INTRODUCTION Granulomatous inflammation is a distinctive ...

Balancing control of the disease with the possibility of serious side–effects of treatment is often
challenging. For PACNS, treatment must often be continued for a year or more.
Patients who fit the typical patient profile of BACNS and who have clinical presentations
compatible with that diagnosis may be candidates for less intensive treatment regimens than those
used in clear–cut cases of PACNS. Patients believed to have BACNS may be treated with calcium–
channel blockers (a class of drug used to treat high blood pressure and spasm of blood vessels that
occurs in a variety of conditions) for a few weeks, along with a comparatively short course of
steroids (prednisone). However, no firm guidelines exist regarding the length of therapy, however,
and decisions about the length of treatment must be made on a case–by–case basis (Scolding et al.,
1997).
Langerhans cell histiocytosis
Histiocytosis is a rare blood disease that is caused by an excess of white blood cells called
histiocytes. The histiocytes cluster together and can attack the skin, bones, lung, liver, spleen,
gums, ears, eyes, or the CNS. The disease can range from limited involvement that spontaneously
regresses to progressive multiorgan involvement that can be chronic and debilitating. In some
cases, the disease can be life-threatening. In some ways, histiocytosis is similar to cancer and has
historically been treated by oncologists with chemotherapy and radiation. Unlike cancer,
histiocytosis sometimes goes into remission without treatment.
It is estimated that 1 in 200,000 children are affected each year. 76 percent of the cases occur
before 10 years of age, but the disease is also seen in adults of all ages. Langerhans cell
histiocytosis has also been known as Histiocytosis-X, Eosinophilic Granuloma, Letterer-Siwe
disease, and Hand-Schuller-Christian Syndrome. The cause of LCH is unknown. It may be
triggered by an unusual reaction of the immune system from something commonly found in the
environment (Vassallo et al., 2000).
Clinical picture
The LCH can affect many organs causing varied clinical picture. Skin Involvement manifest as
scaly, waxy rash that does not respond to treatment. Bone involvement is in the form of Single or
multiple lesions, causing bone pain, headaches (skull lesions), and limping (leg lesions). The eye
may be involvement with vision problems or eye bulging. The gastrointestinal tract manifest as
abdominal pain and jaundice, vomiting, diarrhea, bleeding from the esophagus, and weight loss.
The affection of the lungs causes feeding problems (infants), vomiting, diarrhea, chest pain,
labored breathing, failure to thrive, cough, haemoptesis, and weight loss. The ear Involvement
manifests as inflamed condition of ear canal, rash behind ear or on scalp, formation of cysts in the
ear, and oozing from ear (foul-smelling discharge) (Sheehan and Chu, 1991).
Diabetes insipidus is the hallmark of involvement of the hypothalamic-pituitary region and is the
most common and best recognized feature of CNS disease in LCH. Hypothalamic pituitary
involvement is seen in about 10 percent of LCH patients. The incidence ranges from 5 to 50
percent in the different reports, according to the various stratification systems or treatment
approaches. Polyuria and polydipsia as symptoms of posterior pituitary dysfunction are the most
frequent finding in about 90 percent of patients with morphological changes in this region.
However, other endocrine deficiencies like growth failure, precocious or delayed puberty,
amenorrhea, hypothyroidism, or hypocortisolism resulting from anterior pituitary failure, are
seen in about 50 percent of the DI patients. In rare cases of hypothalamic tumors, disturbances of
appetite and social behavior with binge or rage attacks and dysregulation of sleep or temperature
may be seen (Vassallo et al., 2000).