Biomaterial Database

Variability in the Proteus syndrome: report of an affected child with progressive lipomatosis. 1985

J Mücke, and H Willgerodt, and R Künzel, and D Brock

In 1983 the Proteus syndrome was delineated by Wiedemann et al. [12]. We report a 10-month-old girl, a further child affected by the new syndrome. The typical signs are macrodactyly, hemihypertrophy, pigmented nevi, hyperkeratosis, and subcutaneous hamartomatous tumours. Our patient shows an aggressive lipomatosis on the trunk and local relapses after surgical interventions in the regions involved. Histology of the adipose tissue showed considerable anisocytosis and increased cell volume.

UI	MeSH Term	Description	Entries
D006984	Hypertrophy	General increase in bulk of a part or organ due to CELL ENLARGEMENT and accumulation of FLUIDS AND SECRETIONS, not due to tumor formation, nor to an increase in the number of cells (HYPERPLASIA).	Hypertrophies
D007223	Infant	A child between 1 and 23 months of age.	Infants
D007642	Keratosis	Any horny growth such as a wart or callus.	Keratoderma Blennorrhagicum,Keratoma,Keratosis Blennorrhagica,Blennorrhagica, Keratosis,Blennorrhagicas, Keratosis,Blennorrhagicum, Keratoderma,Blennorrhagicums, Keratoderma,Keratoderma Blennorrhagicums,Keratomas,Keratoses,Keratosis Blennorrhagicas
D008068	Lipomatosis	A disorder characterized by the accumulation of encapsulated or unencapsulated tumor-like fatty tissue resembling LIPOMA.	Lipomatoses
D009508	Nevus, Pigmented	A nevus containing melanin. The term is usually restricted to nevocytic nevi (round or oval collections of melanin-containing nevus cells occurring at the dermoepidermal junction of the skin or in the dermis proper) or moles, but may be applied to other pigmented nevi.	Nevus, Melanocytic,Nevi, Melanocytic,Nevi, Pigmented,Pigmented Moles,Melanocytic Nevi,Melanocytic Nevus,Pigmented Nevi,Pigmented Nevus
D010641	Phenotype	The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment.	Phenotypes
D003238	Connective Tissue	Tissue that supports and binds other tissues. It consists of CONNECTIVE TISSUE CELLS embedded in a large amount of EXTRACELLULAR MATRIX.	Connective Tissues,Tissue, Connective,Tissues, Connective
D005260	Female		Females
D005385	Fingers	Four or five slender jointed digits in humans and primates, attached to each HAND.	Finger
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man