Atrophoderma of Pasini and Pierini

What is atrophoderma of Pasini and Pierini?

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.

Who gets atrophoderma of Pasini and Pierini?

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

Baby aged 10 months

Baby aged 20 months

Visible subcutaneous vessels

*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.

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 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 [2].

Improvement in atrophoderma of Pasini and Pierini has occurred during treatment with doxycycline in a patient with IgM 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.  

• Lesions can be solitary or multiple.
• They tend to be oval or round.
• Hyperpigmented, hypopigmented and normal skin-coloured areas may be present.
• The texture is normal (eg, there is no induration).
• Lesions are 1–12 cm in size.
• The most common site is the back or lumbosacral region, followed by the chest, arms and abdomen, sparing the face, hands and feet [4, 5].

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.

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 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:

• Flattening of the dermal papillae and rete ridges
• Sparse perivascular and/or perifollicular lymphocytic infiltrate
• Clumping of collagen fibres. 

Elastic stains may show a decrease in elastic fibres. 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. Conditions to consider include:

• Anetoderma — characterised by multiple round well-defined 1–2 cm depressed lesions with overlying wrinkly skin (the so-called ‘button-hole’ sign)
• Lichen sclerosus — characterised by white, polygonal papules that coalesce into porcelain-white plaques; depressed ovoid lesions are absent
• Late-stage morphoea — characterised by shiny hypopigmented or hyperpigmented plaques with poorly demarcated depressed areas; there is a preceding history of inflammation and sclerosis
• Postinflammatory hyperpigmentation — this follows a wide range of inflammatory skin disorders; the skin is of normal thickness and does not demonstrate any atrophy.

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:

• Topical corticosteroids
• Topical calcineurin inhibitors
• Oral antibiotics, such as penicillin or tetracycline 
• Hydroxychloroquine 
• Q-switched alexandrite laser [6].

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.