Article

Cutaneous Langerhans cell histiocytosis in children under one year.

Division of Haematology/Oncology, Department of Paediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada.
Pediatric Blood & Cancer (Impact Factor: 2.35). 02/2006; 46(1):66-71. DOI: 10.1002/pbc.20479
Source: PubMed

ABSTRACT 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.
A retrospective review was conducted on 22 LCH patients who were younger than 12 months at the onset of their skin eruption.
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%.
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.

0 Bookmarks
 · 
57 Views
  • [Show abstract] [Hide abstract]
    ABSTRACT: To describe an Asian population with isolated cutaneous LCH, presenting a review on previous studies on this subgroup of patients and comparing our cohort with other studies of cutaneous LCH with systemic involvement. All patients diagnosed as LCH with cutaneous involvement presenting to a tertiary pediatric hospital (KK Women's & Children's Hospital) between January 2001 and December 2011 were reviewed. Information recorded included clinical presentation, investigation results, treatment and outcome. We identified 10 patients with cutaneous manifestations of LCH. 6 had isolated cutaneous LCH with no visceral or bony involvement, while 4 had cutaneous lesions with involvement of other organ systems. Of the patients with isolated skin involvement, 3 were treated with surgical excision, 1 with topical corticosteroids and 2 with multi-agent chemotherapy. The 4 patients with concomitant systemic involvement were treated with multi-agent chemotherapy. None of our patients with isolated cutaneous LCH progressed to multi-system disease during the follow-up period. Cutaneous LCH, with or without other organ involvement at diagnosis has a good overall prognosis. However, long term follow-up is recommended in view of possible recurrence. This article is protected by copyright. All rights reserved.
    Acta Paediatrica 08/2013; · 1.97 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Langerhans cell histiocytosis (LCH) has been previously thought of as a rare illness, but is now increasingly diagnosed as a result of the more intensive investigations of patients with cystic pulmonary disease. In recent years, treatments developed from our new understanding of the molecular biology of malignant disease have been applied to patients with LCH, and responses seen. In this review, we describe the origins, presentation and modern treatment of LCH, showing that there is new hope for patients with this condition.
    QJM: monthly journal of the Association of Physicians 11/2010; 104(2):89-96. · 2.36 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: To describe a rare case of congenital self-healing Langerhans cell histiocytosis (CSHLCH) presenting with atypical eye involvement. Case report. A female newborn presented with purpuric lesions over the trunk, limbs, and face. Liver ultrasonography revealed hypoechogenic lesions with blurred borders. Biomicroscopy showed right posterior synechiae with fibrinoid deposits on the lens. At 7 months she presented with right acute glaucoma. Biomicroscopy showed the presence of inflammatory pseudo-membrane covering the anterior surface of the lens, iris, and iridocorneal angle. Ab externo trabeculotomy was performed; access to the anterior chamber with capsulorrhexis forceps permitted a peeling of the pseudo-membrane with normalization of the intraocular pressure. Histologic examination of the membrane revealed an inflammatory tissue with CD1a and S-100 positive histiocytic cells. This is the first case of CSHLCH describing acute glaucoma secondary to a pseudo-inflammatory membrane with typical histiocytic cells, occluding the iridocorneal angle.
    Ocular immunology and inflammation 08/2011; 19(5):337-9. · 0.72 Impact Factor