Annular Elastolytic Giant-Cell Granuloma

Annular elastolytic giant cell granuloma is a granulomatous process that presents as slowly growing annular papules and plaques on sun-exposed skin.

Elastolytic granulomas include a group of granulomatous conditions - Actinic granuloma ; Atypical necrobiosis lipoidica of face and scalp ; Miescher's granuloma ; Granuloma Multiforme.

Actinic granuloma (O'Brien).J Cutan Pathol. 1988 Apr;15(2):66-74

Twelve cases of actinic granuloma (O'Brien) are reported. It is concluded that actinic granuloma is a specific disease characterized by clinical lesions indistinguishable from granuloma annulare, but occurring on solar damaged skin. Histologically, elastic tissue is destroyed by the granulomatous process in actinic granuloma, but not in granuloma annulare. It would appear that actinic granuloma, granuloma multiforme, necrobiosis lipoidica of the face and scalp (Wilson-Jones) and Miescher's granuloma of the face (Mehregan and Altman) are the same disease.

Annular elastolytic giant cell granuloma. A clinicopathologic study of five cases and a review of similar entities.J Am Acad Dermatol. 1979 Nov;1(5):413-21

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)."

Annular elastolytic giant cell granuloma was first described in patients in the fourth and fifth decades of life.

These lesions are primarily distributed on the head and neck.

Microscopic features include a granulomatous reaction with giant cells at the annular rim and centrally a loss of elastic fibers or the presence of solar elastotic material.

Actinic granuloma is a term coined by O'Brien in 1975 to describe a cutaneous process characterized by multiple annular lesions located on sun-exposed skin areas. The histopathology is defined by the presence of multinucleated giant cells containing elastic fibres at the lesion edge, and the absence of these fibres at the lesion centre. Image Link (Tulane)

Visit: Granulomatous Reaction Pattern of the Skin ; Granuloma Annulare ; Necrobiosis Lipoidica ; Cutaneous Sarcoidosis .

Annular elastolytic giant cell granuloma: a case report located in non-sun exposed areas. Actas Dermosifiliogr. 2006 Oct;97(8):533-5

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.

A reticular variant of elastolytic giant cell granuloma.Clin Exp Dermatol. 2006 Jan;31(1):42-4

An 80-year-old man presented with a distinctive reticular form of elastolytic giant cell granuloma (EGCG). This represents a rare subset of EGCG, thus belonging to a larger group of granulomatous skin diseases with poorly understood pathogenesis. The patient exhibited numerous erythematous to brownish, sharply demarcated patches of reticulate appearance with partial central atrophy and nonelevated margins, which involved the trunk and especially chest, shoulders, upper back and arms. Skin biopsy revealed a granulomatous infiltrate with multinucleate histiocytes containing remnants of elastic fibres in conjunction with a significant loss of elastic fibres throughout the dermis. There is little published information on treatment, which remains unsatisfactory.

The spectrum of primary cutaneous elastolytic granulomas and their distinction from granuloma annulare: a clinicopathological analysis.Histopathology. 2004 Mar;44(3):277-82.

AIMS: Actinic granuloma (AG) and annular elastolytic giant cell granuloma (AEGCG) are terms commonly applied to cutaneous lesions characterized by elastolysis, elastophagocytosis and a multinucleated cell infiltrate. The aim of this study was to clarify the relationship of these lesions to granuloma annulare (GA) which they resemble clinically. METHODS AND RESULTS: The clinicopathological data of 20 patients whose biopsies showed the histology of elastolytic granuloma as well as those of previously published cases with identical tissue changes were analysed to assess differences between AG, AEGCG and GA. The tissue changes of AG and AEGCG are identical and differ substantially from GA because of the predominant elastolysis and elastophagocytosis in the absence of necrobiosis and palisading granuloma. Actinic and/or thermal injury could be elicited frequently in the patient's history, but not always. CONCLUSIONS: AG may be considered as an aetiopathological subdivision of AEGCG. Patients with biopsies showing both GA and AEGCG are occasionally encountered. AEGCG is easily differentiated from incidental elastolysis which may be seen in a variety of aetiologically unrelated processes.

Elastolytic granulomas of the legs in familial visceral lymphangiodysplasia with chronic lymphedema.J Dtsch Dermatol Ges. 2004 Sep;2(9):766-9

Annular elastolytic giant cell granuloma is a rare skin condition of unknown origin characterized by giant cell granulomas with elastophagocytosis and loss of elastic tissue. Sun-exposed areas are most commonly affected. We report on an unusual case of elastolytic granulomas developing in chronic lymphedema of the legs in a female patient with visceral lymphangiodysplasia.

Annular elastolytic giant cell granuloma causes an irreversible disappearance of the elastic fibres. J Eur Acad Dermatol Venereol. 2004 May;18(3):365-8.

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.

Actinic granuloma is a unique and distinct entity: a comparative study with granuloma annulare.Am J Dermatopathol. 2002 Jun;24(3):209-12

Since the initial description of actinic granuloma (AG), debate has continued over whether it should be considered a specific condition or simply granuloma annulare (GA) located in sun-exposed areas of skin. We conducted a case-control study to clarify this issue. Twenty cases given the diagnosis of AG between 1991 and 2001 were retrieved from our archives. We applied the following inclusion criteria: extensive loss of elastic tissue in or at the side of the granuloma, and elastophagocytosis. Sixteen cases of GA that involved sun-exposed and non-sun-exposed sites, 8 cases from each group, were randomly selected as controls. Histologic parameters were quantitated on hematoxylin-eosin, Verhoeff van Gieson, and Alcian blue stains for each case. Results were statistically analyzed by SPSS program version 9. Fourteen cases of AG met our inclusion criteria. Presence of mucin, occurrence of multinucleated giant cells, and the type of granulomata were of high statistical significance (p < 0.01) in distinguishing the two entities. We also found that the location of the granulomata in these conditions is different and of statistical significance (p < 0.05). Based on histomorphology, we believe that AG should be considered a separate, independent condition and should be distinguished from GA even in sun-exposed areas of skin.

Actinically degenerate elastic tissue is the likely antigenic basis of actinic granuloma of the skin and of temporal arteritis.J Am Acad Dermatol. 1999 Feb;40(2 Pt 1):214-22

Staining technique is paramount for detecting and assessing the severe degeneration that occurs in the elastic tissues of the skin and its arteries in response to prolonged exposure to actinic radiation. With a selective "controlled" hematoxylin-and-eosin stain, actinically damaged ("elastotic") elastic tissue stains blue, as Unna described, and contrasts with normal and simply hyperplastic elastic tissue, which stains red. "Special" elastic stains such as Orcein and Verhoeff do not demonstrate this difference. When resorptive (elastolytic) giant cell reactions develop in relation to actinically degenerate elastic tissue of the skin, the papules that arise tend to form expanding, annular rings. A previously used and appropriate name for these autoimmune lesions in the skin is actinic granuloma because this name highlights the likely actinic origin and pathogenesis of many such lesions. Granulomatous inflammation in connection with actinically degenerate internal elastic lamina appears to be the basis of temporal arteritis. Actinic granulomas may occur in the skin concurrently with temporal arteritis. A recent study of temporal arteritis strongly relates its elastic tissue changes to those of "accelerated" atherosclerosis.

Annular elastolytic giant cell granuloma--actinic granuloma?Clin Exp Dermatol. 1996 Jan;21(1):51-3

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 foot. Biopsies from both areas revealed a mid-dermal inflammatory infiltrate with many giant cells engulfing elastic fibres. The current nosological situation of this process is discussed. Actinic granuloma (AG) is a term coined by O'Brien in 1975 to describe a cutaneous process characterized by multiple annular lesions located on sun-exposed skin areas. The histopathology is defined by the presence of multinucleated giant cells containing elastic fibres at the lesion edge, and the absence of these fibres at the lesion centre. The nosology of this disease and the role of actinic exposure as a triggering factor for it has been much discussed in recent years. We report a new case of AG not limited to photo-exposed areas.

Actinic granuloma. A clinical, histopathologic, and immunocytochemical study.Arch Dermatol. 1986 Jan;122(1):43-7

Two cases of actinic granuloma are described with emphasis on distinctive clinical and histopathologic features, including immunoperoxidase staining for lysozyme and immunophenotyping of mononuclear leukocytes. Actinic granuloma presents in chronically sun-damaged skin as normally colored to erythematous papules that coalesce to form centrifugally enlarging annular patterns. By light microscopy, a granulomatous infiltrate of giant cells and histiocytes is seen to be intimately related to the presence of elastotic fibers in the upper dermis. Selective localization of lysozyme in the giant cells of the granuloma is apparent by a tertiary antibody immunoperoxidase technique. Determination of mononuclear leukocyte subsets with monoclonal antibodies reveals a predominance of helper T cells in the lymphocytic infiltrate associated with the granuloma. It is postulated that actinic granuloma represents a cell-mediated immune response to weakly antigenic determinants on actinically altered elastotic fibers.

O'Brien's actinic granuloma, Three cases (author's transl). Ann Dermatol Venereol. 1982;109(2):135-49

We report three cases of 54, 58 and 65-year-old patients presenting annular lesions with centrifugal migratory extension. They occur exclusively on sun-exposed areas. In two cases, the lesions were multiple. In the third case, the lesion was single and mimicked an erythema annulare centrifugum. The past history ranged from 18 months to 8 years. Microscopic examination of the central part showed a disappearance of elastic fibers in upper reticular dermis. Examination of the ring showed in upper reticular dermis an histiocytic granulomatous infiltration with many giant cells, lymphocytes and patterns of elastic fibers phagocytosis. Similar features were found by electronmicroscopy. These three cases illustrate the typical features of O'Brien's actinic granuloma. Relationship between this actinic granuloma and granuloma annulare occurring on sun-exposed areas on one side, and necrobiosis lipoidica, Miescher's granuloma and granuloma multiforme on the other side, are discussed. On the basis of some clinical and histological patterns, the autonomy of O'Brien's actinic granuloma appears to be established.