Langerhans Cell Histiocytosis

 Histiocytosis X


image Eosinophilic granuloma


• Defined as clonal proliferation of Langerhans cells




Clinical Issues




• Newborns and infants usually present with limited skin or bone lesions

• Erythematous, crusted, vesiculopustular rash

• Common sites include skin, bone and bone marrow, and lung

• M:F = 2:1

• Eosinophilic granuloma is restricted to bone

• Hand-Schüller-Christian disease has multiple organ involvement and diabetes insipidus

• Letterer-Siwe disease is aggressive with multisystem involvement


Microscopic




• Langerhans cells infiltrate dermis and subcutis and often extend throughout epidermis to stratum corneum
• Langerhans cells show

image Abundant eosinophilic show cytoplasm

image Coffee bean-shaped nucleus

• Large numbers of eosinophils and other inflammatory cells typically present

• Mitoses common, but usually not atypical


Ancillary Tests




• CD1a and S100 immunostaining

• Birbeck granules seen on EM


Top Differential Diagnoses




• Granulomatous infiltrates

• Rosai-Dorfman disease

• Indeterminate cell histiocytosis

• Hodgkin and non-Hodgkin lymphoma

image
Clinical Photograph of Langerhans Cell Histiocytosis
This example of Langerhans cell histiocytosis (LCH) of the skin presented as a seborrheic-like erythematous rash with petechiae on the abdomen of a 23-month-old child. (Courtesy S. Vanderhooft, MD.)


image
Dermal Involvement by Langerhans Cell Histiocytosis
This example of LCH shows dense collections of histiocytic cells in the superficial dermis, with overlying epidermal atrophy and serum crust containing neutrophils. Scattered dermal eosinophils are also seen image.

image
Higher Magnification of Langerhans Cell Histiocytosis
Higher magnification shows the characteristic cytologic features, including folded or coffee bean-shaped nuclei image, and abundant eosinophilic-staining cytoplasm. This field also shows a mitosis image and a few large multinucleated cells, which may seen in some cases.

image
CD1a Immunohistochemistry in Langerhans Cell Histiocytosis
The Langerhans cells show positive cytoplasmic and membranous staining for CD1a.


TERMINOLOGY


Abbreviations




• Langerhans cell histiocytosis (LCH)


Synonyms




• Histiocytosis X

• Eosinophilic granuloma


Definitions




• Clonal proliferation of Langerhans cells


CLINICAL ISSUES


Epidemiology




• Age
image First 3 decades of life; rarely occurs in older adults

• Sex
image M:F = 2:1


Site




• Skin
image Erythematous, crusted, vesiculopustular rash, or salmon-colored macular-papular rash

• Bone and bone marrow
image Skull, pelvis, long bones, vertebrae

• CNS
image Primary in dura, leptomeninges, or parenchyma

image May be secondary to skull or vertebral involvement

• Other organs that may be affected include lung, lymph nodes, liver, thymus, and GI tract


Presentation




• Newborns and infants usually present with limited skin or bone lesions
• Generalized disease is more common in young children

• Rash may precede systemic findings by several months

• Eosinophilic granuloma

image Single or multiple lesions restricted to bone

Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Langerhans Cell Histiocytosis
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