Langerhans cell histiocytosis manifesting as recurrent simultaneous bilateral spontaneous pneumothorax in early infancy

Abstract

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by infiltration of either single or multiple organs by a distinct cell type that is S-100 and CD1a positive and contains ultrastructural Birbeck granules on electron microscopy. Historically, LCH included four main clinical forms: Letter-Siwe disease, Hand-Schuller-Christian disease, eosinophilic granuloma (together grouped as histiocytosis) and Hashimoto-Pritzker disease. The writing group of the Histiocytotic Society in 1987 proposed the uniform term of 'Langerhans cell histiocytosis' to encompass all the aforementioned eponymous forms. Lung involvement occurs in up to half of all children with multisystem disease and usually parallels overall disease activity. Spontaneous pneumothorax (SP) occurs in approximately 10% of children with pulmonary disease and may be a fatal complication. Patients with pulmonary LCH are likely predisposed to the development of pneumothorax based on destructive changes in the lung parenchyma. Here, we report a case of multisystem LCH in which the patient presented at 2 months of age because of simultaneous bilateral pneumothorax.

Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study

“…Exceptionally, within children, even at a young age, LCH may present as severe lung damage, as typically observed in young adults. Management of these exceptional cases of young children with very severe lung disease is challenging; only few case reports have been published [7,[18][19][20][21][22][23][24][25][26][27]. Certain described children required management in an intensive care unit (ICU) for respiratory decompensation.…”

Concurrence of Two Rare Conditions: Simultaneous Bilateral Spontaneous Pneumothorax and Congenital Pulmonary Airway Malformation

“…3 In a small number of pediatric studies, SBSP was found to be associated with a few underlying lung diseases such as Langerhans cell histiocytosis (LCH). 4 The present study first reported the concurrence of these rare conditions, as a patient presenting a SBSP was diagnosed with of type 2 CPAM based on the typical radiological findings.…”

Langerhans cell histiocytosis (LCH) presenting as pneumothorax and neck lymphadenopathy

“…In another case, report published from Iran LCH was reported in a two-month-old infant with simultaneous bilateral and spontaneous pneumothorax. Due to destructive changes in in lungs, pneumothorax could happen in 10% of LCH cases with respiratory involvement [17]. In 2005, Nakhla et al reported a previously healthy 16 years old girl with sudden death.…”