The Journal of Dermatology Vol. 18: 667-670, 1991
Annular Elastolytic Giant Cell Granuloma: An Unusual Case with Papular Lesions Hideyuki Kato, Yasuo Kitajima* and Hideo Yaoita* Abstract
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.
Key words:
annular elastolytic giant cell granuloma; papular lesion
Introduction The term annular elastolytic giant cell granuloma (AEGCG) was proposed by Hanke et al. (1); it is characterized by a focal consumption of elastic fibers engulfed with multinucleated giant cells and h istiocytes." Clinically, the lesions consist of annular plaques, which develop predominantly on sun-exposed areas of the body. We herein demonstrate an unusual case of AEGCG associated with papular lesions. Fig. 1. Annular lesions on abdomen.
Case Report A 63-year-old farmer presented with a one-year history of multiple annular lesions on the bilateral abdomen. These lesions had fairly clear raised margins with dark-red centers (Fig. 1); they enlarged and coalesced, resulting in circinate patterns. Simultaneously, normal colored papular lesions with depressed centers spread on both arms and the upper back (Fig. 2). These papular lesions resembled those of generalized granuloma annulare. Laboratory examinations including serum angiotensin converting enzyme and fasting plasma glucose showed normal values. However, the challenge with ingestion of 75 g of glucose revealed imReceivedJune 6, 1991; accepted for publication August 23, 1991. Department of Dermatology, Saiseikai Utsunomiya Hospital and *Jichi Medical School, Tochigi,Japan. Reprint requests to: H. Kato, Department of Dermatology, Saiseikai Utsunomiya Hospital, 4-17 Chuo-honcho, Utsunomiya, Tochigi 320,Japan.
FIg. 2. Discrete, normally colored papules on upper back. paired glucose tolerance. Serum levels of anti-Lymeborrelia antibodies showed 0.088 of IgG (normal range; less than 0.216) and 0.075 of IgM (normal range; less than 0.] 40), respectively. A chest X-ray film showed no abnormalities.
668
Kato et al
Histologic findings in the active border of an annular lesion include a focal disappearance of elastic fibers in the upper and mid dermis, as well as giant cells, epitheloid cells, and lymphocytes. Fragments of elastic fibers were engulfed within the multinucleated giant cells (Figs. 3 and 4). The small vessels in the middermis were surrounded by lymphocytes, but there was no definite evidence of vasculitis. Necrobiosis was absent, and there were no deposits of mucin or lipid. There were also no deposits of immunoglobulins or complements in the dermis with immunofluorescence methods. In the center of the annular lesions, no elastic materials were detected. Collagen fibers were intact in the dermis. Papular skin lesion on the arm revealed the same findings as did the annular lesion; both were consistent with AEGCG (Figs. 5 and 6). The lesions did not respond to treatment with intralesional corticosteroid (triamcinolone acetonide, 40 mg every 4 weeks for 3 months). Systemic corticosteroid (predonisolone) was begun at 20 mg a ~
Fig. 3. Focal disappearance of elastic fibers are seen in mid dermis. Some giant cells engulffragments ofelastic fibers. (Skin biopsy specimen from border of annular lesion, van Gieson stain; xlOO)
Fig. 4. Photomicrograph of same biopsy specimen shown in Figure 3 (van Gieson stain; x200). Multinucleated giant cells are digesting elastic materials.
669
Annular Elastolytic Giant Cell Granuloma
day orally for 4 weeks. The erythematous elevated borders and firm papules disappeared, leaving residual depressed plaques. However, the papular lesions on the arms reoccurred after the predonisolone was discontinued.
Discussion
Annular plaques on the face, neck, and arms with the findings of marked elastophagocytosis by giant cells and absence of elastic fibers in the dermis have been previously reported as two clinicopathological entities; O'Brien (2) reported a case of "actinic granuloma", but Hanke et al. (l) used the term "annular elastolytic giant cell granuloma" (AEGCG). AEGCG tends to accompany diabetes mellitus (1, 3, 4), sarcoidosis (l) and generalized granuloma annulare (2), and to occur on sun-exposed areas
~
Fig. 5. Elastic fibers are almost absent in upper reticular dermis. Patchy infiltrates with macrophages, multinucleated giant cells, and lymphocytes are seen throughout mid dermis. Some elastic fibers are phagocytized by giant cells. (Skin biopsy specimen from papular lesion, van Gieson stain; xIOO)
Fig. 6. Photomicrograph of same biopsy specimen shown in Figure 5 (van Gieson stain; x200). Giant cells contain fragments of elastic fibers.
670
Kato et al
of the body in many western countries, but to occur frequently on non-sun-exposed areas in Japan (4, 5). We have already reported that a 55-year-old man with multiple annular lesions on the trunk developed numerous papules on the upper parts of the back and forearms (6). Examination of a biopsy specimen taken from a papule revealed AEGCG. This case with papular lesions seems to be an unusual clinical variant ofAEGCG. It is generally thought that the initial exanthema of AEGCG consists of papules and small nodules with depressed centers; these papular lesions extend centrifugally and are arranged in arciform or serpiginous annular patterns. The annular eruption has erythematous raised borders and slightly depressed or hypopigmented-normal colored centers. It is of interest that the annular lesions mainly developed on non-sun-exposed areas of the body in our two patients, but their papular lesions were predisposed to occur on the sunexposed areas. In our first case, treatment with intralesional triamcinolone acetonide every 4 weeks resulted in a gradual involution of annular lesions within 3 months. His papular lesions on the forearms and upper back spontaneously regressed. However, in our more recent case, treatment with topical or intralesional corticosteroid has been absolutely ineffective. Oral predonisolone has been used successfully to treat both annular and papular lesions. We have already demonstrated four cases (6-8) (including this case) that reside on the westside of Ibaraki Prefecture in Japan, which suggests the possibility that these cases in Japan represent an endemic disease. From this point of view, we examined anti-Lyme-borrelia antibody titers in sera of two patients, but their titers were normal. Thus, this pattern is not correlated with Lyme disease.
The pathogenesis of AEGCG is very little known and controversial; "actinic injury as the primary event, followed by autoaggression to the damaged elastic fibers (2)", "cell mediated immunological response against a weakly antigenic determinant on altered elastic fibers (9)", "diabetes mellitus might be considered one possible causative factor (4)", and "actinic destruction of elastin in elastic tissue in the diabetic condition, particularly in sun-exposed areas (3)". AEGCG might be a distinct clinicopathologic entity which offers many further challenges.
Acknowledgment We thank Dr. Kawabata (Department of Clinical Pathology, School of Medicine, Nihon University) for measuring the anti-Lyrne-borrelia antibody titers.
References 1) Hanke CW, Bailin PL, Roenigk HH jr: Annular elastolytic giant cell granuloma,] Am Acad Dermatol, 1: 413-421, 1979. 2) O'Brien jP: Actinic granuloma, Arch Dermatol, HI: 460-466, 1975. 3) Lee YS, Vijayasingam S, Chan HL: Photosensitive annular elastolytic giant cell granuloma with cutaneous amyloidosis, Am] Dermaiopathol, H: 443-450, 1989. 4) Muramatsu T, Shirai T, Yamashina Y, Sakamoto K: Annular elastolytic giant cell granuloma: an unusual case with lesions arising in non-sun-exposed areas,] Dermatol (Tokyo), 14: 54-58, 1987. 5) Ishibashi A, Yokoyama A, Hirano K: Annular elastolytic giant cell granuloma occurring in covered areas, Dermatologica, 174: 293-297, 1987. 6) Kato H, Uyeki Y,Yaoita H: Papular lesions associated with annular elastolytic giant cell granuloma,] Am Acad Dermatol, 21: 398-400, 1989. 7) Kato H, Yaoita H: A case of annular elastolytic giant cell granuloma and actinic keratosis, Hifu-Rinsho (Tokyo), 30: 469-472, 1988. (in japanese) 8) Kato H, Uyeki Y: A case of annular elastolytic giant cell granuloma, ibid, 33: 486-487, 1991. (injapanese) 9) McGrae jD: Actinic granuloma; a clinical histopathologic and immunocytochemical study, Arch Dermatol, 122: 43-47, 1986.
Annular Elastolytic Giant Cell Granuloma: An Unusual Case with Papular Lesions Hideyuki Kato, Yasuo Kitajima* and Hideo Yaoita* Abstract
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.
Key words:
annular elastolytic giant cell granuloma; papular lesion
Introduction The term annular elastolytic giant cell granuloma (AEGCG) was proposed by Hanke et al. (1); it is characterized by a focal consumption of elastic fibers engulfed with multinucleated giant cells and h istiocytes." Clinically, the lesions consist of annular plaques, which develop predominantly on sun-exposed areas of the body. We herein demonstrate an unusual case of AEGCG associated with papular lesions. Fig. 1. Annular lesions on abdomen.
Case Report A 63-year-old farmer presented with a one-year history of multiple annular lesions on the bilateral abdomen. These lesions had fairly clear raised margins with dark-red centers (Fig. 1); they enlarged and coalesced, resulting in circinate patterns. Simultaneously, normal colored papular lesions with depressed centers spread on both arms and the upper back (Fig. 2). These papular lesions resembled those of generalized granuloma annulare. Laboratory examinations including serum angiotensin converting enzyme and fasting plasma glucose showed normal values. However, the challenge with ingestion of 75 g of glucose revealed imReceivedJune 6, 1991; accepted for publication August 23, 1991. Department of Dermatology, Saiseikai Utsunomiya Hospital and *Jichi Medical School, Tochigi,Japan. Reprint requests to: H. Kato, Department of Dermatology, Saiseikai Utsunomiya Hospital, 4-17 Chuo-honcho, Utsunomiya, Tochigi 320,Japan.
FIg. 2. Discrete, normally colored papules on upper back. paired glucose tolerance. Serum levels of anti-Lymeborrelia antibodies showed 0.088 of IgG (normal range; less than 0.216) and 0.075 of IgM (normal range; less than 0.] 40), respectively. A chest X-ray film showed no abnormalities.
668
Kato et al
Histologic findings in the active border of an annular lesion include a focal disappearance of elastic fibers in the upper and mid dermis, as well as giant cells, epitheloid cells, and lymphocytes. Fragments of elastic fibers were engulfed within the multinucleated giant cells (Figs. 3 and 4). The small vessels in the middermis were surrounded by lymphocytes, but there was no definite evidence of vasculitis. Necrobiosis was absent, and there were no deposits of mucin or lipid. There were also no deposits of immunoglobulins or complements in the dermis with immunofluorescence methods. In the center of the annular lesions, no elastic materials were detected. Collagen fibers were intact in the dermis. Papular skin lesion on the arm revealed the same findings as did the annular lesion; both were consistent with AEGCG (Figs. 5 and 6). The lesions did not respond to treatment with intralesional corticosteroid (triamcinolone acetonide, 40 mg every 4 weeks for 3 months). Systemic corticosteroid (predonisolone) was begun at 20 mg a ~
Fig. 3. Focal disappearance of elastic fibers are seen in mid dermis. Some giant cells engulffragments ofelastic fibers. (Skin biopsy specimen from border of annular lesion, van Gieson stain; xlOO)
Fig. 4. Photomicrograph of same biopsy specimen shown in Figure 3 (van Gieson stain; x200). Multinucleated giant cells are digesting elastic materials.
669
Annular Elastolytic Giant Cell Granuloma
day orally for 4 weeks. The erythematous elevated borders and firm papules disappeared, leaving residual depressed plaques. However, the papular lesions on the arms reoccurred after the predonisolone was discontinued.
Discussion
Annular plaques on the face, neck, and arms with the findings of marked elastophagocytosis by giant cells and absence of elastic fibers in the dermis have been previously reported as two clinicopathological entities; O'Brien (2) reported a case of "actinic granuloma", but Hanke et al. (l) used the term "annular elastolytic giant cell granuloma" (AEGCG). AEGCG tends to accompany diabetes mellitus (1, 3, 4), sarcoidosis (l) and generalized granuloma annulare (2), and to occur on sun-exposed areas
~
Fig. 5. Elastic fibers are almost absent in upper reticular dermis. Patchy infiltrates with macrophages, multinucleated giant cells, and lymphocytes are seen throughout mid dermis. Some elastic fibers are phagocytized by giant cells. (Skin biopsy specimen from papular lesion, van Gieson stain; xIOO)
Fig. 6. Photomicrograph of same biopsy specimen shown in Figure 5 (van Gieson stain; x200). Giant cells contain fragments of elastic fibers.
670
Kato et al
of the body in many western countries, but to occur frequently on non-sun-exposed areas in Japan (4, 5). We have already reported that a 55-year-old man with multiple annular lesions on the trunk developed numerous papules on the upper parts of the back and forearms (6). Examination of a biopsy specimen taken from a papule revealed AEGCG. This case with papular lesions seems to be an unusual clinical variant ofAEGCG. It is generally thought that the initial exanthema of AEGCG consists of papules and small nodules with depressed centers; these papular lesions extend centrifugally and are arranged in arciform or serpiginous annular patterns. The annular eruption has erythematous raised borders and slightly depressed or hypopigmented-normal colored centers. It is of interest that the annular lesions mainly developed on non-sun-exposed areas of the body in our two patients, but their papular lesions were predisposed to occur on the sunexposed areas. In our first case, treatment with intralesional triamcinolone acetonide every 4 weeks resulted in a gradual involution of annular lesions within 3 months. His papular lesions on the forearms and upper back spontaneously regressed. However, in our more recent case, treatment with topical or intralesional corticosteroid has been absolutely ineffective. Oral predonisolone has been used successfully to treat both annular and papular lesions. We have already demonstrated four cases (6-8) (including this case) that reside on the westside of Ibaraki Prefecture in Japan, which suggests the possibility that these cases in Japan represent an endemic disease. From this point of view, we examined anti-Lyme-borrelia antibody titers in sera of two patients, but their titers were normal. Thus, this pattern is not correlated with Lyme disease.
The pathogenesis of AEGCG is very little known and controversial; "actinic injury as the primary event, followed by autoaggression to the damaged elastic fibers (2)", "cell mediated immunological response against a weakly antigenic determinant on altered elastic fibers (9)", "diabetes mellitus might be considered one possible causative factor (4)", and "actinic destruction of elastin in elastic tissue in the diabetic condition, particularly in sun-exposed areas (3)". AEGCG might be a distinct clinicopathologic entity which offers many further challenges.
Acknowledgment We thank Dr. Kawabata (Department of Clinical Pathology, School of Medicine, Nihon University) for measuring the anti-Lyrne-borrelia antibody titers.
References 1) Hanke CW, Bailin PL, Roenigk HH jr: Annular elastolytic giant cell granuloma,] Am Acad Dermatol, 1: 413-421, 1979. 2) O'Brien jP: Actinic granuloma, Arch Dermatol, HI: 460-466, 1975. 3) Lee YS, Vijayasingam S, Chan HL: Photosensitive annular elastolytic giant cell granuloma with cutaneous amyloidosis, Am] Dermaiopathol, H: 443-450, 1989. 4) Muramatsu T, Shirai T, Yamashina Y, Sakamoto K: Annular elastolytic giant cell granuloma: an unusual case with lesions arising in non-sun-exposed areas,] Dermatol (Tokyo), 14: 54-58, 1987. 5) Ishibashi A, Yokoyama A, Hirano K: Annular elastolytic giant cell granuloma occurring in covered areas, Dermatologica, 174: 293-297, 1987. 6) Kato H, Uyeki Y,Yaoita H: Papular lesions associated with annular elastolytic giant cell granuloma,] Am Acad Dermatol, 21: 398-400, 1989. 7) Kato H, Yaoita H: A case of annular elastolytic giant cell granuloma and actinic keratosis, Hifu-Rinsho (Tokyo), 30: 469-472, 1988. (in japanese) 8) Kato H, Uyeki Y: A case of annular elastolytic giant cell granuloma, ibid, 33: 486-487, 1991. (injapanese) 9) McGrae jD: Actinic granuloma; a clinical histopathologic and immunocytochemical study, Arch Dermatol, 122: 43-47, 1986.
