Stiff skin syndrome
Overview
Stiff skin syndrome (medical condition): A rare inherited disorder characterized mainly by very stiff, thick skin that can affect the ability to move joints
Symptoms
* Limited joint mobility * Patches of stony-hard skin mostly on buttocks and upper thighs * Lordotic stance * Thickened skin
Causes
* Dry skin is a very common problem –Low humidity and cold temperatures make winter xerosis and “winter itch” common complaints –Mild xerosis can cause impaired skin barrier function and allow irritants and allergens to more easily affect the skin –Most common on the legs, but often affecting the entire skin surface –Can present with severe pruritus without much evidence of a rash * Severe xerosis is common in the elderly, and can cause eczema craquelé –Patient's legs often have scale that resembles cracked porcelain –Secondary erythema and excoriations occur because of the persistent itch * Ichthyoses vulgaris –Very common cause of dry skin –A genetic defect in skin barrier function, leading to a higher risk of atopic dermatitis –Patients often have hyperlinearity of their palmar skin and xerotic fish scale on their legs * Many genetic conditions, such as the large family of ichthyoses (including X-linked ichthyoses, Netherton's disease), lead to severely dry skin in association with other systemic manifestations * Hypothyroidism and hyperthyroidism can also cause marked xerosis and/or itch * Anemia * There is an uncommon association between lymphoma and marked xerosis * HIV * Sarcoidosis * Liver and biliary disease, and renal insufficiency, are commonly associated with xerosis and marked pruritus * Diabetes mellitus * Medications (e.g., niacinamide) * Atopic dermatitis
Prognosis
Prognosis of Stiff skin syndrome: stable condition
Treatment
* Emollients and humectants should be incorporated into the patient's daily routine; Avoid harsh antibacterial soaps, and avoid long, hot baths or showers; Apply rich creams (e.g. Keri lotionR, EucerinR) that are fragrance-free and hypoallergenic immediately after bathing and twice daily * Hydroxyzine and even phototherapy can be helpful to these patients. Bile acid-sequestering medications can help liver patients with xerosis and pruritus * Topical steroid ointments are sometimes necessary to control the pruritus until the skin barrier function is restored * Compliance is a problem in xerosis patients who don’t want to put greasy or heavy creams on their skin * Systemic retinoids are sometimes used as adjuvant therapy for patients with certain genetic ichthyoses * For the rare patient that has an associated malignancy, the xerosis should improve once the malignancy is eradicated