Onset occurs in childhood before the age of 16 years and as early as 2 years in 0.
Shagreen patch associated with tuberous sclerosis Connective tissuse naevi Familial cutaneous collagenoma lesions usually appear during adolescence. They are characterised by multiple hard nodules of varying sizes over the upper two thirds of the back. They are sometimes associated with cardiac disease.
Shagreen patch is commonly associated with tuberous sclerosis. Elastomas Elastomas or elastin naevi include: Isolated elastomas Buschke-Ollendorf syndrome Elastosis perforans serpiginosa Buschke-Ollendorf syndrome is a rare hereditary disorder where there is an increased accumulation of elastin in the dermis elastoma.
Lesions may be present at birth but more usually appear within the first year of life. They are firm yellowish wrinkled nodules often group together to form plaques.
The abdomen, back, buttock, thighs or arms are commonly affected.
—The first 3 cases of connective tissue nevus were reported by Lewandowsky 3 () under the name of ``naevus elasticus regionis mammariae.'' Lipschütz 4 in reported 4 more cases as instances of Pflastersteinnaevi (paving stone nevi). In the same year, , With and Kissmeyer, 5 after they read the paper by. The cerebriform connective tissue nevus progressed by expansion into previously uninvolved skin, increased thickness, and development of new lesions. Lipomas increased in size and/or number in 8/10 children with lipomas. In contrast, epidermal nevi and vascular malformations generally did not spread or increase in number. Connective tissue nevus (CTN) is a hamartoma of various components of dermal connective tissue elements, including collagen, elastin, or glycosaminoglycan, showing either an increase, normal range, or decrease in .
Other manifestations of the syndrome appear with time and may include osteopoikilosis inherited bone disorder identified on X-Rayeye disorders and spinal problems. Elastosis perforans serpiginosa EPS is a perforating disorder.
In this case, abnormal elastic fibres are extruded through the epidermis. Groups of scaly papules are generally arranged in an arc or ring shape.
EPS is associated with other disorders of connective tissue such as Marfan syndromeEhler Danlos syndromeosteogenesis imperfecta, pseudoxanthoma elasticum and Down syndrome. Elastosis perforans serpiginosa Other connective tissue naevi Congenital smooth muscle hamartoma is first noted at birth or during the first few weeks of life.
It is usually a single irregularly shaped lesion that once established remains unchanged. Fat naevus naevus lipomatosus superficialis occurring at birth includes the very rare Michelin tyre baby disorder that is characterised by generalised folding of redundant skin.
Congenital fibromatosis infantile myofibromatosis is characterised by single or multiple benign tumours that appear to be derived from connective tissue and smooth muscle cells. They are present at birth or develop within the first few weeks of life. Lesions usually resolve spontaneously, however severe or widespread involvement of internal organs may cause potentially life-threatening complications.
Mucinous naevus is formed by a deposit of mucin and can be present at birth or develop later in life.
Treatment of connective tissue naevi Connective tissue naevi that are not associated with other diseases do not require any treatment. Patients with naevi should undergo full examination to rule out any associated conditions.Number: Policy Introduction.
Aetna plans exclude coverage of cosmetic surgery that is not medically necessary, but generally provide coverage when the surgery is needed to improve the functioning of a body part or otherwise medically necessary even if the surgery also improves or changes the appearance of a portion of the body.
(Case report followed by a retrospective analysis of connective tissue nevi of the collagen-type as related to CD34 positivity.) Shah, KN, Anderson, E, Junkins-Hopkins, J, James, WD.
"Medallion-like dermal dendrocyte hamartoma". Collagenoma, a type of Connective tissue nevus, is an rare condition in which the cells in the deeper layers of the skin experience an excess of collagen.
There are two subtypes of collagenoma: shagreen patches and familial cutaneous Collagenoma (FCC). Additional types of nevi do not involve disorders of pigmentation or melanocytes.
These additional nevi represent hamartomatous proliferations of the epithelium, connective tissue, and vascular malformations. Epidermal nevi. These nevi represent excess growth of specific cells types found in the skin, including those that make up oil and sweat glands.
Although rare, a brown discoloration of the nails may be seen with certain types of skin cancers like a malignant melanoma or basal cell carcinoma. The discoloration evident on the fingernails may actually be due to the cancer affecting the nail bed. secondary AIHA: autoimmune diseases (e.g.
SLE) lymphoproliferative disorders; immune deficiencies (e.g. CVID); infections (e.g. mycoplasma, EBV).