Biomaterial Database

En coup de sabre accompanied by pachydermoperiostosis: a case report. 2007

M Ozdemir, and S Yildirim, and I Mevlitoğlu

Department of Dermatology, Meram Medical Faculty, Selçuk University, Konya, Turkey. mustafaozdemir@yahoo.com

Scleroderma en coup de sabre, a variant of localized scleroderma is a disorder characterized by fibrosis of connective tissue. We report a 21-year-old female with scleroderma en coup de sabre accompanied by pachydermoperiostosis. She was born to consanguineous parents and her older sister also had pachydermoperiostosis characterized by clubbing of the digits, enlargement of distal parts of the extremities. The two disorders were diagnosed by clinical examination, histological and x-ray findings. In contrast to scleroderma, pachydermoperiostosis is a hypertrophic process characterized by periosteal proliferation of the tubuler bones and hypertrophic skin changes. We discuss this interesting coexistence and review the literature.

UI	MeSH Term	Description	Entries
D010004	Osteoarthropathy, Primary Hypertrophic	A condition chiefly characterized by thickening of the skin of the head and distal extremities, deep folds and furrows of the skin of the forehead, cheeks, and scalp, SEBORRHEA; HYPERHIDROSIS; periostosis of the long bones, digital clubbing, and spadelike enlargement of the hands and feet. It is more prevalent in the male, and is usually first evident during adolescence. Inheritance is primarily autosomal recessive, but an autosomal dominant form exists.	Pachydermoperiostosis,Acropachy, Hereditary,Clubbing of Digits,Cranioosteoarthropathy,Currarino Idiopathic Osteoarthropathy,Digital Clubbing, Isolated Congenital,Familial Idiopathic Osteoarthropathy Of Childhood,Hypertrophic Osteoarthropathy, Primary, Autosomal Dominant,Hypertrophic Osteoarthropathy, Primary, Autosomal Recessive,Idiopathic Hypertrophic Osteoarthropathy,Pachydermoperiostosis, Autosomal Dominant,Pachydermoperiostosis, Autosomal Recessive,Primary Hypertrophic Osteoarthropathy, Autosomal Dominant,Touraine-Solente-Gole Syndrome,Acropachies, Hereditary,Autosomal Dominant Pachydermoperiostoses,Autosomal Dominant Pachydermoperiostosis,Autosomal Recessive Pachydermoperiostosis,Cranioosteoarthropathies,Hereditary Acropachies,Hereditary Acropachy,Hypertrophic Osteoarthropathy, Idiopathic,Hypertrophic Osteoarthropathy, Primary,Osteoarthropathy, Currarino Idiopathic,Osteoarthropathy, Idiopathic Hypertrophic,Pachydermoperiostoses, Autosomal Dominant,Primary Hypertrophic Osteoarthropathy,Recessive Pachydermoperiostosis, Autosomal,Touraine Solente Gole Syndrome
D010005	Osteoarthropathy, Secondary Hypertrophic	Symmetrical osteitis of the four limbs, chiefly localized to the phalanges and the terminal epiphyses of the long bones of the forearm and leg, sometimes extending to the proximal ends of the limbs and the flat bones, and accompanied by dorsal kyphosis and joint involvement. It is often secondary to chronic conditions of the lungs and heart. (Dorland, 27th ed)	Clubbed Fingers,Marie-Bamberger Disease,Hypertrophic Osteoarthropathy, Secondary,Secondary Hypertrophic Osteoarthropathy,Clubbed Finger,Disease, Marie-Bamberger,Finger, Clubbed,Fingers, Clubbed,Hypertrophic Osteoarthropathies, Secondary,Marie Bamberger Disease,Osteoarthropathies, Secondary Hypertrophic,Secondary Hypertrophic Osteoarthropathies
D001842	Bone and Bones	A specialized CONNECTIVE TISSUE that is the main constituent of the SKELETON. The principal cellular component of bone is comprised of OSTEOBLASTS; OSTEOCYTES; and OSTEOCLASTS, while FIBRILLAR COLLAGENS and hydroxyapatite crystals form the BONE MATRIX.	Bone Tissue,Bone and Bone,Bone,Bones,Bones and Bone,Bones and Bone Tissue,Bony Apophyses,Bony Apophysis,Condyle,Apophyses, Bony,Apophysis, Bony,Bone Tissues,Condyles,Tissue, Bone,Tissues, Bone
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D001284	Atrophy	Decrease in the size of a cell, tissue, organ, or multiple organs, associated with a variety of pathological conditions such as abnormal cellular changes, ischemia, malnutrition, or hormonal changes.	Atrophies
D012594	Scleroderma, Localized	A term used to describe a variety of localized asymmetrical SKIN thickening that is similar to those of SYSTEMIC SCLERODERMA but without the disease features in the multiple internal organs and BLOOD VESSELS. Lesions may be characterized as patches or plaques (morphea), bands (linear), or nodules.	Dermatosclerosis,Morphea,Scleroderma, Circumscribed,Frontal Linear Scleroderma en Coup de Sabre,Linear Scleroderma,Scleroderma, Linear,Circumscribed Scleroderma,Localized Scleroderma,Morpheas,Sclerodermas, Localized
D012867	Skin	The outer covering of the body that protects it from the environment. It is composed of the DERMIS and the EPIDERMIS.