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(a) Annular elastolytic giant cell granuloma (AEGCG) involving back, and (b) regressed AEGCG lesions in the back b a
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Annular elastolytic giant cell granuloma (AEGCG) is a rare granulomatous skin disease characterized clinically by annular plaques with elevated borders and atrophic centers found mainly on sun-exposed skin and histologically by diffuse granulomatous infiltrates composed of multinucleated giant cells, histiocytes and lymphocytes in the dermis along...
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Citations
... However, the patient in this case had non-alcoholic fatty liver, which may be related principally to her obesity. Various other systemic disorders have been reported to coexist with this entity, like sarcoidosis [18], Hashimoto thyroiditis [19], vitiligo [20], temporal arteritis [21], implantation of cardiac pacemakers [22], stroke [23], Lyme disease [24], lymphoma [25], leukemia [26], syphilis [27], and protoporphyria [28]. A case of systemic elastolytic granulomatosis has also been described [29]. ...
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.
... In the literature a total of 10 articles, limited to individual case reports and letters published from 1991 to 2017 about generalized AEGCG, were identified (Table 1). [8][9][10][11][12][13][14][15][16][17] It emerges that generalized AEGCG, associated to a specific cause, was successfully treated with targeted therapy aimed at resolving the causative Koebner effect. For instance, AEGCG associated to Borrelia burgdorferi infection, was successfully treated with doxycycline. ...
... Hashimoto's thyroiditis completely regressed after thyroid surgery. 10 On the contrary, in cases where no specific causative Koebner effect could be identified, further therapies such as minocycline hydrochloride, chloroquine, hydroxychloroquine and tranilast, associated to topical or oral steroids, were used with partial benefit (Table 1). ...
Annular elastolytic giant cell granuloma (AEGCG) is a rare granulomatous skin disorder, characterized by erythematous plaques with elevated borders and hypopigmented center, occurring mainly on sun exposed‐skin. Histologically it presents with elastophagocytosis and elastolysis. There is no established first line treatment for AEGCG, especially for the generalized form. In a small number of cases antimalarial drugs and tranilast, associated to topical or oral steroids, have been proposed to treat generalized AEGCG with partial benefits. We herein present the case of a patient with AEGCG aged 74 years, who was unresponsive to classical therapies, and then successfully treated with methotrexate.
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... The most commonly reported risk factor is intense sun exposure/tanning beds and is more commonly seen in fair-skinned individuals [15] residing in a sunny climate. Other possible predisposing conditions and associations include diabetes mellitus (DM), sarcoidosis [7], giant cell arteritis [5], polymyalgia rheumatica, polychondritis, vitiligo [3,9], anemia, leukopenia, hepatitis C, focal segmental glomerulosclerosis, Hashimoto's thyroiditis [16], erythema nodosum [17] and protoporphyria [18]. A list of possible differentials for our patient included photodermatoses (lack of suggestive histopathologic findings in this patient rules it out), granuloma annulare, necrobiosis (lack of evidence of DM), drug-induced photosensitivity (lack of consumption of associated medications), dermal manifestations of auto-immune disorders, such as systemic lupus erythematosus (negative ANA titer [5,7]. ...
Annular elastolytic giant cell granuloma (AEGCG) is a rare, often self-limiting chronic inflammatory disorder mostly occurring in the sun-exposed areas such as the dorsum of hands, extensor surfaces of arms, face, anterior neck, and upper chest. The pathognomonic histological findings include the presence of numerous granulomas associated with loss of elastic fibers that appear to be ingested by multi-nucleated giant cells. Here, we present a case of a 56-year-old woman with multiple, anatomically variable erythematous lesions- annular and papular, mainly in the upper body. The clinical presentation and histopathologic findings support our diagnosis.
... Granulomatous infiltrate with lymphocytes, histiocytes and multinucleated giant cells in the dermis, degradation of elastic fibers and elastophagocytosis by giant cells are the major histopathologic findings of AEGCG (8). ...
... However, necrobiosis and increased dermal mucin is absent in AEGCG unlike granuloma annulare and necrobiosis lipoidica. Elastolysis may be seen in granuloma annulare, but the complete loss of elastic fibers in the central area is characteristic of AEGCG (4,8,9). Furthermore, a hybrid pattern has been described, indicating an overlap between AEGCG and granuloma annulare (10). ...
... AEGCG can also be accompanied by systemic and dermatologic disorders such as diabetes mellitus, Hashimato's thyroiditis, stroke, temporal arteritis and vitiligo (5,8,(15)(16)(17). In our study diabetes mellitus, Hashimato's thyroiditis, hypertension, coronary artery disease, microscopic polyangiitis, Behçet's disease were found to be accompanying diseases to AEGCG. ...
Objective:
Annular elastolytic giant cell granuloma (AEGCG) is a rare granulomatous disease characterized by annular plaques. In this study, we aimed to describe the clinical and pathological features of the patients diagnosed with AEGCG.
Methods:
The demographic, clinical and pathological features of patients who diagnosed with AEGCG were recorded retrospectively.
Results:
Ten patients with AEGCG included in the study (nine females and one male). The mean age of the patients was 60±9.53 years. The mean duration of disease was 24.2±36.30 months. On dermatologic examination, multiple, well-demarcated, elevated borders and central atrophic erythematous annular plaques were seen in all patients. In the most of the patients (90%) lesions were on the sun-exposed regions. Six of the patients had accompanying diseases. Histopathologic examination of the punch biopsies revealed foreign body type multinucleated giant cells and lymphocytic cell infiltration in the dermis. There were intracellular elastic fiber fragments as sign of elastophagocytosis in the giant cells.
Conclusion:
AEGCG is a rare granulomatous disease which can accompany various diseases. There is debate on the terminology, classification and pathogenesis. Further studies are required to elucidate the unknowns.
Acquired elastolytic disorders comprise a complex spectrum of rare skin diseases that are characterized by loss of elastic tissue. In this review, acquired elastolytic skin disorders are briefly introduced, including acquired cutis laxa, anetoderma, annular elastolytic giant cell granuloma, mid-dermal elastolysis, papular elastorrhexis, perifollicular elastolysis, sporadic acrokeratoelastoidosis, and a relatively new condition named fibroelastolytic papulosis with its subtypes pseudoxanthoma-like papillary dermal elastolysis and white fibrous papulosis of the neck. Given the paucity of reported patients and limited research in this field, the pathomechanisms of acquired elastolytic skin diseases are poorly understood and effective therapeutic approaches are absent.
After a review of the physiology in the formation and degradation of cutaneous elastic tissue, we describe the clinicopathologic disorders characterized by increased and decreased cutaneous elastic tissue. Cutaneous disorders characterized by increased and/or abnormal elastic tissue in the dermis include elastoma, also named nevus elasticus, dermatosis lenticularis disseminata, pseudoxanthoma elasticum, late-onset focal dermal elastosis, linear focal elastosis, elastoderma, elastofibroma dorsi, and elastosis perforans serpiginosa. In some of these conditions, the specific histopathologic diagnosis may be rendered with hematoxylin-eosin stain, whereas in other ones special elastic tissue stains are necessary to demonstrate the anomalies. Cutaneous disorders characterized by decreased dermal elastic tissue include nevus anelasticus, papular elastorrhexis, perifollicular elastolysis, anetoderma cutis laxa, postinflammatory elastolysis and cutis laxa, white fibrous papulosis of the neck, pseudoxanthoma elasticum-like papillary dermal elastolysis, and mid dermal elastolysis. In most of these conditions, the histopathologic anomalies are only seen with elastic tissue stains, and cutaneous biopsies of these processes stained with hematoxylin-eosin show appearance of normal skin. The diagnosis of some of these disorders characterized by increased or decreased elastic dermal tissue should be followed by general exploration of the patient to rule out associated severe systemic anomalies, and in some cases, a genetic counseling should be offered to the family.
Background:
Annular elastolytic giant cell granuloma (AEGCG), a rare entity first described in 1979, is very similar to O'Brien actinic granuloma (AG), first described in 1975. Since then, many cases have been published under one or other of the two names. We performed a single-centre histopathology study to identify the distinguishing features and determine whether there was any objective difference between AEGCG and AG.
Patients and methods:
Cases classed as AEGCG or AG at the dermatopathology laboratory in Strasbourg were included and analysed using haematoxylin-eosin, orcein and Alcian blue staining. The diagnosis was made in the event of granuloma rich in multi-nucleated giant cells and reduction or disappearance of elastic tissue. Clinical data were collected from the analysis requests and clinical files.
Results:
We identified 73 cases: 12 classed as AEGCG and 61 classed as AG. Mean age was 60.5 years with a sex ratio of 0.55. The duration of the disease ranged from 8 days to 17 years. A single lesion was seen in 52% of cases with multiple lesions in the remaining cases. Lesions measured between 0.3 and 10cm and exhibited a predilection for photo-exposed areas, chiefly on the head, neck and upper limbs. In most cases, an annular erythematous edge was seen together with a light centre, and slow centrifugal spread. The diagnosis was made by a clinician in only 5.5% of cases. These granulomas were chiefly in the superficial and mid dermis and only rarely deep, and contained numerous giant cells with a constant contingent of lymphocytes, but plasma cells were also seen in half of the cases. Orcein staining revealed marked decrease or total disappearance of elastic tissue within the granulomatous area together with elastophagocytosis in practically all images. More rarely, there was evidence of necrobiosis, palisading granuloma, vascular involvement or orcein-stained asteroid bodies. There were no notable clinical or histological differences between the cases initially classed as AEGCG or AG.
Discussion:
AEGCG is a separate entity from annular granuloma that must be assimilated with O'Brien actinic granuloma. The central role of elastic fibres seems clear, but that of ultraviolet radiation remains more controversial. The term annular elastolytic giant cell granuloma seems more appropriate since it is more descriptive and carries no physiopathological implications. There appears to be a significant association with sarcoidosis and with diabetes in a minority of cases, thus warranting screening for these diseases.
