DefinitionThis section has been translated automatically.
Poikiloderma (poikilos = mottled, marbled) refers to clinico-morphologically defined, etiologically distinct, congenital or acquired, variegated clinical pictures with circumscritic or diffuse atrophies, small-spot to reticular hyper- and depigmentation, telangiectasias, and erythema. Poikiloderma can occur in genetic, inflammatory, and neoplastic disorders.
The term "poikiloderma" also includes clinical pictures which can be counted among the so-called pre-aging syndromes.
ClassificationThis section has been translated automatically.
According to origin or association with syndromes one distinguishes:
Congenital (primary) poikiloderma (classification in a narrower sense, poikiloderma as a dermatological-clinical signature is indicative of an underlying polyorganic syndrome).
- Rothmund-Thomson syndrome (mutation in the RECQL4 gene) (Former distinction between Thomson syndrome and Rothmund syndrome /mutation in the RECQL4 gene)
- Baller-Gerold syndrome (mutation in the RECQL4 gene - see also Rapadilino syndrome
- Dyskeratosis congenita (Zinsser)
- Poikiloderma, congenital with warty hyperkeratosis (Dowling)
- Kindler syndrome (poikiloderma, congenital with blistering; Marghescu-Braun-Falco syndrome; Brain syndrome)
- Dysplasia, congenital ectodermal with cataract.
Poikiloderma in the context of other congenital syndromes:
- Progeria adultorum (Werner syndrome).
- Hartnup syndrome (metabolic disorder)
- Goltz-Gorlin syndrome (dysplasia syndrome)
- Bloom syndrome
- Amyloidosis, familial poikilodermic
- Incontinentia pigmenti, Bloch-Sulzberger type
- Clericuzio-type poikiloderma with neutropenia (mutation in C16orf57)
Symptomatic (secondary) poikiloderma (in the context of chronic inflammatory or neoplastic underlying diseases):
HistologyThis section has been translated automatically.
Due to the various causes, the histology of poikiloderma can be very variable. Common to all forms is an atrophic epidermis with striated rectal ridges, mild hyperkeratosis, pigment incontinence with melanophages in the papillary body and telangiectasia. Occasionally, vacuolization of the junctional zone with lymphocytes and dyskeratosis is detectable.
LiteratureThis section has been translated automatically.
- Vogl A et al (2005) Skin and alcohol. J Dtsch Dermatol Ges 3: 788-790
Incoming links (25)Amyloidosis cutaneous special forms; Baller-Gerold syndrome; Berlin syndrome; Dyskeratosis congenita; Dysplasia, congenital ectodermal with cataract; Familial cancer syndrome; Hartnup syndrome; Interface dermatitis; Lower leg pigmentation, hereditary atrophy associated with atrophy; Poikiloderma, acquired; ... Show all
Outgoing links (27)Acrodermatitis chronica atrophicans; Baller-Gerold syndrome; Bloom syndrome; Dermatomyositis (overview); Dyskeratosis congenita; Dysplasia, congenital ectodermal with cataract; Erythema; Focal dermal hypoplasia; Graft-versus-host disease chronic; Hartnup syndrome; ... Show all
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.