Infantile Cutaneous Langerhans Cell Histiocytosis Presenting as Benign Skin Lesions: Clinical Pitfalls and Diagnostic Clues
Keywords:
Langerhans cell histiocytosis, multisystem, congenital skin lesionsAbstract
Introduction: Langerhans cell histiocytosis (LCH) is a rare but potentially life-threatening hematologic disorder characterized by a wide spectrum of skin manifestations that may mimic a variety of common neonatal and pediatric diseases.
Objectives: To raise awareness of the broad clinical spectrum of cutaneous manifestations important for early recognition.
Methods: Herein, we present seven cases of LCH with diverse skin manifestations, disease type, and course of disease.
Results: Dermatological examination was key in suspecting LCH in all cases, enabling prompt oncological evaluation with skin signs preceding other organ involvement. Skin manifestations ranged from isolated to widespread skin rashes. Common lesions included erythematous, reddish-brown, or skin-colored crusted or scaly papules or maculopapular lesions. However, plaques, patches, vesicles, and urticarial lesions may also occur.
Conclusions: Timely dermatological consultation should be sought in cases of non-resolving, therapy-resistant skin lesions and an unclear diagnosis.
References
Krooks J, Minkov M, Weatherall AG, Langerhans cell histiocytosis in children: History, classification, pathobiology, clinical manifestations, and prognosis. J Am Acad Dermatol, 2018. 78(6): p. 1035-1044. DOI: 10.1016/j.jaad.2017.05.059. PMID: 29754885.
Rodriguez-Galindo C, Clinical features and treatment of Langerhans cell histiocytosis. Acta Paediatr, 2021. 110(11): p. 2892-2902. DOI: 10.1111/apa.16014. PMID: 34192374.
Jezierska M, Stefanowicz J, Romanowicz G, Kosiak W, Lange M, et al., Langerhans cell histiocytosis in children - a disease with many faces. Recent advances in pathogenesis, diagnostic examinations and treatment. Postepy Dermatol Alergol, 2018. 35(1): p. 6-17. DOI: 10.5114/pdia.2017.67095. PMID: 29599667.
Leung, AKC, Lam JM, Leong KF, Childhood Langerhans cell histiocytosis: a disease with many faces. World J Pediatr, 2019. 15(6): p. 536-545. DOI: 10.1007/s12519-019-00304-9. PMID: 31456157.
Minkov M, Prosch H, Steiner M, et al., Langerhans cell histiocytosis in neonates. Pediatr Blood Cancer, 2005. 45(6): p. 802-7. DOI: 10.1002/pbc.20362. PMID: 15770639.
Li Z, Yanqiu L, Yan W, et al., Two case report studies of Langerhans cell histiocytosis with an analysis of 918 patients of Langerhans cell histiocytosis in literatures published in China. Int J Dermatol, 2010. 49(10): p. 1169-74. DOI: 10.1111/j.1365-4632.2009.04360.x. PMID: 20883405.
Krooks J, Minkov M, Weatherall AG, Langerhans cell histiocytosis in children: Diagnosis, differential diagnosis, treatment, sequelae, and standardized follow-up. J Am Acad Dermatol, 2018. 78(6): p. 1047-1056. DOI: 10.1016/j.jaad.2017.05.060. PMID: 29754886.
Hussein, MRA, Skin-limited Langerhans' cell histiocytosis in children. Cancer Invest, 2009. 27(5): p. 504-11. DOI: 10.1080/07357900802216452. PMID: 19308811.
Poompuen S, Chaiyarit J, Techasatian L, Diverse cutaneous manifestation of Langerhans cell histiocytosis: a 10-year retrospective cohort study. Eur J Pediatr, 2019. 178(5): p. 771-776. DOI: 10.1007/s00431-019-03356-1. PMID: 30826864.
Ehrhardt MJ, Humphrey SR, Kelly ME, Chiu YE, Galbraithet SS, The natural history of skin-limited Langerhans cell histiocytosis: a single-institution experience. J Pediatr Hematol Oncol, 2014. 36(8): p. 613-6. DOI: 10.1097/MPH.0000000000000248. PMID: 25171449.
Morren MA, Broecke KV, Vangeebergen L, et al., Diverse Cutaneous Presentations of Langerhans Cell Histiocytosis in Children: A Retrospective Cohort Study. Pediatr Blood Cancer, 2016. 63(3): p. 486-92.. DOI: 10.1002/pbc.25834. PMID: 26586230.
Salotti JA, Nanduri V, Pearce MS, Parker L, Lynn R, Windebank KP, Incidence and clinical features of Langerhans cell histiocytosis in the UK and Ireland. Arch Dis Child, 2009. 94(5): p. 376-80. DOI: 10.1136/adc.2008.144527. PMID: 19060008.
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