Langerhans cell histiocytosis of skin: a clinicopathologic analysis of five cases

Indian J Dermatol Venereol Leprol. May-Jun 2006;72(3):211-4. doi: 10.4103/0378-6323.25782.

Abstract

Background and aims: Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of histiocytes characterized by a proliferation of abnormal and clonal Langerhans cells. We retrospectively studied clinicopathologic features of this disorder in five cases.

Methods: Clinical and histopathological findings of five cases of cutaneous LCH were reviewed based on the hospital records.

Results: The age of patients ranged from 28 days to 5 years and M: F ratio was 1:1.5. Clinically, the diagnoses suggested were histiocytosis, varicella, transient neonatal pustular melanosis, keloid, sarcoidosis, seborrheic keratosis and LCH. The most common type of skin lesion was a generalized papular lesion. Histologically, all cases showed aggregates of large mononuclear histiocytes (Langerhans cells) with reniform, irregular, cleaved nuclei and abundant eosinophilic cytoplasm. There was multi-systemic involvement in two patients and single-system involvement in three patients.

Conclusion: Cutaneous lesions may be the sole presenting feature of LCH. Diagnosis is based on demonstration of S-100 positive histiocytes.

MeSH terms

  • Adult
  • Child, Preschool
  • Female
  • Histiocytosis, Langerhans-Cell / diagnosis
  • Histiocytosis, Langerhans-Cell / pathology*
  • Humans
  • Infant
  • Infant, Newborn
  • Langerhans Cells / pathology
  • Male
  • Retrospective Studies
  • Skin / pathology