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Disabling pansclerotic morphoea of childhood. BMJ Case Rep 2018 Feb 17;2018

Date

02/20/2018

Pubmed ID

29455178

Pubmed Central ID

PMC5836610

DOI

10.1136/bcr-2017-222132

Abstract

Disabling pansclerotic morphoea (DPM) of childhood is a severe and often fatal variant of deep morphoea. It usually starts in childhood and rarely seen in adults. The course of the disease is progressive with lifelong morbidity in the form of joint contractures and immobility. The causes of mortality include complications such as sepsis, gangrene and cardiopulmonary involvement. Herein, we discuss the case of a 15-year-old girl with limb deformity and finger contractures, that is, bone involvement. The diagnosis of DPM of childhood was fortuitously made after the correction of limb deformity, when the patient was seen in the dermatology department for evaluation of skin discolouration on the thighs.

Author List

Jamalpur I, Mogili HR, Koratala A

Author

Abhilash Koratala MD Assistant Professor in the Medicine department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Adolescent
Biopsy
Contracture
Disease Progression
Elastic Tissue
Extremities
Female
Fingers
Humans
Scleroderma, Localized
Skin