Unilesional self‐limited Langerhans cell histiocytosis: a case report and review of the literature

Abstract

Self-limited Langerhans cell histiocytosis (LCH) represents a rare, congenital, cutaneous form of LCH associated with a good prognosis. Only 35 cases of solitary lesion self-limited LCH have been reported. Herein, we present an additional case in a 3-month-old boy who presented with an isolated pigmented nodule on his left posterior shoulder, which had been present since birth. Punch biopsy showed histopathologic features of LCH with positive CD1a and CD68 stains. Further examination and investigation showed no features of systemic involvement. The lesion spontaneously resolved within 2 months, and there has been no evidence of recurrence on follow up. As several cases of recurrence and complications have been reported in self-limited LCH, we emphasize the need for long-term, and perhaps indefinite, follow up for symptoms and signs associated with LCH in these patients.

Langerhans cell histiocytosis: Differences and similarities in long-term outcome of paediatric and adult patients at a single institutional centre

“…32 With respect to skeletal bones, the lesions affecting the bones of the foot in one adult and one child in our series were rarely reported previously. 33 Many published case reports show a diversity of self-limited or disseminated 18,34,35 cutaneous manifestations mimicking various diseases, such as varicella, scabies, nodular prurigo, molluscum contagiosum, or leprosy. [36][37][38][39][40] In our study, seborrhoeic dermatitis of the scalp was the most common type of cutaneous manifestation, and mainly affected children.…”

Cutaneous Hematopathology

“…CD1a staining is specifi c for LCH (Newman et al 2007 ). CD68 staining can be positive (Wheller et al 2013 ). Langerin (CD207) is also specifi c for LCH (Chikwava and Jaffe 2004 ;Lau et al 2008 ).…”

“…Thorough investigation and follow-up of patients with LCH should be performed because clinically, histopathologically, and immunohistochemically, one cannot determine those individuals with more aggressive disease (Kapur et al 2007 ;Wheller et al 2013 ). In self-limited disease, one group suggests a follow-up period of 2 years including laboratory tests and radiographs (abdominal ultrasound and chest x-ray (Zunino-Goutorbe et al 2008 ).…”

Diverse Cutaneous Presentations of Langerhans Cell Histiocytosis in Children: A Retrospective Cohort Study

“…This type of LCH starts at birth or in the neonatal period as a single system cutaneous presentation. Multiple lesions are most common, but the presence of a solitary skin lesion has also been reported . By definition, there is absence of systemic manifestations and the cutaneous involvement spontaneously involutes within weeks to a few months.…”