Langerhans cell histiocytosis—a case report

Abstract

A 17-year-old male presented for dermatologic consultation with slightly elevated reddish papules covered by yellowish scales in the scalp for the last two years and reddish and indurated ulcers in the perineum lasting six months. Additional complaints included polyuria, polydipsia, delay in the development of secondary sexual characteristics and hearing loss of the right ear secondary to a medium otitis. Lesions from scalp and perineum were sampled for histopathologic examination and revealed a dense cellular infiltrate made up of mononuclear cells with conspicuous eosinophilic cytoplasm and large cleaved vesicular nucleus, some of them with shapes resembling the format of a kidney and others reminiscent of coffee beans. Numerous intermingling eosinophils were present. The diagnosis of Langerhans cell histiocytosis was then rendered and confirmed by positive immunostaining of neoplastic cells for anti-CD1a and anti-S100 protein antibodies. The work-up revealed diabetes insipidus, hypogonadotropic hypogonadism, hiperprolactenemia, growing-hormone deficiency and thickness of the pituitary stalk. The patient was treated with prednisone and vinblastin based chemotherapy regimen for six months with complete remission, but presented recurrence of some lesions in the scalp, which were handled with topical mustard and corticosteroids. After chemotherapy, the endocrinologic disturbances were corrected with hormonal replacement therapy. The patient is currently in good health with a follow-up of five years. abstRaCt

A Hematologic Disease Disguised as Cutaneous Candidiasis

“…Clinical classification of LCH depends on the number of affected organ systems, being divided into single or multi-system diseases. All systems can be affected, but the most frequent is the bone (80% of cases) followed by the skin (about 40%) [ 2 , 5 ]. Isolated cutaneous LCH in adults is rare (2% in a large series) and can present as a single lesion or, more often, as multifocal [ 4 , 6 ].…”

Langerhans cell histiocytosis: A case report with oral manifestations and the role of pediatric dentists in the diagnosis

“…Recurrent mouth ulceration and gingival necrosis are nonspecific signs that can be caused by various disorders such as leukocyte adhesion deficiency (LAD), hypophosphatasia, scurvy, leukemia, and medication‐related osteonecrosis of the jaw, but when there is hypermobility of teeth, then bone destruction should be suspected and radiological investigation should be requested and this emphasizes the rule of a pediatric dentist in diagnosis of systemic disorders such as LCH . The definitive diagnosis of LCH is made by histopathology where sheets of large histiocytosis with longitudinal groove (coffee‐bean‐like) nuclei and typical and atypical mitoses in a eosinophils rich background, and immunohistochemical stains are of utmost importance in highlighting reactivity to S‐100 and CD1a .…”

“…18 Recurrent mouth ulceration and gingival necrosis are nonspecific signs that can be caused by various disorders such as leukocyte adhesion deficiency (LAD), hypophosphatasia, scurvy, leukemia, and medication-related osteonecrosis of the jaw, but when there is hypermobility of teeth, then bone destruction should be suspected and radiological investigation should be requested and this emphasizes the rule of a pediatric dentist in diagnosis of systemic disorders such as LCH. 19 The definitive diagnosis of LCH is made by histopathology where sheets of large histiocytosis with longitudinal groove (coffee-bean-like) nuclei and typical 15,[20][21][22] A monoclonal antibody against CD207 (langerin) is a highly specific and sensitive protein that is needed for formation of the Birbeck-Broadbent granules. 23 FDG-PET scan is required to exclude intense radiotracer activity in other sites of the body and to assess the response for treatment; in the present case, Tc 99m -MDP Bone Isotope scan was used and showed increased radiotracer uptake within the maxillary and mandibular bones only.…”

Infiltrating the Heart and Kidney: A Rare Pediatric Case of Multisystem Langerhans Cell Histiocytosis from Pakistan

“…A definitive unequivocal diagnosis requires demonstration through electron microscopy of intracytoplasmic ‘Birbeck bodies’ or immunostaining of the cells by anti-CD1a and anti-S100 antibodies, with negativity for anti-CD68 along with positive CD1a. Langerin (CD-207) is another useful highly specific Langerhans cell marker [17]. Additionally, fine needle aspiration (FNA) can be used to establish the extent of disease or recurrence of LCH.…”