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An. Bras. Dermatol. vol.86 no.4 supl.1 Rio de Janeiro July/Aug. 2011
Annular elastolytic giant cell granuloma - case report*
Rossana Ruth da Veiga GonçalvesI; Mario Fernando Ribeiro de MirandaII; Fernanda de Oliveira VianaIII; Luíza Helena dos Santos CavaleiroIII; Diana Mendes da FonsecaIII
IAssociate Professor, Dermatology Service, Federal University of Pará (UFPA) - Lecturer, Dermatology Residency, Federal University of Pará (UFPA) and Fundação Santa Casa de Misericórdia do Pará (FSCMPA), Belém, PA, Brazil
IIDermatopathologist accredited by the International Committee for Dermatopathology (ICDP) and the Union Européenne des Médecins Spécialistes (UEMS). Associate Professor of Dermatology, Federal University of Pará (UFPA). Head of the Dermatopathology Laboratory in the Dermatopathology Service, ICS-UFPA, Belém, PA, Brazil
IIIResident Dermatologist, Dermatology Service, Federal University of Pará (UFPA), Belém, PA, Brazil
Annular elastolytic giant cell granuloma is a rare granulomatous skin disease characterized histologically by phagocytosis of elastic fibers by multinucleated giant cells and clinically by annular patches with elevated borders and atrophic centers found mainly on sun-exposed skin. The pathogenesis of the disease is controversial. We report the case of a 44-year-old woman with classical clinical and histological findings of the disease whose condition improved when hydroxychloroquine was administered over a four-week period.
Keywords: Elastic tissue; Granuloma; Granuloma annulare; Histology
Annular elastolytic giant cell granuloma (AEGG) is a rare dermatosis belonging to an interesting group of diseases that affect the elastic fibers in the skin and are clinically and/or histopathologically similar to granuloma annulare (GA) and necrobiosis lipoidica. 1 We describe the case of a 44-year-old patient with the characteristic clinical and histopathological findings of this disease.
A 44-year-old female patient from Pará presented with a one-year complaint of erythematous papules on the right arm that gradually increased in number and size without associated symptoms. She reported having used various topical and oral medications without any improvement. Dermatological examination revealed annular plaques with papular, erythematous borders and reddish-brown atrophic centers spread over the arms and upper torso, either in isolation or in clusters with an arabesque-like appearance (Figure 1). A diagnosis of AEGG was suspected, and a biopsy of the lesion was requested. Histopathological examination revealed changes in collagen morphology and staining (collagen fibers with a fragmented, amphophilic appearance); abundant histiocytes, some of which were multinucleated; the absence of elastic fibers in the granuloma on Verhoeff staining; and signs of elastophagocytosis (Figures 2, 3, 4 and 5). The clinical and histological findings confirmed the hypothesis of AEGG.
The relationship between GA and AEGG is the subject of controversy. Some authors believe that AEGG is a type of GA preferentially located on sunexposed skin, while others believe it to be an entity in its own right. 1, 2 It affects mainly middle-aged white women. 3 Lesions are usually isolated or clustered in annular plaques with raised borders and atrophic centers. However, atypical forms have also been described. 1 The condition is found mainly on sunexposed skin and is rarely described in covered areas. 1,3 Diagnosis is based on histopathological findings of a granulomatous reaction with elastolysis and phagocytosis of elastic fibers by multinucleated giant cells and the absence of elastic fibers in the center of the granuloma. 1,3,4 Elastophagocytosis is a non-specific phenomenon that can also be found in cases of generalized granuloma annulare and granulomatous slack skin. However, in AEGG there is a distinct zone of elastophagocytosis, and the quantity and distribution of giant cells differ from those observed in GA. In addition, in AEGG there is no necrobiosis of collagen or mucin deposition and elastosis is absent in some cases. 1 The pathogenesis of the condition is unknown. It has been suggested that ultraviolet radiation may cause changes in the antigenicity of elastic fibers, leading to a cellular immune response to these fibers. This theory is supported by immunohistochemical findings of an increased CD4/CD8 T-cell ratio. 5 Therefore, the elastophagocytosis and granulomatous reaction may be a reflex of the inflammatory process, resulting in a reduction in, and subsequent complete loss of, elastic fibers. 3 This pathological condition is normally confined to the skin. However, one case in which the eyes, lymph nodes and intestine were affected has been documented. 6 The disease follows a chronic course, but there have been reports of spontaneous remission. 7 In some cases it may be associated with diabetes mellitus. 8 Therapy options include clofazimine, chloroquine and systemic corticosteroids. 1,3,5,6 Notable in our case was the excellent response after one month of treatment with hydroxychloroquine in a daily dose of 400 mg.
1. Pock L, Blaková J, Caloudová H, Varjassyová R, Konkolová J, Hercogová. Annular elastolytic giant cell granuloma causes an irreversible disappearance of the elastic fibres. J Eur Acad Dermatol Venereol. 2004;18:365-8. [ Links ]
2. Ackerman AB, Chongchitnant N, Sanchez J, Guo Y, Bennin B, Reichel M, et al. Histologic diagnosis of inflammatory skin diseases. An algorithmic method based on pattern analysis. 2th ed. Baltimore: Williams & Wilkins; 1997. p.50-3. [ Links ]
3. Zkaya-bayazit EO, Kbabani NY, Baykal C, Ozturk A, Okçu¸ M, Soyer HP. Annular elastolytic giant cell granuloma: sparing of a burn scar and successful treatment with chloroquine. Br J Dermatol. 1999;140:525-30. [ Links ]
4. Ortel B, Rappersberger K, Konrad K. Middermal elastolysis in an elderly man with evidence of elastic fiber phagocytosis. Arch Dermatol. 1992;128:88-90. [ Links ]
5. Vehring KH, Bonsmann G, Brocker EB, Hamm H. Das anulare elastolytische Riesenzellgranulomeine - eine Differentialdiagnose kutaner Granulomatosen. Hautarzt. 1991;42:391-5. [ Links ]
6. Kurose N, Nakagawa H, Iozumi K, Nogita T, Furue M, Ishibashi Y. Systemic elastolytic granulomatosis with cutaneous, ocular, lymph nodal, and intestinal involvement. Spectrum of annular elastolytic giant cell granuloma and sarcoidosis. J Am Acad Dermatol. 1992;26:359-63. [ Links ]
7. Hermes B, Haas N, Czarnetzki BM. Annular elastolytic giant cell granuloma with a spontaneous healing tendency. Hautarzt. 1995;46:490-3. [ Links ]
8. Almeida Jr HL, Jannke HA, Amaral, MG. Granuloma anular elastolítico gigantocitário. An Bras Dermatol. 1998;73:266-8. [ Links ]
Mailing address: Received on 28.12.2010. * Study carried out in the Dermatology Service, Federal University of Pará (UFPA), Belém, PA, Brazil.
Fernanda de Oliveira Viana
Rua Tibúrcio Cavalcante, 2777 - Apartamento 702 Dionísio Torres
60125-101 Fortaleza CE Brazil
Approved by the Advisory Board and accepted for publication on 28.12.2010.
Conflict of interest: None
Financial funding: None
Received on 28.12.2010.
* Study carried out in the Dermatology Service, Federal University of Pará (UFPA), Belém, PA, Brazil.