collagen used as a control also induced a granulomatous tissue reaction, but did not undergo calcification and gradually disappeared. These results suggest that the heterologous elastin and collagen particles exhibit different susceptibilities to digestion. However, it is not clear why the heterologous elastin underwent calcification.

Topics: Animals; Calcification, Physiologic; Collagen; Elastin; Granuloma, Giant Cell; Male; Mice; Mice, Hairless; Microscopy, Electron; Skin Diseases

A 15-year-old Japanese girl had widespread annular serpiginous erythematous plaques, bilateral granulomatous uveitis, bloody diarrhea, and seronegative arthralgia. She also had anemia and leukopenia. The histopathologic findings were compatible with those of annular elastolytic giant cell granuloma. Elastolytic granulomas were also found in the cervical lymph nodes, terminal ileum, parietal peritoneum, and mesentery. Bilateral hilar lymphadenopathy, hypercalcemia, and an increased level of angiotensin converting enzyme were not observed throughout the clinical course. To the best of our knowledge, systemic elastolytic granulomatosis has not been previously described in annular elastolytic giant cell granuloma or sarcoidosis. This case may represent a type of granulomatosis in the broad spectrum of annular elastolytic giant cell granuloma and sarcoidosis.

Topics: Adolescent; Elastin; Erythema; Female; Granuloma Annulare; Granuloma, Giant Cell; Humans; Intestinal Diseases; Lymphatic Diseases; Neck; Sarcoidosis; Uveitis

Topics: Elastin; Granuloma, Giant Cell; Humans; Necrobiosis Lipoidica; Phagocytosis; Skin Diseases

This report reviews thirteen cases of generalized granuloma annulare. The light and electron microscopic appearance of intracellular elastin particles in eleven of these cases is described. These bodies were also noted in deep granuloma annulare, elastosis perforans serpiginosa, and a small percentage of localized granuloma annulare. They were not found in necrobiosis lipoidica.

Topics: Adult; Aged; Biopsy; Elastic Tissue; Elastin; Female; Granuloma; Granuloma, Giant Cell; Histiocytes; Histocytochemistry; Humans; Male; Middle Aged; Skin; Skin Diseases