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CLINICAL IMAGE
Year : 2020  |  Volume : 152  |  Issue : 7  |  Page : 159-160

Multisystem Langerhans cell histiocytosis: A diagnostic chameleon


Department of Pulmonary Medicine, Employee State Insurance - Postgraduate Institute of Medical Sciences & Research (ESI-PGIMSR), New Delhi 110 015, India

Date of Submission20-Nov-2019
Date of Web Publication25-May-2021

Correspondence Address:
Dipti Gothi
Department of Pulmonary Medicine, Employee State Insurance - Postgraduate Institute of Medical Sciences & Research (ESI-PGIMSR), New Delhi 110 015
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijmr.IJMR_2248_19

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How to cite this article:
Vaidya S, Gothi D. Multisystem Langerhans cell histiocytosis: A diagnostic chameleon. Indian J Med Res 2020;152, Suppl S1:159-60

How to cite this URL:
Vaidya S, Gothi D. Multisystem Langerhans cell histiocytosis: A diagnostic chameleon. Indian J Med Res [serial online] 2020 [cited 2021 Aug 4];152, Suppl S1:159-60. Available from: https://www.ijmr.org.in/text.asp?2020/152/7/159/316794

Patient's consent obtained to publish clinical information and images.


A 32 yr old non-smoker female reported to the department of Dermatology, ESI-PGIMSR (Employee State Insurance-Postgraduate Institute of Medical Sciences & Research), New Delhi, India, in November 2018, for remitting and relapsing rashes all over the body for the last two years [Figure 1]A and [Figure 1]B with low-grade fever, malaise and weight loss. Routine haematological investigations were normal. The skin biopsy of the thigh region showed epithelioid cell granuloma with necrosis. The patient was started on anti-tubercular therapy. She was referred to the Pulmonary Medicine department for cough and abnormal chest radiograph [Figure 2]A and the high-resolution computed tomography (HRCT) of the chest [Figure 2]B and [Figure 2]C. The sputum was negative for acid-fast bacilli. The skin biopsy of the patient was re-evaluated as the radiological findings were not indicative of tuberculosis. On immunohistochemical staining, it was positive for S-100 and CD1a [Figure 3]A and [Figure 3]B confirming the diagnosis of systemic Langerhans cell histiocytosis. The patient had near-normal lung function on spirometry with 48 per cent diffusion capacity of carbon monoxide. At six months of follow up, the forced vital capacity dropped by 200 ml, but still was 77 per cent predicted. The patient is continued on oral prednisolone of 5 mg/day, with her symptoms controlled.
Figure 1: (A) Hyperpigmented ill-defined macular rash on medial aspect of the right thigh. (B) Petechial rashes on the hand and forearm.

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Figure 2: (A) X-ray chest posteroanterior view showing bilateral reticular opacity (white arrow). (B & C) High-resolution computed tomography chest lung window showing septal thickening (green arrow), randomly distributed bizarre cysts (white arrow) and multiple centrilobular nodules (red arrow).

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Figure 3: (A) Photomicrograph of skin biopsy (×10); positive for CD1a. (B) Photomicrograph of skin biopsy showing epithelioid cell granuloma positive for CD1a (×40).

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Acknowledgment: Authors acknowledge Dr Ritu Kulshreshtha, department of Pathology, Vallabhbhai Patel Chest Institute, New Delhi, for providing illustrated histopathological images and their interpretations.

Conflicts of Interest: None.


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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