Judge Misch Wright syndrome
Overview
A very rare syndrome characterized mainly by thickened skin on palms and soles as well as around the mouth.
Symptoms
* Abnormal fingernails * Sparse eyebrows * Absent eyebrows * Thickened skin on palms * Thickened skin on soles * Light sensitivity * Sparse scalp hair * Absent scalp hair * Impaired vision * Abnormal corneas * Dry 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
Diagnosis
A careful eye examination including tonometry and slit lamp examination should be done. A referral to an ophthalmologist may be necessary to accomplish this. If there is nuchal rigidity, a CT scan followed by a spinal tap should be done in conjunction with a neurologic consultation. If there is fever without nuchal rigidity, the workup can proceed as outlined on page 168 . A histamine test may be helpful in diagnosing migraine.
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