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Indeterminate cell histiocytosis successfully treated with phototherapy
Maria Claudia Nogueira Zerbini1, Mirian Nacagami Sotto1, Fernando Peixoto Ferraz de Campos2
1Department of Pathology - Faculty of Medicine - University of São Paulo, São Paulo/SP - Brazil .
Insights
Intermediate cell histiocytosis is a rare skin condition involving indeterminate cell proliferation. Narrowband ultraviolet B phototherapy showed excellent short-term results for a patient with this rare disorder.
Area of Science:
- Dermatology
- Pathology
- Immunohistochemistry
Background:
- Intermediate cell histiocytosis (ICH) is a rare disorder affecting cutaneous dendritic cells.
- It is characterized by the proliferation of indeterminate cells (ICs).
Observation:
- A case of a healthy elderly woman with generalized, dome-shaped, reddish cutaneous nodules over 18 months is presented.
- The patient exhibited no internal organ involvement.
- Skin biopsy confirmed intermediate cell histiocytosis.
Findings:
- Immunophenotypically, indeterminate cells (ICs) are positive for CD1a, CD68, and faintly/focally for S100.
- CD207 (langerin) is negative in these cells.
- The patient's condition was diagnosed as intermediate cell histiocytosis.
Implications:
- This case highlights the varied clinical presentation of intermediate cell histiocytosis.
- Narrowband ultraviolet B phototherapy demonstrated an excellent short-term outcome for this rare cutaneous disorder.
- Further research into long-term efficacy and alternative treatments for ICH is warranted.
Abstract:
First described in 1985, intermediate cell histiocytosis is a rare disorder of the cutaneous dendritic cell group with a varied clinical presentation and evolution. The pathologic substrate is constituted by the proliferation of indeterminate cells (ICs) that are immunophenotypically characterized by the positivity of CD1a, CD68, and faint/focal S100, plus the negativity for CD207 (langerin). The authors present the case of a healthy elderly woman who presented generalized dome-shaped reddish cutaneous nodules over her trunk, neck, face, and extremities over a period of 18 months. A laboratory and imaging work-up ruled out internal involvement. The skin biopsy was consistent with IC histiocytosis. The patient was treated with narrowband ultraviolet B phototherapy, which resulted in an excellent short-term outcome.
