Zambuschs disease or Von Zambuschs disease is also known as Csillag's Disease, Guttate Morphea, Guttate Scleroderma, Hallopeau I Disease, Lichen Sclerosus and White Spot Disease. It is a very uncommon condition which leads to patchy, white skin that's thinner than normal. It is a chronic inflammatory dermatosis that results in white plaques with epidermal atrophy. It mostly affects the genital like skin of the vulva, foreskin of the penis and anal areas. Sometimes, lichen sclerosus appears on the upper body, breasts, and upper arms.

Postmenopausal women and children who have not reached puberty are at highest risk. Male genital lichen sclerosus is seen almost exclusively in uncircumcised men and boys. Lichen sclerosus has no associated increased mortality unless the patient develops a malignancy in the area. Cancer arising in extra genital presentations is described only rarely and may be coincident with other factors. Many pediatric cases will improve with puberty. Recalcitrant cases, those associated with erosion or progressive scarring, may result in severe sexual dysfunction. Disease has a sexual preponderance. Females are six times more affected with the disease as compared to the males. Extragenital lichen sclerosus is rare in children. Disease may not need treatment because sometimes lichen sclerosus improves on its own.

Signs and Symptoms

• Painful and difficult intercourse
• Severe Itching at the affected area
• White Spots at the affected area
• White Spots may deteriorate into severe wrinkles
• Tenderness at the affected area
• Bleeding, blistering and Ulceration of the affected area
• The affected skin has tendency for easy bruising and tearing

Causes

The exact cause is unknown but some hormonal disturbances especially the sex hormone deficiency and the autoimmune pahtophysiology is also described for the disease. It is believed that Inflammation and altered fibroblast function in the papillary dermis leads to fibrosis of the upper dermis.

Diagnosis and Investigations

• Physical findings
• Biopsy of the affected tissue for microscopic examination

Treatment and Medications

• Corticosteroid Creams and Ointments
• Immune system modulators like tacrolimus and pimecrolimus
• Ultraviolet light treatment is recommended in some cases in which the involvement of the disease is non genital
• Few patients are often prescribed sex hormones if the etiology is proved