Atrophoderma of Pierini and Pasini

Synonyms

2

Overview

Atrophoderma of Pierini and Pasini is thought to possibly represent a late stage of morphea a type of localized scleroderma. It is a progressive skin disease characterized by violet or brown skin lesions of varying sizes. The disease results in round or oval patches of hyper-pigmented skin. The darkened skin patches may sometimes have a bluish or purplish hue when they first appear and are often smooth to the touch and hairless. It is characterized by large lesions with a sharp peripheral border dropping into a depression with no outpouching, which, on biopsy, elastin is normal, while collagen may be thickened.

Symptoms

• Multiple oval, darkened (hyperpigmented) plaques in which tissue under the skin breaks down so that there is a depression (dent) within the skin
• Violet skin lesions
• Brown skin lesions
• Small lesions
• Large lesions

Causes

There is not known cause of this disease but it is suggested that there is a higher frequency of family history of autoimmune diseases in patients with this disease.

Some findings suggest that atrophoderma of Pierini and Pasini may be associated with B burgdorferi,a bacteria that causes Lyme disease, in some cases.

Treatment

No single treatment of atrophoderma of Pierini and Pasini is consistently effective. Therapies that have been tried include topical corticosteroids, antibiotics, and antimalarials. There have been reports of symptom improvement with the use of hydroxychloroquine, potassium aminobenzoate, and improvement following surgical care using a Q-switched alexandrite laser, however these findings have not been confirmed by larger studies. If a person with atrophoderma of Pierini and Pasini tests positive for B burgdorferi antibody, standard Lyme disease therapy is often recommended.

Resources

• NIH