What is hand Schuller Christian disease?

Hand-Schüller-Christian disease belongs to the spectrum of disorders, now known as “eosinophilic granulomatosis” or “Langerhans cell granulomatosis”. It occurs in children and is characterized by the clinical triad of diabetes inspidus, exophthalmos, and lytic bone lesions.

What is letterer SIWE disease?

Letterer-Siwe disease (LSD) is one of the Langerhans cell histiocytoses (LCH). Physiologically, Langerhans cells detect non-self-antigens and present them to the cells of the immune system (T cells), thus allowing an appropriate immune response from the body.

What is Langerhan Cell Histiocytosis?

Langerhans cell histiocytosis is a rare disorder that can damage tissue or cause lesions to form in one or more places in the body. Langerhans cell histiocytosis (LCH) is a rare disease that begins in LCH cells. LCH cells are a type of dendritic cell that normally helps the body fight infection.

How is Langerhans Cell Histiocytosis diagnosed?

Testing for Langerhans cell histiocytosis (LCH) may include bronchoscopy with biopsy , x-ray , skin biopsy, bone marrow biopsy, complete blood count, skeletal X-rays survey, pulmonary function tests and liver funcion tests, as well as MRI and CT scanning of the head to evaluate possible abnormalities of the …

What causes histiocytosis?

The exact cause of histiocytosis is unknown. However, recent studies indicate that it is caused by the development and expansion of an abnormal Langerhans cell that subsequently leads to the accumulation of other cells of the immune system, resulting in collections or tumors in various areas of the body.

Is LCH genetic?

Langerhans cell histiocytosis is usually not inherited and typically occurs in people with no history of the disorder in their family. A few families with multiple cases of Langerhans cell histiocytosis have been identified, but the inheritance pattern is unknown.

What does a letterer do?

A letterer is a member of a team of comic book creators responsible for drawing the comic book’s text. The letterer’s use of typefaces, calligraphy, letter size, and layout all contribute to the impact of the comic.

What is the survival rate of Langerhans cell histiocytosis?

What are the survival rates for Langerhans cell histiocytosis? About 99% of children with low-risk LCH survive. About 80% of children with high-risk LCH survive.

Is histiocytosis an autoimmune disease?

Langerhans cell histiocytosis historically was thought of as a cancer-like condition, but more recently researchers have begun to consider it an autoimmune phenomenon in which immune cells begin to overproduce and attack the body instead of fighting infection.

Does Langerhans Cell Histiocytosis go away?

In many people with Langerhans cell histiocytosis, the disorder eventually goes away with appropriate treatment. It may even disappear on its own, especially if the disease occurs only in the skin.

Is Langerhans Cell Histiocytosis fatal?

Langerhans cell histiocytosis can cause damage to tissues and organs all over the body if it’s not treated. One example is pulmonary histiocytosis. This condition damages the lungs. Damage to the body can be so severe that the condition becomes fatal.

How do you treat histiocytosis?

Histiocytosis Treatment

1. Surgery. Sometimes histiocytosis involves only one area of the body.
2. Radiation Therapy. Radiation therapy can be extremely effective for certain types of histiocytosis, especially Langerhans cell histiocytosis.
3. Immunosuppressants and Chemotherapy.
4. Targeted Medications for Histiocytosis.

Is there such a thing as Hand Schuller Christian disease?

CONCLUSION: This is a rare case of Hand-schuller-Christian disease and affecting both the twin male children is rarest among the rare. and multifocal Langerhans’ cell histiocytosis (previously known as Hand-Schuller-Christian disease ).

Which is a synonym for Schuller Christian disease?

Synonym (s): Christian disease (1) , Christian syndrome, Schüller syndrome. A condition of multiple destructive HISTIOCYTOSIS of bone causing skull defects, pituitary gland disturbances with DIABETES INSIPIDUS, middle ear infection and loss of teeth.

What kind of lung disease does Hand Schuller have?

The histiocytosis X cells are derived from Langerhans cell origin and are positive for CD1a and HLA-DR. Pulmonary interstitial lung disease occurs in approximately 20% of patients with eosinophilic granuloma. Chest radiographs demonstrate an alveolar pattern in an early state.

Is the Hand Schuller disease a reactionary lesion?

However, a lesion that is histologically somewhat similar (eosinophilic granuloma or ulcer) can be traumatic in origin or reactionary without any history of trauma, and there is some doubt about the nature of eosinophilic granulomas of soft tissues reported in the past.