BACKGROUND Congenital self-healing Langerhans cell histiocytosis (Hashimoto-Pritzker disease) is the rarest form of Langerhans cell histiocytosis, usually confined to the skin and/or mucous membranes. Cutaneous eruption is mostly generalized, papular, nodular or vesicular. Despite impressive clinical presentation in a newborn it infrequently spreads to internal organs (which then portends a gra...

BACKGROUND The purpose of this study was to test the disease-controlling effect of low-dose oral etoposide monotherapy in adult-onset multisystem Langerhans cell histiocytosis. There are no previous reports of low-dose etoposide monotherapy for this condition. OBSERVATIONS A 27-year-old man with a 7-year history of multifocal chronic Langerhans cell histiocytosis presented with severe disabli...

The lungs may be involved in patients with histiocytosis X as part of the generalised disease, but histiocytosis X confined to the lungs without extrapulmonary involvement is rare. This report describes two cases of primary pulmonary histiocytosis arising in patients with Hodgkin's disease. It is impossible to state with certainty whether the pulmonary histiocytosis arose as a response to the d...

Histiocytosis represents a group of rare disorders characterized by nonneoplastic proliferation of histiocytes and solitary cutaneous reticulohistiocytosis (SCR) is a form of non-Langerhans histiocytosis. SCR consists of small papule, usually single, varying in color from yellow to brownish-red, more frequent in young adults, and uncommon in childhood. We report a case of SCR in an 11-year-old ...

Histiocytosis X, or Langerhans cell histiocytosis (LCH), is a rare disease, with an estimated incidence of 1/200,000 per year in children under 15 years of age. It has a wide clinical spectrum, from single bone involvement (eosinophilic granuloma) to multisystemic disease with organ failure. The treatment of LCH is still controversial. While single system disease may spontaneously recover or re...

Pulmonary Langerhans' cell histiocytosis (PLCH) remains an important diagnostic consideration in the differential diagnosis of diffuse infiltrative lung disease, particular among smokers. This review highlights recent progress in our understanding of the etiology, clinical presentation, and diagnostic and therapeutic approaches to this unusual lung disorder. Evolving studies strongly link the b...

Pulmonary Langerhans cell Histiocytosis is a rare granulomatous disease affecting both sexes, with greater incidence in the second and third decades of life; smoking appears to be the most important risk factor. Its etiology is unknown, although there are data indicating an uncontrolled immune response as possible cause, sustained by the Langerhans cells, antigen presenting cells for T lymphocy...

A 5 year old boy presented with papules and nodules on face, chest and upper extremities and seborrhea of scalp for 4 years. Some old healed scars were also present. Lesions were pruritic to begin with but were non-itchy. Examination revealed erythematous plaques and nodules on face and upper limbs. Lesions were more marked on forehead, cheek (Fig. 1) and dorsum of hands. There were contracture...

Langerhans cell histiocytosis (LCH) is a rare disorder with numerous clinicopathological variants with differing clinical courses, treatment methods, and prognoses. We report one patient with atypical LCH of the bilateral lower eyelids and subsequent successful treatment with local radiation therapy.

We describe a pediatric patient with histiocytic sarcoma involving the T6 and L4 vertebral bodies and the lungs. His tumor progressed during chemotherapy designed for Langerhans' cell histiocytosis and sarcoma. High-dose radiation, on the other hand, was effective.