Ehlers-Danlos syndrome, type II (mitis)

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The type II (mitis) variant of Ehlers-Danlos syndrome (EDS) is an autosomal dominant disorder characterized by mild skin hyperextensibility, mild joint hypermobility, easy bruisability, and poor wound healing with formation of wide atrophic fish-mouthed scars following minor trauma. The course of the disease is benign and affected patients have a normal life expectancy. We report a family with type II EDS and include a review of this variant and an updated classification of EDS.

Case report

A 12-year-old girl presented to the Department of Dermatology at The Cleveland Clinic Foundation in January 1982, with a history of delayed wound healing, easy bruisability, and double-jointedness. She had frequent episodes of gingival bleeding after routine dental flossing, but denied any other unusual bleeding tendency. She was born three weeks prematurely, the product of an otherwise normal pregnancy and delivery. The remainder of her personal and familial history was unremarkable except for the known occurrence of double-jointedness in several members of the maternal side of the family.

Physical examination revealed several gaping, atrophic, “fish-mouthed” scars on the extensor surfaces of the upper and lower extremities (Fig.1); hypermobility of several joints, particularly those of the hands and vertebral column (Fig. 2); mild hyperextensibility of the skin; marked stria formation on the breasts; and a few crusted papules on the nose and chin. Ecchymoses were absent and the tongue was not hyperextensible (negative Gorlin’s sign). Examination of the patient’s mother revealed mild skin stretchability and moderate joint laxity. The patient’s 10-year-old brother had several scars . . .