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Purpose <p>Approximately 10% of all lymphomatoid papulosis (LyP) cases affect pediatric patients. Pediatric LyP is clinically similar to adult LyP, frequently with cutaneous involvement, whereas the affectation of mucosal surface is rare. The LyP clinicopathological spectrum includes type A, B, C, D, E, and LyP with DUSP22/IRF4 rearrangement, with LyP type D representing &lt; 5% of all LyP cases.</p> Methods <p>We have previously reported 2 cases of exclusively intraoral LyP type C and E. To date, about 50 cases of LyP type D have been reported; of them, 8 cases, all but one with exclusive skin involvement, corresponded to pediatric patients. Rare LyP type C cases can lack CD30 expression, creating diagnostic difficulties with lymphoma.</p> Results <p>Here, we report an extremely rare case of intraoral LyP type D with scarce/absent CD30 expression affecting a 12-year-old white Brazilian boy.</p> Conclusions <p>To our best knowledge, this is the first pediatric case of LyP type D with exclusive intraoral involvement.</p>

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Intraoral Lymphomatoid Papulosis Type D Showing Scarce/Absent CD30 Expression in A Pediatric Patient: Case Report and Literature Review

  • 期刊论文

  • 发表日期：

    2025年2月5日

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  • Mariana Paravani Palaçon,
  • Marcelo Borges Marques,
  • Débora Fernandes Mendes Silveira,
  • Karina Helen Martins,
  • Heitor Albergoni Silveira,
  • Valfrido Antonio Pereira-Filho,
  • Andreia Bufalino,
  • Fernando Chahud,
  • Jorge Esquiche León

摘要

Purpose

Approximately 10% of all lymphomatoid papulosis (LyP) cases affect pediatric patients. Pediatric LyP is clinically similar to adult LyP, frequently with cutaneous involvement, whereas the affectation of mucosal surface is rare. The LyP clinicopathological spectrum includes type A, B, C, D, E, and LyP with DUSP22/IRF4 rearrangement, with LyP type D representing < 5% of all LyP cases.

Methods

We have previously reported 2 cases of exclusively intraoral LyP type C and E. To date, about 50 cases of LyP type D have been reported; of them, 8 cases, all but one with exclusive skin involvement, corresponded to pediatric patients. Rare LyP type C cases can lack CD30 expression, creating diagnostic difficulties with lymphoma.

Results

Here, we report an extremely rare case of intraoral LyP type D with scarce/absent CD30 expression affecting a 12-year-old white Brazilian boy.

Conclusions

To our best knowledge, this is the first pediatric case of LyP type D with exclusive intraoral involvement.