Atrophoderma of Pasini and Pierini

Author: Dr. Karen Koch, Consultant Dermatologist, WITS Donald Gordon Mediclinic, Johannesburg, South Africa. DermNet New Zealand Editor-in-Chief: Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy editor: Gus Mitchell, November 2017.

What is atrophoderma of Pasini and Pierini?

Atrophoderma of Pasini and Pierini is a rare disorder of dermal atrophy characterised by well-circumscribed slightly depressed areas, usually on the trunk. The border between normal skin and the area of atrophy has a well-defined, ‘cliff drop’ edge.

Who gets atrophoderma of Pasini and Pierini?

Atrophoderma of Pasini and Pierini most commonly starts during adolescence or early adulthood. Paediatric and congenital onset have been reported [1]. It occurs more frequently in women than men [2].

What causes atrophoderma of Pasini and Pierini?

The exact cause of atrophoderma of Pasini and Pierini is not known. It is unclear whether it is a distinct disease entity or a disorder on the spectrum of morphoea (localised scleroderma). 

An immunological mechanism triggered by Borrelia burgdorferi, the cause of Lyme disease, has been proposed in at least a subset of patients, based on histological findings of an abnormal T-cell immunophenotype in the dermis and significantly raised B. burgdorferi IgG levels in affected individuals [2].

Improvement in atrophoderma of Pasini and Pierini has occurred during treatment with doxycycline in a patient with immunoglobulin M antibodies to B. burgdorferi [3].

What are the clinical features of atrophoderma of Pasini and Pierini? 

Atrophoderma of Pasini and Pierini classically presents as asymptomatic depressed areas on the trunk and extremities.  

Atrophoderma of Pasini and Pierini may be stable or gradually progressive. Older lesions enlarge with time and may coalesce to form large irregularly pigmented areas, referred to as ‘footprints in the snow’ or a ‘moth-eaten’ appearance. New lesions may continue to develop for several decades.

Established lesions demonstrate a sharp demarcation between normal skin and atrophic areas known as a ‘cliff drop’ edge. The depth of depression ranges from 1–8 mm. This may best be observed with lighting from an angle.

What are the complications of atrophoderma of Pasini and Pierini?

Atrophoderma of Pasini and Pierini tends to be progressive with further disfigurement of the affected area. There have been no reports of progression to morphoea or other complications.

How is atrophoderma of Pasini and Pierini diagnosed?

The diagnosis of atrophoderma of Pasini and Pierini is usually made on history and clinical examination. Skin biopsy is most often non-contributory.

A wedge biopsy of involved and uninvolved skin may show:

IgG and IgM serology for B. burgdorferi should be assessed in endemic areas.

What is the differential diagnosis for atrophoderma of Pasini and Pierini?

Several skin conditions should be considered in the differential diagnosis of atrophoderma of Pasini and Pierini.

What is the treatment for atrophoderma of Pasini and Pierini?

There is no completely effective treatment for atrophoderma of Pasini and Pierini. Cosmetic camouflage may be considered.

Specific treatments include:

What is the outcome for atrophoderma of Pasini and Pierini?

The long-term outcome of atrophoderma of Pasini and Pierini is usually good. Spontaneous improvement may occur over years or decades.

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