BACKGROUND: To evaluate the clinical course and outcome of infants with Langerhans cell histiocytosis (LCH) involving skin and to estimate the incidence of progression to multi-system (M-S) disease in those with isolated skin involvement. METHODS: A retrospective review was conducted on 22 LCH patients who were younger than 12 months at the onset of their skin eruption. RESULTS: Twelve patients had isolated skin involvement at diagnosis and 10 were evaluable for progression. Four of the 10 (40%) evaluable patients progressed to multi-system (M-S) disease. Of the 10 patients with M-S disease at diagnosis, 5 had a history of a preceding skin eruption 2 to 13 months prior to diagnosis. Eleven of the 14 (79%) patients with M-S disease had risk organ involvement. The mortality rate of M-S disease was 50%. CONCLUSIONS: It is important for primary caregivers to recognize that isolated cutaneous LCH in infants is not always a benign disorder. The diagnosis of self-healing cutaneous LCH should only be made in retrospect. Careful, albeit non-invasive, follow-up is recommended to monitor for disease progression and development of long-term complications.
BACKGROUND: To evaluate the clinical course and outcome of infants with Langerhans cell histiocytosis (LCH) involving skin and to estimate the incidence of progression to multi-system (M-S) disease in those with isolated skin involvement. METHODS: A retrospective review was conducted on 22 LCH patients who were younger than 12 months at the onset of their skin eruption. RESULTS: Twelve patients had isolated skin involvement at diagnosis and 10 were evaluable for progression. Four of the 10 (40%) evaluable patients progressed to multi-system (M-S) disease. Of the 10 patients with M-S disease at diagnosis, 5 had a history of a preceding skin eruption 2 to 13 months prior to diagnosis. Eleven of the 14 (79%) patients with M-S disease had risk organ involvement. The mortality rate of M-S disease was 50%. CONCLUSIONS: It is important for primary caregivers to recognize that isolated cutaneous LCH in infants is not always a benign disorder. The diagnosis of self-healing cutaneous LCH should only be made in retrospect. Careful, albeit non-invasive, follow-up is recommended to monitor for disease progression and development of long-term complications.
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
Authors: Stephane Ducassou; Fanny Seyrig; Caroline Thomas; Anne Lambilliotte; Perrine Marec-Berard; Claire Berger; Genevieve Plat; Laurence Brugiere; Marie Ouache; Mohamed Barkaoui; Corinne Armari-Alla; Patrick Lutz; Guy Leverger; Xavier Rialland; Ludovic Mansuy; Helene Pacquement; Eric Jeziorski; Virginie Gandemer; François Chalard; Jean François Chateil; Abdellatif Tazi; Jean François Emile; Jean Donadieu Journal: Pediatr Blood Cancer Date: 2013-06-29 Impact factor: 3.167