A possible case of Langerhans-cell histiocytosis? Differential diagnosis in a rare case from the Late Antiquity Bavaria (Germany)

Int J Paleopathol. 2021 Jun:33:270-279. doi: 10.1016/j.ijpp.2021.05.005. Epub 2021 May 26.

Abstract

Objective: To outline the importance of accurate diagnosis in ancient rare diseases by presenting a possible case of Langerhans-cell histiocytosis.

Materials: Skeletal elements from a well-preserved skeleton of a nine to eleven-year-old, probably female child who lived around 300-400 AD Late Roman Neuburg / Donau (Germany).

Methods: Macroscopic, radiologic, light and scanning-electron microscopic and physical techniques were used.

Results: Resorptive defects, particularly in the cranium, but also in the left hip bone and the right femur, suggest the presence of Langerhans-cell histiocytosis macroscopically and radiologically. The presence of morphological changes along the edges of osteolytic lesions and in the diploic spaces appear to be post-mortem artifacts based on microscopic investigation and elemental analysis.

Conclusions: Re-evaluation of morphological structures and elemental constitution of lesions is critical to differential diagnosis. In the case examined here, the identification of post-mortem structures rules out the former diagnosis of Langerhans-cell histiocytosis. Re-evaluation of cases of rare diseases require applying a range of methods during the analysis, as every single case makes a difference in the numbers of this very small group of diseases.

Significance: This study emphasizes the importance of utilizing different analytical techniques to avoid false diagnoses.

Limitations: Not all morphological features can reliably be diagnosed using microscopic and elemental techniques.

Suggestions for further research: In the case of rare diseases that are difficult to diagnose, the widest possible spectrum of techniques should always be used, particularly microscopy.

Keywords: Ancient rare diseases (ARD); Histiocytosis-X; Microscopy; Pseudopathology; Taphonomy.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Diagnosis, Differential
  • Female
  • Femur
  • Germany
  • Histiocytosis, Langerhans-Cell* / diagnosis
  • Humans
  • Skull / diagnostic imaging