Poikiloderma of Kindler
Overview
Poikiloderma of Kindler: A rare disorder characterized by fragile skin which blisters easily even after a mild trauma as well as photosensitivity and striated skin pigmentation (diffuse poikiloderma striate.
Symptoms
Pigmentation abnormalities Blisters following mild trauma Vescico-pustules on hands Vescico-pustules on feet Eczematoid diffuse dermatitis
Causes
Kindler syndrome has been shown to result from mutations in the KIND1 gene on band 20p12.3 (see Pathophysiology). An autosomal recessive pattern of transmission has been reported, but sporadic cases are common, with many originating in consanguineous families. Variable expressivity within families has also been documented. Mutations in the gene encoding type VII collagen (COLA7A1) have been excluded, distinguishing Kindler syndrome from dystrophic epidermolysis bullosa.
Treatment
Medical Care Treatment is mainly symptomatic and preventative in nature. Patients should be advised to avoid trauma, which helps prevent blister formation. Sun avoidance and photoprotection 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. Surgical Care Telangiectasias can be treated with pulsed-dye laser therapy. Surgical correction of urethral, anal, or esophageal stenosis may be needed.