Havada malignite kokusu var: yaşlı erkekte yaygın erode, hemorajik ve likenifiye plaklar

Abstract

Paraneoplastik pemfigus (PNP), altta yatan neoplazi ile ilişkili nadir bir otoimmün mukokutanöz hastalıktır. Tipik olarak ağrılı mukozal erozyonlar ve koyu yamasal döküntülerle ile karakterizedir. Altmış altı yaşında erkek hasta ani başlayan kızarıklık, iştahsızlık, hazımsızlık, halsizlik ve açıklanamayan aşırı kilo kaybı şikayetleri ile dahiliye polikliniğine başvurdu. Kaşektik olan hastanın fizik muayenesinde her iki kol ve bacakta, el ve ayak sırtında yaygın aşınmış, eritematöz, ortası kalın, sarı-çekirdekli, likenifiye plaklar saptandı. Gövdede dağınık seboreik keratoz lezyonları da olan hastanın yüksek CA 19-9 düzeyi ile mikrositer anemisi vardı. Karın bilgisayarlı tomografisinde antrumda malign bir kitle görüldü. Mide kitlesinin endoskopik biyopsisinde diskohezif taşlı yüzük hücrelerinden oluşan az diferansiye adenokarsinom saptandı ve cilt biyopsisi paraneoplastik pemfigus ile uyumlu izlendi. Yapısal semptomlarla birlikte hızla gelişen cilt lezyonları olan hastalarda altta yatan maligniteler akılda tutulmalıdır.

Keywords

• Paraneoplastik pemfigus
• Leser Trelat
• gastrik adenokarsinom

Malignancy smells in the air: widespread eroded, hemorrhagic, and lichenified plaques in an older man

Abstract

Paraneoplastic pemphigus (PNP) is a rare autoimmune mucocutaneous disease associated with underlying neoplasia. It is typically characterized by painful mucosal erosions and dark, patchy skin eruptions. A 66-year-old male was admitted to the internal medicine outpatient clinic with complaints of suddenly started rashes, loss of appetite, dyspepsia, weakness, and unexplained gross weight loss. The patient was cachectic and physical examination revealed widespread eroded, erythematous, thick-middle, yellow-pitted, lichenified plaques on bilateral arms and legs and the hands and feet dorsum. Scattered seborrheic keratosis lesions on the trunk were also detected. He had microcytic anemia with elevated CA 19-9 measures. Abdomen computed tomography showed a malignant mass in the antrum. Endoscopic biopsy of the gastric mass revealed poorly differentiated adenocarcinoma composed of discohesive signet ring cells and skin punch biopsy was compitable with paraneoplastic pemphigus. In patients with rapidly developing skin lesions with constitutional symptoms, underlying malignancies should be kept in mind.

Keywords

• Paraneoplastic pemphigus
• Leser Trelat
• gastric adenocarcinoma

References

1. 1. Kim JH, Kim SC. Paraneoplastic pemphigus: paraneoplastic autoimmune disease of the skin and mucosa. Front Immunol 2019;10:1-11. https://doi.org/10.3389/fimmu.2019.01259
2. 2. Basir N, Telisinghe PU, Chong VH. Gastric cancer and paraneoplastic pemphigus. Indian J Surg 2015;77:545-546. https://doi.org/10.1007/s12262-015-1311-y
3. 3. Lepekhova A, Olisova O, Teplyuk N, Zolotenkov D, Allenova A. A rare association of paraneoplastic pemphigus with gastric signet cell ring carcinoma. Australas J Dermatol 2019;60:168-169. https://doi.org/10.1111/ajd.12963
4. 4. Bernett CN, Schmieder GJ. Leser Trelat Sign. [Updated 2022 Sep 12]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022.
5. 5. Svoboda SA, Huang S, Liu X, Hsu S, Motaparthi K. Paraneoplastic pemphigus: revised diagnostic criteria based on literature analysis. J Cutan Pathol 2021;48:1133-1138. https://doi.org/10.1111/cup.14004