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CLINICAL IMAGE
Year : 2022  |  Volume : 3  |  Issue : 3  |  Page : 286-287

Langerhans cell histiocytosis in a newborn


Department of Neonatology, Sir Ganga Ram Hospital, New Delhi, India

Date of Submission07-Nov-2022
Date of Acceptance12-Nov-2022
Date of Web Publication28-Dec-2022

Correspondence Address:
Dr. Arun Soni
Department of Neonatology, Sir Ganga Ram Hospital, Rajender Nagar, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JME.JME_139_22

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How to cite this article:
Arora M, Soni A, Saluja S, Modi M. Langerhans cell histiocytosis in a newborn. J Med Evid 2022;3:286-7

How to cite this URL:
Arora M, Soni A, Saluja S, Modi M. Langerhans cell histiocytosis in a newborn. J Med Evid [serial online] 2022 [cited 2023 Feb 1];3:286-7. Available from: http://www.journaljme.org/text.asp?2022/3/3/286/365852



Skin lesions that present at birth can be of varied types each pointing towards a peculiar aetiology. The lesions' colour, aspect, consistency and associated systemic symptoms can steer the clinician towards one diagnosis. Skin lesions at birth can have various possible causes and one should conduct the whole battery of tests to correctly diagnose and intervene at the earliest.

A term, 38 + 1 week, a male baby was born to a primigravida mother with multiple vesiculonodular lesions involving the whole body. Baby had cried immediately after birth and was haemodynamically stable. On examination, lesions were found to be vesiculonodular, multiple in number covering predominantly the face along with the trunk, back and extremities, palms and soles [Figure 1]a. Mucous membrane was characteristically spared. The size of lesions was ranging from 0.5 to 2 cm, reddish to brownish and firm in consistency.
Figure 1: (a) On admission – vesiculonodular lesion, reddish to brown (b) On day 6 of life, lesions extensively covering the whole face and majority of the trunk

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Baby also appeared icteric. The baby was investigated keeping the possibility of sepsis, congenital intrauterine infections and congenital leukaemia. However, the workup was negative and skin biopsy was done. On day 8, the baby developed features of shock with severe anaemia requiring blood transfusions and inotropic support. The dermal lesions increased in number with extensive coverage of the whole body [Figure 1]b. The histopathology report of the biopsy was suggestive of Langerhans cell histiocytosis (LCH), for which the baby was started on hydrocortisone and vinblastine. On day 9 of life, despite maximum support; the baby succumbed to refractory shock.

The skin lesions seen in the baby resembled 'blueberry muffin rash' which suggests the presence of dermal erythropoiesis. Various differential diagnosis of this condition could be congenital infections (toxoplasmosis, rubella, herpes simplex, cytomegalovirus, coxsackie B2 and congenital syphilis), haemolytic disease of the newborn, congenital leukaemia, metastatic neuroblastoma, transient myeloproliferative disease, LCH, metastatic rhabdoid tumour, etc.

LCH is a neoplastic disorder that primarily affects skin and bones but has a vulnerability to affect any and every organ. LCH is considerably more common in children than adults with male predominance seen. The exact incidence of the disease is poorly defined as it is underreported due to its rarity. Estimates are as high as five cases per million children. LCH is so called due to the resemblance of the neoplastic cells to the dendritic Langerhans cells of the skin and mucosa. The BRAF V600E mutation is present in more than half of cases, and activation of the mitogen-activated protein kinase pathway is a key driver of this neoplastic disorder. Clinical manifestation can be confined to single site or multiple sites in one organ (e.g. in bone or skin) or it can present in multiple organ systems simultaneously or sequentially. Skin involvement can vary from macular lesions which are localised to papular nodular lesions diffusely involving the skin. Up to 40% of cases of skin LCH have been reported to progress to multisystem involvement. Treatment involves spontaneous regression with close follow-up in isolated dermal lesions to topical steroids (triamcinolone) to vinblastine–prednisone induction therapy in multisystemic involvement.

A skin biopsy is always necessary to confirm the diagnosis of cutaneous LCH. It is characterised by infiltration of the papillary dermis by monomorphous, medium-sized histiocytes with pink cytoplasm and kidney-shaped grooved nucleus. The nodular form especially is seen to have large histiocytes and ulcerated overlying epidermis. On immunohistochemistry, cells are positive for CD1a and CD207. The image (in our case - [Figure 3]a) showed extensive infiltration of the dermis with round-to-oval cells with vesicular nuclei. Many cells have folded/lobulated/grooved nuclei with inconspicuous nucleoli [Figure 2]a. Some of the cells aggregate in the subcutaneous fat show central necrosis [Figure 3]b. Immunohistochemistry shows positivity for S100 and CD1a with variable focal positivity with CD68 [Figure 2]b.
Figure 2: (a) Cells with Grooved Nuclei (black arrow), (b) Immunohistochemistry with CD 1a positivity

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Figure 3: (a) Infiltration of the dermis with round-to-oval cells (black arrow) with epidermal hyperkeratosis (blue arrow) cells with folded/grooved nuclei, (b) Central necrosis (black arrow)

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Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.




    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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