Academic journal article The Journal of the Royal Society for the Promotion of Health

Lessons to Be Learned - Langerhans' Cell Histiocytosis

Academic journal article The Journal of the Royal Society for the Promotion of Health

Lessons to Be Learned - Langerhans' Cell Histiocytosis

Article excerpt


Langerhans' cell histiocytosis (LCH) is a neoplastic disease due to uncontrolled proliferation of Langerhans' cells (LCs); damage to organs involved appears to be due to the various cytokines secreted. The true cause of LCH remains a mystery. LCH can present at any age; there is female predominance and the skin is often the first site to be involved. Scaly papules, vesicles, purpuric nodules, plaques and ulcers are the main diverse presentations typically found on the scalp, flexures (including the perineum), crease below the breast, palms, soles and nails. The patient described here is a 35-year-old Saudi Arabian woman with a vulval ulcer that had been misdiagnosed as an infective lesion 11 months before the correct diagnosis of LCH was made by means of biopsy examination. Diffuse infiltration of LCs with large pale reniform/convoluted nuclei and abundant amphophilic cytoplasm in the company of neutrophils, eosinophils, lymphocytes and plasma cells were observed on light microscopy. Immunohistochemical staining, indicating S100 protein positivity in LCs and the demonstration of Birbeck granules by means of electron microscopy, confirmed the diagnosis.

The 'skin-only' LCH may later progress to involve other organs; alternatively skin lesions may be part of the multisystem, disseminated disease. There is need for careful follow-up of all LCH patients, as the course of the disease cannot be reliably predicted from either the clinical presentation or histology of the lesion. In many cases the prognosis is good, there being limited involvement that does not progress. Surgery or local steroids might suffice as therapy. However, for severely symptomatic disease, in which there are complications associated with dysfunction (or failure) of vital organs such as the liver, lungs, and central nervous system, the use of a variety of immunosuppressive treatments - and the recently described 2-chloro-2-deoxyadenosine (2CdA) (cladribine) - have been suggested; however, the type and extent of disease that will respond to this therapy has not yet been clarified. Solid laboratory research and rigorous clinical trials are required for more effective and less toxic therapies for patients with progressive LCH.

Key words

Birbeck granules; cladribine (2CdA); clonal; cytokines; Langerhans' cell histiocytosis (LCH); Langerhans' cells (LCs)


Langerhans' cell histiocytosis (LCH), previously called histiocytosis X,u is now interpreted as neoplastic proliferation of abnormal LCs that have been joined by a mixture of inflammatory cells, this being analogous to the situation of fully developed plaques of mycosis fungoides in which disorder abnormal T lymphocytes are allied with a mixture of inflammatory cells. This is a challenging disease of unknown cause and mechanism.3 Historical terms for LCH include 'eosinophilic granuloma', 'LettererSiwe disease' and 'Hand-Schiiller-Christian disease'. LCH may manifest in a variety of ways, ranging from spontaneous regression of a solitary lesion of bone to a multisystem life-threatening disorder.4 This rare disorder primarily affects children, mainly boys, but it is also found in adults.5 However, females predominate in patients above 15 years of age.6 Adult LCH patients are true 'orphans', as they cannot find a medical 'home'; if they present to a dermatologist only the skin may be evaluated, a pulmonologist might investigate only the lung problems and a gynaecologist is likely to deal only with female genitalia. Moreover, adult LCH is full of unknowns, because no organized treatment protocols have been in place long enough to define optimal therapies and the natural history of the disease.6

LCH, if not progressive, was previously considered to be a disease of excellent prognosis; this contention, however, has been challenged by recent data which clearly demonstrates that although many adult patients and children above two years of age with LCH do well, a significant proportion do develop considerable morbidity and may die prematurely. …

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