Archives

  • 2018-07
  • 2018-10
  • 2018-11
  • 2019-04
  • 2019-05
  • 2019-06
  • 2019-07
  • 2019-08
  • 2019-09
  • 2019-10
  • 2019-11
  • 2019-12
  • 2020-01
  • 2020-02
  • 2020-03
  • 2020-04
  • 2020-05
  • 2020-06
  • 2020-07
  • 2020-08
  • 2020-09
  • 2020-10
  • 2020-11
  • 2020-12
  • 2021-01
  • 2021-02
  • 2021-03
  • 2021-04
  • 2021-05
  • 2021-06
  • 2021-07
  • 2021-08
  • 2021-09
  • 2021-10
  • 2021-11
  • 2021-12
  • 2022-01
  • 2022-02
  • 2022-03
  • 2022-04
  • 2022-05
  • 2022-06
  • 2022-07
  • 2022-08
  • 2022-09
  • 2022-10
  • 2022-11
  • 2022-12
  • 2023-01
  • 2023-02
  • 2023-03
  • 2023-04
  • 2023-05
  • 2023-06
  • 2023-08
  • 2023-09
  • 2023-10
  • 2023-11
  • 2023-12
  • 2024-01
  • 2024-02
  • 2024-03
  • 2024-04

    • br Discussion TEPD is a very rare subgroup

    2018-11-12


    Discussion TEPD is a very rare subgroup among EMPDs, and only a few case reports or case series have been published. This is the first reported Taiwanese case of TEPD. Most cases were reported from Japan. There was a male predominance in the patient groups. Including our case, all but one of the 29 cases were male. In most TEPD cases, for unknown reasons, genital lesions preceded the axillary lesions except in one Japanese case and our own case. The presentation of TEPD, especially axillary lesion, was variable. Some reports in Japan showed subclinical diseases in axillae, which were confirmed by biopsies. Accordingly, some authors recommended to carefully check for almost invisible axillary lesions, even considering random biopsy, in patients with genital Paget\'s disease. To our knowledge, our patient is the first case of TEPD to receive long-term cryotherapy even before the final diagnosis was made. The pigmentary change and tissue scarring caused by cryotherapy distracted our attention initially, and fortunately we still noticed the asymptomatic small positive people lesion over the right armpit during routine physical examination. The incidence of secondary EMPD in Asian populations is reported to be lower than that in Caucasian populations. Including two Caucasian cases, all cases of TEPD reported were primary diseases. The incidence of invasive EMPD was reported as 18.8% in a retrospective study in China, including 48 patients (all were solitary EMPDs). To our knowledge, including this case, there were 12% TEPD patients (4/33 patients) with dermal invasion. There was a lack of solid evidence to clarify whether the severity of surface involvement can predict the risk of dermal invasion. However, dermal invasion had been confirmed as a predictor of distant metastasis, although it should be noted that the metastatic cases were not associated with larger skin lesions. Adachi et al reported one invasive TEPD case that presented an erythematous plaque over the left axilla for 20 years without treatment, and this lesion proceeded lately with an erosive tumor on the left axillary lesion, and erythematous plaques over the right axilla and genital area. Those three sites of lesions were pathologically confirmed as EMPD, and the erosive tumor corresponded to the invasive part. Similarly, our case also had long-lasting lesions over the left axilla and genital area, which were pathologically confirmed as invasive EMPD, even though no tumor lesion was noticed. In TEPD, further study is necessary to clarify the relationship between the chronicity of disease, the measurement of epidermal involvement, and the development of invasive disease. Whether immunohistochemistry can be a reliable tool to differentiate primary from secondary EMPD is still debatable, and many studies have led to inconsistent results. Paget\'s cells of both origins are usually immunosensitive to CK7, CEA, and epithelial membrane antigen, but the expression of CK20 and GCDFP-15 is variable. A review article noticed that CK20 was found more frequently in cases of secondary EMPD, whereas GCDFP-15 was more associated with the primary disease. However, only 58% of primary EMPD cases had the immunophenotype CK20(–)/GCDFP-15(+) reported in a Taiwanese study. The study of immunophenotype in TEPD has been scanty. Among the 32 reported cases of primary type, only two presented immunophenotype: one was positive to CK7, CEA, and estrogen and progesterone receptors, but negative to CK20 and GCDFP-15; the other was immunosensitive to CK7 but not CD20. As a primary TEPD, our case was observed to be immunosensitive to CK7, GCDFP-15, CEA, and estrogen receptor, which was consistent with previous studies. TEPD, regarded as a multicentric EMPD, seems to display similar immunophenotype to solitary EMPD. The first specimen from the left axilla was observed to be immunoreactive to GATA3, which is well known as a useful stain to label breast cancer cells, especially in axillae. However, a recent study in China presented GATA3 expression in all cases of vulvar Paget\'s disease. Therefore, further study is necessary to confirm the role of GATA3 expression in vulvar/nonvulvar EMPD. It was reasonable to survey for underlying adenocarcinoma, especially breast cancer, in the clinical setting where GATA3-positive cells in axillae were encountered.