Deterrence/Prevention
Avoiding the sun and protecting the skin from light exposure may prevent or slow the progression of poikiloderma. Good wound care includes the use of topical and systemic antibiotics for infected bullous lesions, which may reduce morbidity.
Complications
Secondary infections from congenital blisters and bullae may occur. The blisters usually heal without residual scarring, but they can result in hypopigmentation and transient milia. Mucosal involvement can lead to urethral, anal, and esophageal stenosis. Accelerated periodontal disease is common. Ophthalmic complications, such as conjunctival scarring, may occur.
Prognosis
Patients usually have normal intelligence and a normal life span. Blistering improves with age, but poikiloderma and atrophy are progressive. Morbidity and mortality are mostly related to secondary infections arising from cutaneous bullae and to cosmetic disfigurement.
Patient Education
Patients should be advised to avoid trauma, which helps prevent blister formation. The use of broad-spectrum photoprotection should be emphasized.
For patient education resources, see the Skin, Hair, and Nails Center.
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Images show the progression of lesions. A and B: At birth, acral blisters and erosions are present. C and D: At age 5 years, atrophy and reticulated erythema with dyschromic patches are noted. E and F: At age 7 years, progressive poikilodermatous changes with reticulated erythema and telangiectasia occur. G and H: At age 10 and 15 years, poikiloderma with telangiectasia and depigmentation are observed. Excoriations are due to pruritus. Reprinted from Yasukawa K, Sato-Matsumura KC, McMillan J, et al: Exclusion of COL7A1 mutation in Kindler syndrome. J Am Acad Dermatol 2002 Mar; 46(3): 447-50. Courtesy of the American Academy of Dermatology.