Annular Elastolytic Giant Cell Granuloma Associated with Polycythemia Vera
Abstract
Introduction: The authors describe a case of annular elastolytic giant cell granuloma (AEGCG) associated with polycythaemia vera. Both diseases resolved with treatment with hydroxyurea.
Case report: A 63-year-old man refers fatigue, nausea, right upper abdominal pain and marked weight loss for 4 months. During last month he presents multiple grouped erythematous skin papules and plaques in his back. He had a moderate increase of his spleen. The patient was diagnosed with polycythaemia vera (JAK 2 mutation positive in bone marrow). Skin biopsy revealed AEGCG. The patient was treated with hydroxyurea; there was normalization of hematologic changes and regression of skin lesions.
Discussion: AECG is, in most cases, an isolated cutaneous disease but it can be found in association with other systemic diseases. Our patient is the first described with and association of AEGCG and polycythaemia vera. The regression of both diseases with hydroxyurea suggests a possible relationship.
Downloads
References
Hanke CW, Bailin PL, Roenigk HH Jr. Annular elastolytic giant cell granuloma: A clinicopathologic study of five
cases and a review of similar entities. J Am Acad Dermatol. 1979; 1:413-21.
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.
Boneschi V, Brambilla L, Fossati S, Parini F, Alessi E. Annular elastolytic giant cell granuloma. Am J Dermatopathol. 1988; 10:224-8
Herron MD, Coffin CM, Vanderhooft SL. Annular elastolytic giant cell granuloma. Pediatr Dev Pathol. 2002;
:305-9.
Gutzmer R, Breuer K, Kiehl P, Kapp A, Werfel T. Successful therapy of annular elastolytic giant cell granuloma with fumaric acid esters. Dermatology. 2002; 205:421-4.
Özaya-Bayazit E, Büyükbabani N, Baykal C, Öztürk 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.
Ventura F, Vilarinho C, Duarte ML, Pardal F, Brito C: Two cases of annular elastolytic giant cell granuloma: Different response to the treatment. Dermatol Online J. 2010;16:11.
Watabe D, Akasaka T: Annular elastolytic giant cell granuloma developing on lesions of vitiligo. Int J Dermatol.
; 52:1458-60.
Limas C: The spectrum of primary cutaneous elastolytic granuloma and their distinction from granuloma annulare: A clinicopathological analysis. Histopathology. 2004; 44:277-82.
Misago N, Ohtsuka Y, Ishii K, Narisawa Y. Papular and reticular elastolytic giant cell granuloma: rapid spontaneous regression. Acta Derm Venereol. 2007; 87:89-90.
Lee Y-S, Vijayasingam S, Chan HL. Photosensitive annular elastolytic giant cell granuloma with cutaneous
amyloidosis. Am J Dermatol. 1989; 11:443-50.
Basak PY, Icke I, Akkaya VB, Basak K. Lack of response to isotretinoin in annular elastolytic giant cell granuloma. J Dermatol. 2004; 31:678-81.
Kelly BJ, Mrstik ME, Ramos-Caro FA, Iczkowski KA: Papular elastolytic giant cell granuloma responding to hydroxychloroquine and quinacrine. Int J Dermatol. 2004; 43:964-6.
Shoimer I, Wismer J. Annular elastolytic giant cell granuloma associated with temporal arteritis leading to blindness. J Cutan Med Surg. 2011; 15:293-7.
Merino de Paz N, Rodríguez-Martín M, Bustínduy MG, Martín-Herrera A, Noda-Cabrera A: Strict Anatomical
Colocalization of Vitiligo and Elastolytic Granulomas. Case Rep Dermatol. 2010; 2:13-7.
Kuramoto Y, Watanabe M, Tagami H: Adult T cell leukemia accompanied by annular elastolytic giant cell granuloma. Acta Derm Venereol. 1990; 70:164-7.
Boussault P, Tucker ML, Weschler J, Riaux A, Bervas-Quenehervé C, Quist D, Derancourt C: Primary cutaneous
CD4+ small/medium-sized pleomorphic T-cell lymphoma associated with an annular elastolytic giant cell granuloma. Br J Dermatol. 2009; 160:1119-38.
Garg A, Kundu RV, Plotkin O et al: Annular elastolytic giant cell granuloma heralding onset and recurrence
of acute myelogenous leukemia. Arch Dermatol. 2006; 142:532-3.
Rongioletti F, Baldari M, Burlando M, Parodi A. Papular elastolytic giant cell granuloma: report of a case
associated with monoclonal gammopathy and responsive to topical tacrolimus. Clin Exp Dermatol. 2010;
:145-8.
Asahina A, Shirai A, Horita A, Saito I. Annular elastolytic giant cell granuloma associated with prostate carcinoma: demonstration of human metalloelastase (MMP-12) expression. Clin Exp Dermatol. 2012; 37:70-2.
Can B, Kavala M, Türkoğlu Z, Zindancı I, Topaloğlu F, Zemheri E. Successful treatment of annular elastolytic giant cell granuloma with hydroxychloroquine. Int J Dermatol. 2013; 52:509-11.
Klemke C-D, Siebold D, Dippel E, Hildenbrand R, Bleyl U, Goerdt S. Generalised annular elastolytic giant cell
granuloma. Dermatology. 2003; 207:420-2.
Espiñeira-Carmona MJ, Arias-Santiago S, Aneiros-Fernández J, Fernández-Pugnaire MA, Naranjo-Sintes R,
Aneiros-Cachaza J. Annular erythematous papules in the neckline. Dermatol Online J. 2011; 17:7.
Morita K, Okamoto H, Miyachi Y. Papular elastolytic giant cell granuloma: a clinical variant of annular elastolytic
giant cell granuloma or generalized granuloma annulare? Eur J Dermatol. 1999; 9:647-9.
All articles in this journal are Open Access under the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC 4.0).