Systemic elastolytic granulomatosis with cutaneous, ocular, lymph nodal, and intestinal involvement

Successful Therapy of Annular Elastolytic Giant Cell Granuloma with Fumaric Acid Esters

“…This is emphasized by the term 'actinic granuloma', which has been coined to describe a similar entity [5]. However, cases with systemic involvement [7] and with lesions in non-sun-exposed areas [6,8,9] as well as lesions with only moderate or absent solar elastosis [1, 10] have been described. Therefore, the term AEGCG appears to be more appropriate than actinic granuloma for the designation of this entity since other (unknown) factors than UV light altering elastic fibers might also play a role in the pathogenesis of this skin disease [6].…”

Granuloma Anular Elastolítico de Células Gigantes Associado a Policitemia Vera

“…[5][6][7] Esta teoria é suportada pelos achados imuno-histoquímicos que revelam um predomínio de linfócitos CD4 sobre os linfócitos CD8 nas lesões. [6][7][8] No entanto, a doença pode surgir em áreas cobertas [2][3][4][5][6][7][8][9][10][12][13]22 e, num caso foi descrita uma disseminação sistémica (pele, olhos, gânglios linfáticos e aparelho digestivo), 2 o que leva a pensar que podem existir outros fatores para além da radiação ultravioleta que alteram as fibras elásticas e podem desempenhar um papel na patogénese desta doença cutâ-nea. 5 Na maioria dos casos trata-se de uma dermatose 10,22 O nosso caso é, como foi referido, a primeira descrição de associação com a policitemia vera.…”

Generalised Annular Elastolytic Giant Cell Granuloma

“…In some patients the typical histological features were found in the mid and deeper dermis instead of the upper dermis; these variations occurred mainly in male Japanese patients. While in almost all patients the disease is confined to the skin, 1 unusual case was reported by Kurose et al [10] showing ocular, lymph node and intestinal involvement.…”