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Author: Dr. Karen Koch, Consultant Dermatologist, Wits University, Donald Gordon Medical Centre, Johannesburg, South Africa. DermNet New Zealand Editor-in-Chief: Hon A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy edited by Gus Mitchell. November 2017.
Atrophoderma of Pasini and Pierini is a rare dermal atrophy disorder characterised by well-circumscribed slightly depressed areas, usually on the patient's trunk. The border between normal skin and the area of atrophy has a well-defined, ‘cliff-drop’ edge.
Atrophoderma of Pasini and Pierini most commonly starts during adolescence or early adulthood, although paediatric and congenital onset have been reported . It occurs more frequently in women than men .
*First two images are reproduced with permission from John Wiley and Sons. Liu S Oliver F, Agnew K (2018), An unusual infantile rash. Clin Exp Dermatol, 43: 835-838. doi:10.1111/ced.13541.
An immunological mechanism triggered by Borrelia burgdorferi, the cause of Lyme disease, has been proposed as the trigger 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 immunoglobulin (Ig) G levels in the affected individuals .
Improvement in atrophoderma of Pasini and Pierini has occurred during treatment with doxycycline in a patient with IgM antibodies to B. burgdorferi .
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 as having 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 ofthe depression is 1–8 mm in size. This may best be observed with lighting from an angle.
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.
The diagnosis of atrophoderma of Pasini and Pierini is usually made on the taking of a patient history and a clinical examination. Skin biopsy is most often non-contributory.
A wedge biopsy of involved and uninvolved skin may show:
Elastic stains may show a decrease in elastic fibres. IgG and IgM serology for B. burgdorferi should be assessed in endemic areas.
Several skin conditions should be considered in the differential diagnosis of atrophoderma of Pasini and Pierini. Conditions to consider include:
There is no completely effective treatment for atrophoderma of Pasini and Pierini. Cosmetic camouflage may be considered.
Specific treatments include:
The long-term outcome of atrophoderma of Pasini and Pierini is usually good. Spontaneous improvement may occur over years or decades.
Atrophoderma of Pasini and Pierini – Medscape
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