Keratosis linearis-ichthyosis congenita-sclerosing keratoderma syndrome

Orpha code: 281201OMIM code: 601952

Definition

Keratosis linearis-ichthyosis congenita-sclerosing keratoderma syndrome is an inherited epidermal disorder characterized by palmoplantar keratoderma, linear hyperkeratotic papules on the flexural side of large joints (cord-like distribution around wrists, in antecubital and popliteal folds), hyperkeratotic plaques (on neck, axillae, elbows, wrists, and knees), mild ichthyosiform scaling, and sclerotic constrictions around fingers that present flexural deformities.

Disease data
Classification

Disease

Synonyms
KLICK syndrome
Zespół KLICK
ORPHA code
281201
OMIM code
601952
ICD10 code
Q82.8
ICD11 code
-

No additional description.

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