Paget disease extramammary
Overview
A rare cancer characterized by a chronic rash that resembles eczema and usually occurs on the genital and anal areas.
Symptoms
* Burning armpits * Sore armpits * Itchy armpits * Scratching lesions * Eczema-like genital rash * Eczema-like anal rash * Eczema-like armpit rash * Eczema-like genital rash
Causes
The disease occurs worldwide, but is more common in Europe, Australia, and New Zealand, where it’s seen in up to 5% of the elderly population. Although its exact cause is unknown, one theory holds that early viral infection causes a dormant skeletal infection that erupts many years later as Paget’s disease. Genetic factors are also suspected
Diagnosis
he clinical differential diagnosis includes squamous cell carcinoma in situ and superficial fungal infection. It is generally thought to be an adenocarcinoma of the epidermis, from which it extends into the contiguous epithelium of hair follicles and eccrine sweat ducts.
Treatment
Primary treatment consists of drug therapy and includes one of the following: * Calcitonin (subcutaneously or intranasally) is used to retard bone resorption (which relieves bone lesions) and reduce levels of serum alkaline phosphate and urinary hydroxyproline secretion. Although calcitonin therapy requires long-term maintenance, improvement is noticeable after the first few weeks of treatment. * Bisphosphonates, such as etidronate, alendronate, pamidronate, tiludronate, and risedronate, produce rapid reduction in bone turnover and relieve pain. They also reduce serum alkaline phosphate and urinary hydroxyproline secretion. Therapy produces noticeable improvement after 1 to 3 months. * Plicamycin, a cytotoxic antibiotic, is used to decrease calcium, urinary hydroxyproline, and serum alkaline phosphatase. It produces remission of symptoms within 2 weeks and biochemical improvement in 1 to 2 months. Plicamycin is used to control the disease and is reserved for severe cases with neurologic compromise and for those resistant to other therapies. However, it may destroy platelets or compromise renal function. Orthopedic surgery is used to correct specific deformities in severe cases, reduce or prevent pathologic fractures, correct secondary deformities, or relieve neurologic impairment. Joint replacement is difficult because bonding material (methyl methacrylate) doesn’t set properly on pagetic bone. Other treatment varies according to symptoms. Analgesics or nonsteroidal anti-inflammatory drugs may be given to control pain.