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Langerhans Cell Histiocytosis with Isolated Cutaneous Involvement Refractory to Polychemotherapy: A Case Report
Francisco Javier Lira-Valero1, Nancy Pulido-Díaz1, Marissa De Jesús Quintal-Ramírez1
1All authors are with the Department of Dermatology at the Instituto Mexicano del Seguro Social and Centro Médico Nacional La Raza in México City, México.
Insights
Langerhans cell histiocytosis (LCH) is a rare adult skin condition. This case highlights an adult LCH presentation resistant to standard chemotherapy, emphasizing the need for alternative treatment strategies.
Area of Science:
- Oncology
- Dermatology
- Hematology
Background:
- Langerhans cell histiocytosis (LCH) is a clonal neoplasm of the monocyte-macrophage lineage.
- LCH predominantly affects children, with skin involvement in 30-60% of cases.
- Isolated cutaneous LCH is rare, occurring in only 4-12% of cases, and typically has a good prognosis.
Observation:
- This report details a rare case of LCH presenting with isolated cutaneous involvement in an adult patient.
- The adult patient's LCH was refractory to standard polychemotherapy regimens.
Findings:
- Adult-onset LCH with isolated skin lesions can be refractory to conventional treatments.
- The case underscores the heterogeneity of LCH presentation and treatment response.
Implications:
- This case suggests that adult patients with isolated cutaneous LCH may require alternative therapeutic approaches.
- Further research into novel treatments for refractory LCH in adults is warranted.
Abstract:
BACKGROUND: Langerhans cell histiocytosis (LCH) is a neoplasm of the monocyte-macrophage lineage, characterized by clonal proliferation and dissemination of cells that express CD1a+ and CD207. It is a disorder that predominates in childhood. Although the skin is the second most frequently affected organ (30-60%), isolated cutaneous involvement is rare; its frequency does not exceed 4 to 12 percent of cases. Single system-LCH usually has a good prognosis. We describe a case of LCH with isolated cutaneous involvement that presented in an adult patient and was refractory to polychemotherapy.
