环状弹性组织溶解性巨细胞肉芽肿1例

患者男,50岁。双手背部及颈部多发环形红斑2年余。颈部皮损组织病理示:真皮浅层见较多的多核巨细胞浸润,可见吞噬,特殊染色显示弹性纤维碎裂和吞噬。诊断:环状弹性组织溶解性巨细胞肉芽肿。予中药活血散结治疗,症状较前好转。     

Adult T cell leukemia accompanied by annular elastolytic giant cell granuloma

We report a 74-year-old Japanese patient with adult T-cell leukemia who concurrently developed annular elastolytic giant cell granuloma. Initially, itchy granulomatous lesions developed on his face, nape of the neck and dorsa of the hands, but gradually erythematous plaques appeared on the back and lower limbs. The histology of the granulomatous lesions revealed coexistence of an epithelioid cell granuloma with giant cells that phagocytosed elastic fibres in the dermis and Pautrier's microabscesses in the overlying epidermis. Subsequent sequential histological studies of an erythematous plaque revealed the development of granulomatous changes in pre-existing lymphomatous lesions. Laboratory data revealed the presence of antibody to human T cell leukemia/lymphoma virus I and 14,200 white cells/mm3 in the peripheral blood with 2% atypical lymphocytes which eventually amounted to 30%, one month before his death.     

Papular elastolytic giant cell granuloma: a clinical variant of annular elastolytic giant cell granuloma or generalized granuloma annulare?

A 71-year-old man with asymptomatic red papules on the trunk and upper arms was reported as a case of papular elastolytic giant cell granuloma. A skin biopsy specimen from a papule on the back showed similar findings to those of annular elastolytic giant cell granuloma. However, centrifugal annular lesions were not clinically observed. He was successfully treated with tranilast and topical steroids.     

Annular elastolytic giant cell granuloma. A clinicopathologic study of five cases and a review of similar entities

Five patients with annular lesions of the face, scalp, and other exposed surfaces were studied using the radial triple zone biopsy technic. Interestingly, one of the patients had necrobiosis lipoidica diabeticorum (NLD) and another had systemic sarcoidosis. Clinically, the lesions were annular patches with erythematous borders and hypopigmented centers. The histopathology of the lesions showed many multinucleated giant cells, (often with prominent asteroid bodies), histiocytes, lymphocytes, scattered epithelioid cells, total lysis of elastic tissue, no necrobiosis, and absence of both mucin and lipid. This entity can be differentiated from granuloma annulare (GA), NLD, and cutaneous sarcoidosis. It is identical with several previously described entities; we propose a more appropriate term: "annular elastolytic giant cell granuloma (AEGCG)."     

Lack of response to isotretinoin in annular elastolytic giant cell granuloma

Annular elastolytic giant cell granuloma is a rare disease characterized by annular configurated lesions with typical histopathologic findings. We report a case of annular elastolytic giant cell granuloma in a 50-year-old male patient. Treatment modalities for this disease are limited and mostly unsatisfactory, although isotretinoin was found to be effective in a single case. However, systemic isotretinoin treatment of 12 weeks' duration was of no benefit in the presented case.     

Photosensitive annular elastolytic giant cell granuloma with cutaneous amyloidosis

Annular erythematous indurated plaques with central clearing developed on sun-exposed areas of a diabetic patient. The lesions were aggravated by sun exposure. Histological examination revealed features as described in annular elastolytic giant cell granuloma. In addition, subepidermal amyloid deposits were identified. Though a coincidental occurrence cannot be excluded, a possible pathogenetic relationship between the two conditions is postulated. It is believed that they are caused by actinic damage to skin altered by diabetes.     

环状弹性纤维溶解性巨细胞肉芽肿

报告10例环状弹性纤维溶解性巨细胞肉芽肿.男4例,女6例,年龄29～77岁.大多数患者皮损表现为大小不一的环状斑块,中央轻度萎缩,遗留色素减退,周围有一轻度隆起的红色边缘.皮损最常见于双手背和躯干.组织病理检查显示真皮中上层的间质中有组织细胞、多核巨细胞和淋巴细胞浸润,皮损中央弹性纤维减少或缺失,并且可见多核巨细胞吞噬弹性纤维的碎片;未见渐进性坏死和黏蛋白沉积.该文报告的10例患者临床表现和组织病理学改变均符合环状弹性纤维溶解性巨细胞肉芽肿的诊断.     

Annular elastolytic giant cell granuloma: an unusual case with papular lesions.

A 63-year-old Japanese woman with multiple annular lesions on the bilateral abdomen developed numerous papules on the upper part of the back and forearms. A skin biopsy specimen taken from a papule showed the findings of annular elastolytic giant cell granuloma.     

Generalized elastophagocytic granuloma.

The case of an eighty-three-year-old woman with the sudden onset of a generalized pruritic eruption is reported. The skin lesions resembled disseminated subacute lupus erythematosus on clinical examination, but actinic granuloma or annular elastolytic giant cell granuloma was seen in biopsy specimens of the lesions. Our case was differentiated from generalized granuloma annulare by the distinct zoning of elastolysis and the distribution of giant cells.     

Annular elastolytic giant cell granuloma—actinic granuloma?

A 56-year-old woman with annular elastolytic giant cell granuloma is reported. She had annular, slowly growing lesions on sun-exposed areas and the dorsum of one font. Biopsies from both areas revealed a mid-dermal inflammatory infiltrate with main giant cells engulfing elastic fibres. The current nosological situation of this process is discussed.     

Annular elastolytic giant cell granuloma: a case report located in non-sun exposed areas

Annular elastolytic giant cell granuloma (AEGCG) is an uncommon entity clinically characterized by erythematous annular plaques with atrophic and hypopigmented center, that predominates in sun-exposed zones. The histology shows a granulomatous infiltrate without palisading image, made up of lymphocytes, histiocytes and giant cells, with phagocytosis of elastic fibers, without necrobiosis or mucin deposit. We present the case of a male patient with atypical clinical manifestation on the non-sun exposed skin and AEGCG characteristic histology.     

儿童泛发型环状弹性组织溶解性巨细胞肉芽肿

报告1例儿童泛发型环状弹性组织溶解性巨细胞肉芽肿.患儿男,13岁.全身红色斑块3年,微痒,日晒后加重,皮损呈环状逐渐向外离心性扩展.皮肤科检查:躯干及四肢泛发指头至双手掌大的不规则环状斑块,边缘红肿隆起,中央萎缩,面部可见散在黄豆至蚕豆大虫蚀状萎缩性斑块.皮损组织病理检查:真皮全层弥漫性以组织细胞、多核巨细胞为主的肉芽肿浸润.弹性纤维染色示肉芽肿浸润部位弹性纤维缺失或仅有少量碎片.诊断:环状弹性纤维组织溶解性巨细胞肉芽肿.     

Dermoscopy of annular elastolytic giant cell granuloma

Annular elastolytic giant cell granuloma is an uncommon granulomatous cutaneous disease that usually affects sun-exposed skin. Non-scarring alopecia is a possible presentation. Although histopathology is mandatory for the diagnosis, dermoscopy may help to narrow down the clinical differential diagnosis. The authors report a case of annular elastolytic giant cell granuloma in the scalp of a female adult patient, showing multiple yellowish/orange follicular dots in a diffuse erythemato-whitish background in the dermoscopy.     

Annular elastolytic giant cell granuloma: sparing of a burn scar and successful treatment with chloroquine

Annular elastolytic giant cell granuloma is a rare granulomatous skin disease characterized by phagocytosis of elastic fibres by multinucleated giant cells. Lesions are either solitary or grouped in a few annular patches with elevated borders and central atrophy. Sun-exposed areas are more commonly involved than covered skin. The pathogenesis of the disease is still controversial. We report a 72-year-old fair-skinned woman with unusual clinical findings. An irregularly shaped erythematous plaque covered the entire face, and hundreds of lichenoid papules were present on both sun-exposed and covered areas which gradually evolved into annular lesions of about 0.5-1 cm in diameter. Sparing of an old burn scar and a nearly complete lack of elastic fibres in the scar site were noted, illustrating the presumed importance of dermal elastic tissue in the pathogenesis. The course of the disease is chronic. Several treatments have been tried, with variable success. In our patient, improvement was achieved with chloroquine over a period of 16 weeks.     

Papular elastolytic giant cell granuloma: report of a case associated with monoclonal gammopathy and responsive to topical tacrolimus

Papular elastolytic giant cell granuloma is an unusual variant of annular elastolytic giant cell granuloma. Its rarity makes the assessment of the real efficacy of any treatment difficult, as spontaneous remission is possible. We report a case whose interest, besides the rarity of the occurrence, rests in the pure papular expression of the clinical features, the association with a monoclonal gammopathy and the apparent efficacy of topical tacrolimus.     

Annular erythematous papules in the neckline

A 45-year-old woman with personal history of hypertension presented with an erythematous lesion in the neckline for a year and with a progressive growth. A physical examination revealed an annular lesion with erythematous papules in the edge. Histological exam showed phagocytosis of elastic fibers by multinucleated cells compatible with annular elastolytic giant-cell granuloma. The patient did not present any other associated systemic manifestation. Treatment with tacrolimus 0.1 percent ointment was prescribed with a very good response after two months.     

Annular Elastolytic Giant Cell Granuloma: Response to Cyclosporin A

A case of annular elastolytic giant cell granuloma with a good response to cyclosporin A is reported. A 62-year-old man developed multiple annular patches on the trunk with elevated and indurated borders. Biopsy specimens from the border showed granulomatous inflammation in the mid dermis with phagocytosis of elastic fibers by giant cells. Biopsy specimens from the center showed dense collagen formation without inflammation. Immunological investigation of perivascular infiltrating cells in the lesions revealed a predominance of CD4+ cells over CD8+ cells. Our case showed a good response to cyclosporin A (5 mg/kg/day) for eight weeks. There were no adverse effects and no recurrences for one month after discontinuation of cyclosporin A.     

Annular elastolytic giant cell granuloma causes an irreversible disappearance of the elastic fibres

A 67-year-old man presented with grouped red papules with a smooth surface coalescing to relatively well-demarcated plaques on his left thigh, in the axillae and on the lateral parts of the trunk. The plaques were growing slowly, and the older ones had a frilled surface. A skin biopsy showed a zone of disappearance of the elastic fibres with a rim of giant multinuclear cells with fragments of the elastic fibres in their cytoplasm. This finding is typical of annular elastolytic giant cell granuloma (AEGCG). After corticosteroid therapy, the inflammation resolved, causing the frilled surface of the lesions due to the disappearance of the elastic fibres. Remission of the skin lesions lasted for 1.5 years. A second skin biopsy taken from the site of the previous lesion showed the absence of the elastic fibres, thus their phagocytosis was irreversible.     

环状弹力纤维溶解性巨细胞肉芽肿1例

患者女,23岁。面部红色萎缩性斑片1年。组织病理检查示真皮弥漫以组织细胞、多核巨细胞为主的肉芽肿性浸润。弹力纤维染色示肉芽肿浸润部位弹力纤维缺失、溶解,部分断裂。诊断:环状弹力纤维溶解性巨细胞肉芽肿。     

Annular elastolytic giant cell granuloma: an unusual case with lesions arising in non-sun-exposed areas

A 70-year-old man with a 2-year history of annular elastolytic giant cell granuloma associated with diabetes mellitus was reported. The lesions mainly developed in non-sun-exposed areas. Histologic examination revealed phagocytosis of elastic fibers by histiocytic cells. Immunoperoxidase staining for lysozyme disclosed positive reactivity within the cytoplasm of these histiocytic cells. Electron microscopic study also showed elastic fibers and numerous lipid-like substances in the cytoplasm of these cells. These findings indicate high phagocytolytic activity by these infiltrating cells. In our case, actinic damage was not considered to be a primary causative factor, and a possible pathogenesis was also discussed.