Atrophoderma of Pasini and Pierini

Atrophoderma of Pasini and Pierini is a rare disorder of dermal atrophy described by Pasini in 1923 and Pierini & Vivoli in 1936. The typical presentation is of single or multiple sharply demarcated, mildly depressed, hyperpigmented lesions on the trunk. It is believed to be a distinct entity from morphoea.

This chapter is set out as follows:

• The cause is unknown 

• It is more common in Caucasian's, and has a female to male ratio of 6:1
• Lesions can arise at any age, but most commonly in the second and third decades 

Distribution

• ​Most commonly, lesions are distributed symmetrically on the trunk, particularly on the back, and to a lesser extent on the extremities, with a few displaying a unilateral and zosteriform pattern

Morphology

• Single or multiple sharply demarcated, round to oval, hyperpigmented, mildly depressed patches ranging in size from a few mm to several cm. Hypopigmented lesions have been reported 
• The distinct edge is described as having a “cliff-drop border'' 
• The lesions may be discrete or confluent, increase in size very slowly, and eventually lighten before remaining stable for 10-20 years 
• The skin surface feels normal, unlike in morphoea where the skin is firm 

• Histology
  • ​Histopathological changes are often minimal and non-diagnostic
  • The epidermis - is usually normal or slightly atrophic, and there may be a decrease in the size of the dermal papillae with flattening of the rete pegs. Melanin may be increased in the basal layer
  • The dermis - the dermal thickness is reduced and collagen bundles show varying degrees of homogenisation and clumping in the mid and reticular dermis. There may be a mild perivascular infiltrate consisting of lymphocytes and histiocytes 

• Although often persistent, the condition is self-limiting
• No treatment has been shown to be reliably effective