Clinical Guidance

Kindler syndrome (KS), a rare subtype of epidermolysis bullosa, is characterized by skin fragility and acral blister formation beginning at birth or in early infancy, diffuse cutaneous atrophy, photosensitivity (most prominent during childhood and usually decreasing after adolescence), poikiloderma, palmoplantar hyperkeratosis, and pseudosyndactyly. Mucosal manifestations are also common and include hemorrhagic mucositis and gingivitis, periodontal disease, premature loss of teeth, and labial leukokeratosis. Other mucosal findings include ectropion, urethral stenosis, and severe phimosis. Severe long-term complications of KS include periodontitis, mucosal strictures, and aggressive squamous cell carcinomas.

Diagnostic Criteria

No consensus clinical diagnostic criteria for Kindler syndrome (KS) have been published.

Suggestive Findings

KS should be suspected in individuals with the following clinical findings, skin biopsy results, and family history.

Clinical findings

Source: GeneReviews — "Kindler Syndrome"

Management Guidelines

No clinical practice guidelines for Kindler syndrome (KS) have been published. Evaluation Following Initial Diagnosis To establish the extent of disease and needs in an individual diagnosed with KS, the evaluations summarized (if not performed as part of the evaluation that led to the diagnosis) are recommended. Table 4. Recommended Evaluations Following Initial Diagnosis in Individuals with Kindler Syndrome

Surveillance Guidelines

Table 6.

Recommended Surveillance for Individuals with Kindler Syndrome

System/Concern | Evaluation | Frequency

Risk for skin cancer

| Screen for premalignant keratoses early squamous cell carcinomas. | Starting in adolescence; annually thereafter

Risk for oral mucosa tumors