Poikiloderma is a skin condition that consists of areas of
hypopigmentation
Hypopigmentation is characterized specifically as an area of skin becoming lighter than the baseline skin color, but not completely devoid of pigment. This is not to be confused with depigmentation, which is characterized as the absence of all pi ...
,
hyperpigmentation
Hyperpigmentation is the darkening of an area of skin or nails caused by increased melanin.
Causes
Hyperpigmentation can be caused by sun damage, inflammation, or other skin injuries, including those related to acne vulgaris.James, William; Ber ...
,
telangiectasias
Telangiectasias, also known as spider veins, are small dilated blood vessels that can occur near the surface of the skin or mucous membranes, measuring between 0.5 and 1 millimeter in diameter. These dilated blood vessels can develop anywhere on ...
and
atrophy
Atrophy is the partial or complete wasting away of a part of the body. Causes of atrophy include mutations (which can destroy the gene to build up the organ), poor nourishment, poor circulation, loss of hormonal support, loss of nerve supply t ...
.
Poikiloderma of Civatte is most frequently seen on the chest or the neck, characterized by red colored pigment on the skin that is commonly associated with sun damage.
Types
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Poikiloderma vasculare atrophicans
Poikiloderma vasculare atrophicans (PVA), is a cutaneous condition (skin disease) characterized by hypo- or hyperpigmentation (diminished or heightened skin pigmentation, respectively), telangiectasia and skin atrophy. Other names for the conditi ...
*
Poikiloderma of Civatte
Poikiloderma of Civatte is a cutaneous condition and refers to reticulated red to red-brown skin patches with telangiectasias. It is identifiable as a reddish-brown discoloration on the side of the neck, usually on both sides. It is more common in ...
*
Hereditary sclerosing poikiloderma
Hereditary sclerosing poikiloderma is an autosomal dominant conditions with skin changes consisting of generalized poikiloderma appearing in childhood.James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical ...
Causes
*Congenital
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Rothmund-Thompson syndrome
#
Dyskeratosis congenita
Dyskeratosis congenita (DKC), also known as Zinsser-Engman-Cole syndrome, is a rare progressive congenital disorder with a highly variable phenotype. The entity was classically defined by the triad of abnormal skin pigmentation, nail dystrophy, and ...
#
Mendes da Costa syndrome
*Other hereditary causes
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Degos-Touraine syndrome
Poikiloderma is a skin condition that consists of areas of hypopigmentation, hyperpigmentation, telangiectasias and atrophy.
Poikiloderma of Civatte is most frequently seen on the chest or the neck, characterized by red colored pigment on the skin ...
#Diffuse and macular atrophic
dermatosis
#
Hereditary sclerosing poikiloderma
Hereditary sclerosing poikiloderma is an autosomal dominant conditions with skin changes consisting of generalized poikiloderma appearing in childhood.James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical ...
of weary
#
Kindler syndrome
Kindler syndrome (also known as "bullous acrokeratotic poikiloderma of Kindler and Weary",) is a rare congenital disease of the skin caused by a mutation in the KIND1 gene.
Symptoms and signs
Infants and young children with Kindler syndrome hav ...
#
Xeroderma pigmentosum
Xeroderma pigmentosum (XP) is a genetic disorder in which there is a decreased ability to repair DNA damage such as that caused by ultraviolet (UV) light. Symptoms may include a severe sunburn after only a few minutes in the sun, freckling in sun- ...
*Acquired
#Injury to cold, heat, ionizing radiation, exposure to sensitizing chemicals
#
Lichen planus
Lichen planus (LP) is a chronic inflammatory and immune-mediated disease that affects the skin, nails, hair, and mucous membranes. It is not an actual lichen, and is only named that because it looks like one. It is characterized by polygonal, fla ...
#
Dermatomyositis
Dermatomyositis (DM) is a long-term inflammatory disorder which affects skin and the muscles. Its symptoms are generally a skin rash and worsening muscle weakness over time. These may occur suddenly or develop over months. Other symptoms may inc ...
#
Lupus erythematosus
Lupus erythematosus is a collection of autoimmune diseases in which the human immune system becomes hyperactive and attacks healthy tissues. Symptoms of these diseases can affect many different body systems, including joints, skin, kidneys, blo ...
#
Systemic sclerosis
Systemic scleroderma, or systemic sclerosis, is an autoimmune rheumatic disease characterised by excessive production and accumulation of collagen, called fibrosis, in the skin and internal organs and by injuries to small arteries. There are two m ...
#
Cutaneous T cell lymphoma
Cutaneous T-cell lymphoma (CTCL) is a class of non-Hodgkin lymphoma, which is a type of cancer of the immune system. Unlike most non-Hodgkin lymphomas (which are generally B-cell-related), CTCL is caused by a mutation of T cells. The cancerous T ...
s
Pathogenesis
The exact cause of poikiloderma of Civatte is unknown; however, extended sun exposure, namely the
ultraviolet light
Ultraviolet (UV) is a form of electromagnetic radiation with wavelength from 10 nm (with a corresponding frequency around 30 PHz) to 400 nm (750 THz), shorter than that of visible light, but longer than X-rays. UV radiation i ...
emitted by the sun, is the primary factor.
Diagnosis
Treatment
Albeit difficult, treatment of poikiloderma of Civatte involves the delivery of multiple wavelengths of intense pulsed light (IPL) to the affected area.
[PubMed.go]
"Treatment of poikiloderma of Civatte with an intense pulsed light source"
''PubMed.gov'', referenced July 22, 2011.
See also
*
Osteopoikilosis
Osteopoikilosis is a benign, autosomal dominant sclerosing dysplasia of bone characterized by the presence of numerous bone islands in the skeleton.
Presentation
The radiographic appearance of osteopoikilosis on an X-ray is characterized by a patt ...
*
List of cutaneous conditions
Many skin conditions affect the human integumentary system—the organ system covering the entire surface of the body and composed of skin, hair, nails, and related muscle and glands. The major function of this system is as a barrier against t ...
References
Disturbances of human pigmentation
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