BACKGROUND: The cutaneous presentation of Langerhans cell histiocytosis (LCH) is very rare and can be highly variable among individuals, which can often lead to a delay in diagnosis. OBJECTIVE: To discuss a case report and literature review of important clinical indicators, histology, diagnosis, evaluation, and treatment guidelines. METHODS: Case report and literature review. RESULTS: Skin biopsies positive for CD1a and/or langerin are diagnostic for LCH. A thorough review of systems, baseline laboratory tests, and imaging studies can determine the extent of LCH. Treatment of cutaneous disease is largely based on case report and small case studies, but baseline treatment should generally begin with oral or topical steroids. When patients have more severe disease that requires a systemic approach, the efficacy of therapy should be assessed 6 weeks into therapy, with subsequent treatment intensification in patients with limited response. CONCLUSION: Owing to the rarity of this condition, there are no specific guidelines for treatment of LCH, but guidelines put forth by the Histiocyte Society assist in categorization and basic treatment approaches for patients with systemic disease.
BACKGROUND: The cutaneous presentation of Langerhans cell histiocytosis (LCH) is very rare and can be highly variable among individuals, which can often lead to a delay in diagnosis. OBJECTIVE: To discuss a case report and literature review of important clinical indicators, histology, diagnosis, evaluation, and treatment guidelines. METHODS: Case report and literature review. RESULTS: Skin biopsies positive for CD1a and/or langerin are diagnostic for LCH. A thorough review of systems, baseline laboratory tests, and imaging studies can determine the extent of LCH. Treatment of cutaneous disease is largely based on case report and small case studies, but baseline treatment should generally begin with oral or topical steroids. When patients have more severe disease that requires a systemic approach, the efficacy of therapy should be assessed 6 weeks into therapy, with subsequent treatment intensification in patients with limited response. CONCLUSION: Owing to the rarity of this condition, there are no specific guidelines for treatment of LCH, but guidelines put forth by the Histiocyte Society assist in categorization and basic treatment approaches for patients with systemic disease.
Authors: Stephen J Simko; Benjamin Garmezy; Harshal Abhyankar; Philip J Lupo; Rikhia Chakraborty; Karen Phaik Har Lim; Albert Shih; M John Hicks; Teresa S Wright; Moise L Levy; Kenneth L McClain; Carl E Allen Journal: J Pediatr Date: 2014-10-21 Impact factor: 4.406