Langerhan cell histiocytosis with multisystem involvement in an adult

ABSTRACT

Langerhan cell histiocytosis [LCH] is a rare disorder that primarily affects children. Its occurrence in adults is very rare. We report a case of 37- year - old male patient who presented with complaints of increased thirst, excessive passage of urine, shortness of breath and skin lesions. The diagnostic workup revealed endocrine involvement with diabetes insipidus, restrictive lung disease, skin biopsy consistent with LCH, bone and periodontal involvement. The skin lesions responded well to Psoralen- Ultraviolet Radiation A therapy[PUVA]. He showed general improvement on systemic chemotherapy