Rare diagnosis in a neonate with isolated skin lesions

Authors

  • Supriya Gujjar Suresh Department of Paediatrics Hematology and Oncology, Kanchi Kamakoti CHILDS Trust Hospital, The CHILDS Trust Medical Research Foundation, Chennai, Tamil Nadu, India
  • Saraswathy Jagadeesan Department of Paediatrics, Kanchi Kamakoti CHILDS Trust Hospital, The CHILDS Trust Medical Research Foundation, Chennai, Tamil Nadu, India
  • Arathi Srinivasan Department of Paediatrics Hematology and Oncology, Kanchi Kamakoti CHILDS Trust Hospital, The CHILDS Trust Medical Research Foundation, Chennai, Tamil Nadu, India
  • Julius Xavier Scott Department of Paediatrics Hematology and Oncology, Kanchi Kamakoti CHILDS Trust Hospital, The CHILDS Trust Medical Research Foundation, Chennai, Tamil Nadu, India

DOI:

https://doi.org/10.18203/2349-3291.ijcp20172702

Keywords:

Langerhan cell histiocytosis, Neonates

Abstract

Langerhan Cell Histiocytosis (LCH) represents a group of diseases with varied spectrum of presentation. LCH limited to skin is not very common with 100 cases reported in literature. A definitive diagnosis is important for both management and follow up. A 30 days old male infant was brought with vesiculopustular skin lesions over the trunk and back since day 12 of life. Systemic examination was normal. Preliminary investigations revealed a normal haemogram with normal liver and renal function tests. He was initially managed symptomatically. In view of persisting skin leisons, biopsy done was suggestive of Langerhans Cell Histiocytosis and confirmed by CD1a postivity on immunohistochemistry. Skeletal survey did not reveal any lytic leison, bone scan was normal. There was no biochemical evidence of diabetes insipidus. The child was diagnosed to have neonatal isolated cutaneous LCH. He was followed up periodically without any therapy. Presently, he has an Event Free Survival (EFS) of 2 years without any progression. Though isolated cutaneous Langerhan Cell Histiocytosis is rare in neonates, it should still be considered in the differential diagnosis by the treating paediatrician. Though it can be a self-limiting entity, it is very important to recognize this condition, since it is mandatory to look for multisystem involvement by LCH as it determines the treatment intensity. It is essential to closely follow up these children to know if this heralds involvement of risk organs by LCH or a self-limiting disorder. This case also highlights the multidisciplinary dialogue required in the diagnosis and management of such rare cases.

References

Mohr MR, Sholtzow M, Bevan HE, Fisher RG. Exploring the differential diagnosis of hemorrhagic vesicopustules in a newborn. Pediatrics; 2011.

Araújo T, Schachner L. Erupções Vesicopustulosas Benignas No Neonato. An Bras Dermatol; 2006.

Stålemark H, Laurencikas E, Karis J, Gavhed D, Fadeel B, Henter J-I. Incidence of Langerhans cell histiocytosis in children: a population-based study. Pediatr Blood Cancer. 2008;51(1):76-81.

Stein SL, Paller AS, Haut PR, Mancini AJ. Langerhans cell histiocytosis presenting in the neonatal period: a retrospective case series. Arch Pediatr Adolesc Med. 2001;155(7):778-83.

Berry DH, Gresik MV, Humphrey GB. Natural history of histiocytosis X: a Pediatric Oncology Group Study. Med Pediatr Oncol. 1986;14(1):1-5.

Hashimoto K, Bale GF, Hawkins HK, Langston C, Pritzker MS. Congenital Self‐Healing Reticulohistiocytosis (Hashimoto‐Pritzker Type). Internat J Dermatol. 1986;25(8):516-23.

Isaacs H. Fetal and neonatal histiocytoses. Pediatr Blood Cancer. 2006;47(2):123-9.

Hashimoto K, Pritzker MS. Electron microscopic study of reticulohistiocytoma. An unusual case of congenital, self-healing reticulohistiocytosis. Arch Dermatol. 1973;107(2):263-70.

Kapur P, Erickson C, Rakheja D, Carder KR, Hoang MP. Congenital self-healing reticulohistiocytosis (Hashimoto-Pritzker disease): ten-year experience at Dallas Children's Medical Center. J Am Acad Dermatol. 2007;56(2):290-4.

Minkov M, Prosch H, Steiner M. Langerhans cell histiocytosis in neonates. Pediatr Blood Cancer. 2005;45(6):802-7.

Sankilampi, Ulla. "Congenital Langerhans Cell Histiocytosis Mimicking A “Blueberry Muffin Baby”"J PediatR Hematol/Oncol. 2008;30(3):245-8.

Lau L, Krafchik B, Trebo MM, Weitzman S. Cutaneous Langerhans cell histiocytosis in children under one year. Pediatr Blood Cancer. 2006;46(1):66-71

Battistella M, Fraitag S, Teillac DH, Brousse N, de Prost Y, Bodemer C. Neonatal and early infantile cutaneous langerhans cell histiocytosis: comparison of self-regressive and non-self-regressive forms. Arch Dermatol. 2010;146(2):149-56.

Downloads

Published

2017-06-21

Issue

Section

Case Reports