Clinical Guidance

Classic Ehlers-Danlos syndrome (cEDS) is a heritable connective tissue disorder characterized by skin hyperextensibility, abnormal wound healing, and generalized joint hypermobility. Skin. Skin is hyperextensible; it extends easily and snaps back after release (unlike lax, redundant skin, as in cutis laxa). The skin is soft, velvety, or doughy to the touch. The skin is fragile, as manifested by splitting of the dermis following relatively minor trauma, especially over pressure points (knees, elbows) and areas prone to trauma (shins, forehead, chin). Skin fragility may cause dehiscence of sutured incisions in skin or mucosa. Wound healing is poor, and stretching, thinning, and pigmentation of scars is characteristic.

Diagnostic Criteria

There are no consensus clinical diagnostic criteria for classic Ehlers-Danlos syndrome (cEDS); diagnosis requires molecular testing. Suggestive Findings Classic EDS should be suspected in a proband with either of the following: • skin hyperextensibility and atrophic scarring OR • generalized joint hypermobility (GJH) and/or ≥3 Major criteria • Skin hyperextensibility is measured by pinching and lifting the cutaneous and subcutaneous layers of the skin on the volar surface at the middle of the nondominant forearm as described by . Skin is hyperextensible if it can be stretched over a standardized cutoff in three of the following areas: 1.5 cm for the distal part of the forearms and the dorsum of the hands; 3 cm for neck, elbows, and knees. • Atrophic scarring (See .)

Management Guidelines

For a detailed review of complications and management of classic Ehlers-Danlos syndrome (cEDS), see . Evaluations Following Initial Diagnosis To establish the extent of disease in an individual diagnosed with cEDS, the evaluations summarized (if not performed as part of the evaluation that led to the diagnosis) are recommended. Table 5. Classic Ehlers-Danlos Syndrome: Recommended Evaluations Following Initial Diagnosis

Surveillance Guidelines

To monitor existing manifestations, the individual's response to supportive care, and the emergence of new manifestations, the evaluations summarized in are recommended.

Table 7.

Classic Ehlers-Danlos Syndrome: Recommended Surveillance

System/Concern | Evaluation | Frequency

Skin | Assessment for skin fragility | At each visit or as needed