Dermatology Online Journal

Cutaneous Langerhans cell histiocytosis in an elderly woman
Jenny Chong Hu MD MPH¹, Seong Ra MD², Miguel A Gutierrez MD¹
Dermatology Online Journal 16 (10): 6

1. Division of Dermatology. jennhu@ucla.edu
2. Department of Pathology
David Geffen School of Medicine at UCLA

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Abstract

Langerhans cell histiocytosis (LCH) is a clonal proliferative disorder of Langerhans cells typically seen in infants and children. Rare adult cases usually have systemic involvement. We report an uncommon case of skin-limited LCH in an elderly woman, who is to our knowledge one of the oldest patients reported with this condition.

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Case report

Figure 1A	Figure 1B
Figures 1A and 1B. Erythematous papules, plaques, and nodules of face, trunk, and extremities

Figure 2A	Figure 2B
Figure 2A. Sheets of atypical histiocytoid cells with characteristic “coffee bean-like” nuclei replacing the dermis and showing some epidermotropism into the overlying epidermis (H&E, x200). Figure 2B. Immunohistochemical studies with positive staining for CD1a

An 83-year-old woman, with a history of hypertension and gastritis, presented with an 18-month history of pruritic papules and nodules that began on her face and gradually progressed to involve her trunk and extremities. She reported a 17-pound weight loss over the past several months, but otherwise denied other constitutional or systemic symptoms including fever, bone pain, and dyspnea. On physical exam, she had erythematous plaques and nodules on her face (Figures 1A and 1B) and erythematous papules on her trunk and upper and lower extremities. Lymphadenopathy and hepatosplenomegaly were absent.

Histopathological examination demonstrated sheets of atypical histiocytoid cells with characteristic “coffee bean-like” nuclei replacing the dermis and showing some epidermotropism into the overlying epidermis (Figure 2A). Immunohistochemical studies showed positive staining with antibodies to S-100, CD68, and CD1a (Figure 2B), consistent with Langerhans cell histiocytosis. A complete bone survey; CT brain, neck, chest, abdomen; radionuclide whole body bone scan; bone marrow aspiration; and bone marrow core biopsy including fluorescent in situ hybridization analysis were all negative for systemic involvement. Despite the extensive work-up, the cause of the patient’s weight loss was not determined. She refused treatment of her skin disease and was subsequently lost to follow-up.

Discussion

Langerhans cell histiocytosis (LCH) is a clonal proliferative disorder of Langerhans cells typically seen in infants and children [1]. It can be subdivided into the clinical variants of Letterer-Siwe disease, Hand-Schuller-Christian disease, eosinophilic granuloma, and congenital self-healing reticulohistiocytosis [2, 3]. However, these variants have some overlapping clinical features and, thus, many no longer classify LCH into separate clinical variants [3]. Instead, many recognize LCH as a disease with a broad clinical spectrum [2, 3]. Occurrences in the adult population are rare, but even rarer in the elderly. Furthermore, adults uncommonly present with the disease limited to the skin [4, 5]. The clinical presentation of cutaneous LCH in adults is variable. It can present as papules, plaques, or nodules localized to a single anatomic site or in a generalized distribution; pruritic papules or nodules appearing as prurigo nodularis; scaling eruptions mimicking seborrheic dermatitis, eczema, or dermatophytosis; and papules or ulcers of the external genitalia [4, 5, 6, 7]. Sites most frequently involved include the scalp, trunk, flexural and intertriginous areas, glabrous skin, and external genitalia [4, 5]. Systemic involvement – most frequently of the bone, lungs, liver, lymph nodes, hypothalamic-pituitary axis, and central nervous system – may occur several years following initial skin lesions [5]. Patients who have systemic involvement often present with fever and weight loss, as well as extensive skin disease [8]. Patients with limited cutaneous involvement often carry a better prognosis than those with systemic disease and prognosis correlates with the extent of systemic disease [5, 8]. Patients with systemic involvement may die from LCH directly or associated conditions such as bronchopneumonia and congestive heart failure [8]. Langerhans cell histiocytosis has also been associated with hematologic malignancies and solid tumors, including lung and breast cancers [8]. Therefore, prompt diagnosis and close monitoring for the possible development of systemic disease is imperative, especially in adult patients who present with skin-limited disease. Patients with a new diagnosis of LCH should undergo a full work-up including complete blood count, chemistry panel including renal function tests, liver function tests, sedimentation rate, and C-reactive protein. There should be a consideration for obtaining a complete bone survey, CT whole body, liver biopsy, bone marrow biopsy, and evaluation for diabetes insipidus (a manifestation of posterior pituitary dysfunction).

Histologically, LCH demonstrates a proliferation of Langerhans cells with reniform nuclei in the papillary dermis with infiltration into the epidermis. Often, there is an admixture of inflammatory cells in the dermis, including eosinophils, neutrophils, lymphocytes, and plasma cells. Langerhans cells demonstrate positive staining with antibodies to S-100 protein and CD1a. On electron micrograph, these cells also reveal racquet-shaped Birbeck granules in their cytoplasm [9].

Successful treatments for cutaneous LCH have included topical steroids, systemic steroids, oral isotretinoin, thalidomide, nitrogen mustard, radiation, excimer laser, surgical resection, interferon-α, and chemotherapy [4, 5, 6, 10, 11]. Systemic involvement is typically treated with chemotherapy [8].

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