Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2012 Jan;57(1):58-60.
doi: 10.4103/0019-5154.92683.

Multisystem Langerhans cell histiocytosis in adult

Affiliations

Multisystem Langerhans cell histiocytosis in adult

Anubhav Garg et al. Indian J Dermatol. 2012 Jan.

Abstract

Langerhans cell histiocytosis (LCH), is a rare disorder, clinically presents with heterogeneous manifestations, and has an unpredictable outcome. Commonly seen in infancy or early childhood, the disorder is characterized by proliferation of abnormal and clonal Langerhans cell in skin, bone, lymph nodes, lungs, liver, spleen, and bone marrow. Occurrence of LCH in adults is rare. Here, we report the case of an adult with acute onset of polymorphic eruptions all over the body, which on biopsy showed features of multisystem LCH, and was confirmed by immunohistochemistry. Although multisystem LCH has a poor prognosis, our patient responded well to chemotherapy.

Keywords: Adult LCH; histiocytic infiltrate; langerhans cell histiocytosis.

PubMed Disclaimer

Conflict of interest statement

Conflict of Interest: Nil.

Figures

Figure 1
Figure 1
Scaling with crusting present over face, mimicking seborrheic dermatitis
Figure 2
Figure 2
Yellow-brown papules with pustules present over chest
Figure 3
Figure 3
Ulcers present over lips
Figure 4
Figure 4
Diffuse histiocytic infiltrate, H and E staining, ×10
Figure 5
Figure 5
Histiocytic infiltrate, H and E staining, ×100
Figure 6
Figure 6
S-100 positive, ×100

References

    1. Lichtenstein L. Histiocytosis X; Integration of eosinophilic granuloma of bone, Letterer–Siwe disease and Hand-Schuller-Christian disease, as related manifestation of single nosologic entity. Arch Pathol. 1953;56:84–102. - PubMed
    1. Howarth DM, Gilchrist GS, Mullan BP, Wiseman GA, Edmonson JH, Schomberg PJ. Langerhans cell histiocytosis: diagnosis, natural history, management and outcome. Cancer. 1999;85:2278–90. - PubMed
    1. Munn S, Chu AC. Langerhans cell histiocytosis of the skin. Haematol Oncol Clin North Am. 1998;12:269–86. - PubMed
    1. Chu T, Angio GJ, Farara BE. The writing group of the histiocytic society: Histiocytosis syndromes in children. Lancet. 1987;1:208–9. - PubMed
    1. Vollum DI. Letterer-Siwe disease in adult. Clin Dermatol. 1979;4:395–406. - PubMed