Hipopiyon belirtisi saptanan bir paraneoplastik pemfigus olgusu ve literatür incelemesi

Paraneoplastik Pemfigus (PNP) ağrılı mukozal erozyonlar ve polimorfik deri lezyonları ile karakterize, sıklıkla neoplazilerle ilişkili bir otoimmun büllöz hastalıktır. Hipopiyon belirtisi kaynak kitaplarda sadece subkorneal püstüler dermatozda tanımlanmakta olan püyün alt yarıda toplandığı püstüllere dönüşen küçük veziküllerin varlığını tanımlayan bir belirtidir. Bununla birlikte literatürde 20’ye yakın otoimmun büllöz hastalığı olgusunda hipopiyon belirtisi bildirilmiştir. Bu belirtinin şiddetli inflamasyonun sonucu olarak çeşitli büllöz hastalıklarda oluşabileceği iddia edilmiştir. Burada hipopiyon belirtisi saptadığımız PNP’li bir olgu sunulmakta ve bu belirtinin izlendiği literatürdeki vakalar gözden geçirilmektedir.

A case of paraneoplastic pemphigus with hypopion sign and literature review

Paraneoplastic pemphigus (PNP) is an autoimmune bullous disease characterized by painful mucosal erosions and polymorphic skin lesions which is usually associated with neoplasias. Hypopyon sign typically defines the presence of the small vesicles turning into pustules with pus at the bottom half and has been descript solely for subcorneal pustular dermatosis in standard texts. However, it has been reported in approximately 20 cases of autoimmune bullous disorders in the literature. It has been suggested that this sign can occur in several bullous eruptions as a result of severe inflammation. Herein a case of PNP is being reported and the cases in the literature with hypopyon sign are being reviewed.

Kaynakça

Eming R, Sticherling M, Hofmann SC, et al.Guidelines for the treatment of pemphigus vulgaris/foliaceus and bullous pemphigoid. J Dtsch Dermatol Ges 2015;13:833-844.

Ruocco E, Wolf R, Ruocco V, Brunetti G, Romano F, Lo Schiavo A. Pemphigus: associations and management guidelines: facts and controversies. Clin Dermatol 2013;31:382-390.

Yazganoğlu KD, Bavbek S, Büyükbabani N,Baykal C. Paraneoplastic pemphigus in a patient with B cell nonhodgkin lymphoma. Türkderm 2007;41:25-27.

Rivollier C, Vaillant L, Machet MC, et al. Paraneoplastic pemphigus: a pustular form during chronic lymphoid leukemia. Ann Dermatol Venereol 2001;128:644-648.

Mahe A, Flageul B, Cısse I, Keita S, Robin P. P. Pemphigus in Mali: a study of 30 cases. Br J Dermatol 1996;134:114-119.

Pacheco D, Lopes L, Soares-Almeida L, Marques MS, Filipe P. “Half-half” blisters in bullous pemphigoid successfully treated with adjuvant high-dose intravenous immunoglobulin. Acta Derm Venereol 2012;21:59-61.

Singh S, Gupta S, Chaudhary R. Hypopyon sign in pemphigus vulgaris and pemphigus foliaceus. Int J Dermatol 2009;48:1100-1102.

Frew JW, Murrell DF. Paraneoplastic pemphigus (paraneoplastic autoimmune multiorgan syndrome): clinical presentations and pathogenesis. Dermatol Clin 2011;29:419-425.

Zimmermann J, Bahmer F, Rose C, Zillikens D, Schmidt E. Clinical and immunopathological spectrum of paraneoplastic pemphigus. J Dtsch Dermatol Ges 2010;8:598-605.

Cervini AB, Tosi V, Kim SH, et al. Paraneoplastic pemphigus or paraneoplastic autoimmune multiorgan syndrome report of 2 cases in children and a review of the literature. Actas Dermosifiliogr 2010;101:879-886.

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