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Hard skin syndrome Parana type

Overview

A rare skin disorder characterized by rigid, thick skin that covers the whole body and affects movement. Tightness of skin over the chest and abdomen can impair breathing and death can result.

Symptoms - Hard skin syndrome Parana type

* Stiff skin * Severe growth retardation * Respiratory insufficiency

Causes - Hard skin syndrome Parana type

* 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

Prevention - Hard skin syndrome Parana type

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Diagnosis - Hard skin syndrome Parana type

In cases of diffuse thickening of the skin, a thyroid profile with T 3 , T 4 , and TSH should be done. This should also identify hypothyroidism. A positive ANA test with a speckled pattern will help identify scleroderma, but a skin biopsy should also be done. An antisclerodermal antibody titer is also useful if available. Esophageal motility studies will be helpful in early diagnosis. A skin biopsy will help identify many of the other conditions mentioned above. Urine for porphyrins will help identify porphyria.

Prognosis - Hard skin syndrome Parana type

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Treatment - Hard skin syndrome Parana type

* 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

Resources - Hard skin syndrome Parana type

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