Kindler Syndrome Follow-up
- Author: Anatoli Freiman, MD, FRCPC, DABD; Chief Editor: Dirk M Elston, MD more...
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.
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.
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.
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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