Indian Journal of Radiology Indian Journal of Radiology  

   Login   | Users online: 1085

Home Bookmark this page Print this page Email this page Small font sizeDefault font size Increase font size     

 

MUSCULOSKELETAL IMAGING Table of Contents   
Year : 2002  |  Volume : 12  |  Issue : 3  |  Page : 391-392
Osteolylic lesions in the calvarium an important manifestation of hand - schulter christian disease


Dept of Dermatology, Deccan College of Medical Sciences, Princess Esra Hospital, Hyderabad AP, India

Click here for correspondence address and email
 

Keywords: Hand-Schuller-Christian disease, Langerhan cells histiocytosis, Osteolytic calvarial lesions

How to cite this article:
Sharm V K, Razvi F, Abkari A, Gayatri K. Osteolylic lesions in the calvarium an important manifestation of hand - schulter christian disease. Indian J Radiol Imaging 2002;12:391-2

How to cite this URL:
Sharm V K, Razvi F, Abkari A, Gayatri K. Osteolylic lesions in the calvarium an important manifestation of hand - schulter christian disease. Indian J Radiol Imaging [serial online] 2002 [cited 2020 Aug 4];12:391-2. Available from: http://www.ijri.org/text.asp?2002/12/3/391/28490
A 2 year old male child weighing 8Kg presented with erythematous papules and greasy crusting over the scalp, forehead, chest and back in the seborrheic distribution since one year of age [Figure - 1]. The child also had polyurea and polydipsia and was failing to thrive. On examination the child had purulent discharge from the right ear with hepatomegaly two fingers below the right costal margin. There was no exophthalmos. Soft to firm swellings of 1-2 inch in diameter three in number were present over the right temple and the parietal areas. There was no generalized Lymphadenopathy

Investigations revealed hemoglobin to be 8gm%, WBC 6,500 cells/c. mm, normal differential and platelet count. Ultrasound abdomen revealed hepatomegaly. The specific gravity of urine was 1.005. Microscopic and biochemical examination of urine were normal. X-ray of the A.P. and lateral views of the skull revealed evidence of 3 punched out lesions without sclerotic margins in the anterior frontal and mid parietal bones [Figure - 2]. Sellar size, shape and contour were normal. Evidence of wormian bones were seen posteriorly in the occipital bones. Anterior fontanels were patent. X-ray chest was normal and a bone survey of the long bones and girdles revealed no abnormality. A diagnosis of langerhan cell histiocytosis was considered. The skin biopsy of the lesion taken from the back revealed heavy diffuse infiltration of the upper dermis by round to polygonal monomorphic cells having moderate acidophilic cytoplasm and round nucleus with nuclear grooving in some [Figure - 3]. Foam cells and multinucleated giant cells are not seen. Immunohistochemistry of the cells of the dermal infiltrate revealed S-100 proteins positivity [Figure - 4].


   Discussion Top


Langerhan cells histiocytosis (LCH) previously called Histiocytosis X is a disease of unknown cause characterized by the proliferation of a distinct cell type that is S-100 and CD 1A positive and contains cytoplasmic langerhans granules. LCH includes 4 main clinical forms. These are Letterer - Siwe disease, Hand- Schuller Christian disease, Eosinophilic granuloma and Hashimoto-Pritzker disease.

Hand - Schuller Christian disease is a chronic progressive multifocal form of LCH beginning between 2nd to 6th year of life in 70% of patients. The disease is characterized by bone lesions, diabetes insipidus, exopthalmos and muco-cutaneous lesions[1],[2],[3]. It is uncommon for all the four manifestations to be seen together in the same patient. The present case also showed all the above features except exopthalmos.

Bone lesions are the most common manifestations of Hand-Schuller-Christian disease seen in 80% of patients, preferentially involving the temporo parietal region of the calvarium [4]. Thus includes the osteolytic lesions in the temporal and parietal bones and the involvement of the mastoid region. Osteolytic lesions are associated with diabetes insipidus [5] and the mastoid involvement is associated with otitis media. All the above features were seen in the case under discussion.

The skeletal survey was undertaken by taking radiographs of the mandible, oblique view, both legs, A-P and lateral views and X-ray of the pelvis and it was found that no other skeletal lesions were present. This finding re-emphasizes the fact that calvarial involvement in the most common feature of this disease.

Liver involvement is a very common part of multisystem LCH producing hepatomegaly and ascites. In children even mild involvement of the gastrointestinal tract which may not cause overt symptoms of diarrhoea may still cause failure to thrive due to malabsorption. The GIT involvement in the present case was indicated by the low body weight, failure to thrive and mild hepatomegaly. This case is reported for its rarity as the incidence of the disease in the world literature reported is 4-6 cases per million. Multisystem involvement is about 76% and single system involvement is only about 24%. This was proved after a study done involving 58 patient and followed upto 7 years [6]. The case under discussion also showed involvement of the skin, bones, GIT and pituitary gland. The osteolytic lesions in the calvarium are usually associated with a favorable prognosis.

 
   References Top

1.Winkelman RK, Cutaneous syndrome of non x histiocytosis. A review of the macrophage-histiocyte disease of the skin. Arch Dermatol 1981; 117:667  Back to cited text no. 1    
2.Gianott F, Caputo R, Histiocyte syndrome A review J Am Acad Dermatol 1985; 13: 383.  Back to cited text no. 2    
3.Blank M et al, Naevo xanttho endothilioma with occular involvement. Pediatrics 1949; 4: 349  Back to cited text no. 3    
4.Mc Gavran MH, Spady H A, Eosinophilic granuloma of bone. A study of 28 cases. J Bone Joint Surgery 1960; 42: 979-92.  Back to cited text no. 4    
5.Dunger DB et al, The frequency and natural history of diabetes insipidus in children with Langerhans Cell histiocytosis. N Engl J Med 1989; 321: 1157  Back to cited text no. 5    
6.Chu AC, Histiocytosis. In: Champion RH, Burton JL Ebling F JG, eds. Text book of Dermatology, 5th ed. London: Blackwell Scientific Publications, 1992; 2041-64.  Back to cited text no. 6    

Top
Correspondence Address:
V K Sharm
Dept of Dermatology, Deccan College of Medical Sciences, Princess Esra Hospital, Hyderabad AP
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


Rights and PermissionsRights and Permissions


    Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]



 

Top
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  


    Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed3954    
    Printed76    
    Emailed2    
    PDF Downloaded0    
    Comments [Add]    

Recommend this journal