Mohammed MTP, Sudhakaran K, Urmila KE.
Department of Pediatrics, Pariyaram medical college, Kannur, Kerala.
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. | | | | Compliance with Ethical Standards | | Funding None | | | | Conflict of Interest None | | |
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| Cite this article as: | | MTP M, K S, KE U. Scleredema Adultorum. Pediatr Oncall J. 2008;5: 146. |
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