Background

This is a histologic pattern and a distinctive clinical pattern. Clinically, there is erythema with mottled pigmentation followed by epidermal atrophy. Histologically, there is a lichenoid dermatitis with basal vacuolar change, melanin incontinence, telagiectasia, and epidermal atrophy. Several clinical variants are recognized.

Poikiloderma Variants

Genodermatoses	Rothmund-Thomson syndrome (Poikiloderma congenitale) Congenital telangiectatic erythema (Bloom's syndrome) Dyskeratosis congenita
Stage in early mycosis fungoides	Poikiloderma atrophicans vasculare
Variant of autoimmune diseases	Systemic lupus erythematosus Dermatomyositis
Stage in chronic graft-versus-host disease	Graft-versus-host disease
Miscellaneous	Following radiation, cold, heat, drugs (arsenicals and busulfan)

Rothmund-Thomson syndrome is an autosomal recessive condition with poikilodermatous skin changes occuring on the extremities. There are associated abnormalities on the eyes and skeletal system with short stature, hypogonadism, and mental retardation. Two variants include Hereditary sclerosing poikiloderma which is autosomal dominant with linear hyperkeratotic and sclerotic bands in the flexural areas and Kindler's syndrome which has an associated dystrophic epidermolysis bullosa.

Congenital telangiectatic erythema is an autosomal recessive disorder with a photodistributed facial rash, stunted growth, and repeated respiratory and gastrointestinal infections. There is an increased risk toward developing malignancies.

Dyskeratosis congenita has a triad of reticulate hyperpigmentation, nail dystrophy, and leukokeratosis of the oral mucous membranes. It is usually X-linked recessive although autosomal dominant inheritance has been documented.

Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.

Last Updated 6/22/2001

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