ISSN - 0973-0958

Scleredema Adultorum

01/09/2014 00:00:00 https://www.pediatriconcall.com/Journal/images/journal_cover.jpg
   
 

Scleredema Adultorum

Mohammed MTP, Sudhakaran K, Urmila KE.
Department of Pediatrics, Pariyaram medical college, Kannur, Kerala.
Cite this article  Copy Citation
MTP M, K S, KE U. Scleredema Adultorum. Pediatr Oncall J. 2008;5: 146.

Address for Correspondence
Mohammed.M.T.P, Associate Professor, Department of Pediatrics Pariyaram medical college, Pariyaram, Kannur, Kerala.
 
Email
drmtpmohd@yahoo.com
 
A six years old girl, the second child of a non consanguineous couple, was referred from a peripheral hospital with history of sudden onset of hardening and woody feel of skin of neck and chest of about 3 days duration. One week before the onset of this problem she had fever and sore throat which was treated with oral amoxycillin and paracetamol. There were no other significant symptoms. Her past history was unremarkable and she was fully immunized. On examination, the girl was of average built and nourishment, active and alert. Vitals: Pulse - 120/min, BP - 90/60mm of hg, Temp - 98.6ºF, respiratory rate - 30/min.General examination : the skin over the face, neck, shoulders, back and chest was symmetrically involved and was woody and could not be pinched. Extremities were spared. There were no other skin lesions. There was minimal trismus and the throat was congested. Joints were normal. Cardiovascular system examination revealed tachycardia, S3 gallop and a short systolic murmur at left sternal edge. There was no cardiomegaly. She had hepatomegaly. Investigations showed hemoglobin of 10.5gm%, total leucocyte count of 15,500/cumm (66% polymorphs, 26% lymphocytes, 4% eosinophils, 4% monocytes), ESR of 112 mm at end of one hour and platelet count of 6,19,000/cumm. ANA, RA factor were negative. ASO was 400 IU/L. Blood sugar, electrolytes, calcium were normal. ECG showed sinus tachycardia. X ray chest was normal. Echocardiography showed mitral valve prolapse without mitral regurgitation. , Blood culture was sterile. Skin biopsy showed swollen collagen bundles with frozen section showing bluish material in between the collagen fibers. Diagnosis of Scleredema Adultorum was made and the child was observed in the ward for ten days. Antibiotics were given for pharyngitis. Topical soothening cream was applied over the affected skin. Period of hospital stay was uneventful. Tachycardia and S3 gallop subsided and her ESR had come back to normal. The skin tightness was persisting.

Scleredema Adultorum (scleredema of Bushcke) is a rare disorder involving the dermis. Etiology is unknown. It was first described by Abraham Buschke, a German dermatologist (1968 - 1943). The word came from scler- hard + edema - swelling. Majority of cases are seen in adults with diabetes where there is a male preponderance. Approximately 30% cases are seen in children < 10 years. In children females are affected twice more common than males. The onset of disease is preceded by infections like streptococcal pharyngitis, influenza, measles, mumps etc. About 1-2 weeks after the febrile illness there will be sudden onset of diffuse, symmetrical, wooden, non pitting hardening of skin in a sweater distribution - face, neck, thorax, arms etc. Due to tightness of skin, there may be mask like facies, inability to open mouth and restricted chest movements. Occasionally it may be associated with dysarthria and dysphagia. Serosal effusions, ECG changes, monoclonal gammopathy and myeloma have also been reported. The condition has to be differentiated from sclerema, scleroderma, myxedema and dermatomyositis. Diagnosis is confirmed by skin biopsy which shows normal epidermis, thickened dermis due to swelling of collagen bundles and increased amount of mucopolysaccharides in dermis. There is no definite therapy. Antibiotics, steroids penicillamine, PUVA, radiation, electron beam have been tried with varying results. The condition is usually self limiting with active phase lasting for 2-8 weeks and spontaneous and complete resolution occurs in 6 months to 2 years. Recurrences are unusual.
 
Funding
None
 
Conflict of Interest
None
 
References :
  1. Nelson's text book of Pediatrics. 17th edn. Saunders: USA, 2004: 2211-2212.
  2. Scleredema Adultorum. Ed: McIntosh N, Helms P, Smyth R, Logan S. In: Forfar & Arneil's Textbook of Pediatrics: 6th ed. Churchill Livingstone. 2003.
  3. Parmar RC, Bavdekar SB, Bansal SS, Doraiswamy AA, Khambadkone SS. Scleredema adultorum. J Postgrad Med 2000;46:91.  [PubMed]
  4. Scleredema Adultorum. Medicine net.com Available at url: http://www.medicinenet.com/script/main/srchcont.asp-src=scleredema+adultorum&op=mm. Accessed on 1st November 2007.

Last Updated : 01 November 2008 Vol 5 Issue 11 Art #46

Cite this article as: :
MTP M, K S, KE U. Scleredema Adultorum. Pediatr Oncall J. 2008;5: 146.
ask a doctor
Ask a Doctor
Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License
Disclaimer: The information given by www.pediatriconcall.com is provided by medical and paramedical & Health providers voluntarily for display & is meant only for informational purpose. The site does not guarantee the accuracy or authenticity of the information. Use of any information is solely at the user's own risk. The appearance of advertisement or product information in the various section in the website does not constitute an endorsement or approval by Pediatric Oncall of the quality or value of the said product or of claims made by its manufacturer.
0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0