Atrophoderma of Pasini and Pierini Workup
- Author: Sarah Jane Adams, MD; Chief Editor: Dirk M Elston, MD more...
The laboratory, imaging and histological findings of idiopathic atrophoderma of Pasini and Pierini are non-specific and the diagnosis is typically made on the basis of clinical features.
Routine baseline studies of the blood and urine may help to exclude other conditions, but they do not help in the diagnosis of idiopathic atrophoderma of Pasini and Pierini.
Screening tests such as the enzyme-linked immunosorbent assay may be performed to detect anti– B burgdorferi antibodies.
One anecdotal case report from 2012 advocates thyroid gland evaluation in all patients with sclerodermalike disorders because of pathophysiological links between cutaneous fibrosis and the thyroid gland.
The thickness of the dermis and subcutis may be measured using magnetic resonance imaging or 13-MHz B-mode ultrasonography.
Skin biopsy is not always necessary to make the diagnosis; however, it may be useful to exclude other entities.
Dermal atrophy is more easily evaluated with wedge excision than punch biopsy. An elliptical biopsy specimen is sectioned longitudinally from an area that includes normal skin and the cliff-drop border of the lesion. If dermal atrophy is present, the transition between normal dermis to atrophied dermis is discernible.
Histopathologic changes, often minimal and nondiagnostic, consist of a decrease in the size of the dermal papillae with flattening of the rete pegs. The epidermis is usually normal or slightly atrophic. Melanin may be increased in the basal layer. Interstitial edema and a mild perivascular infiltrate consisting of lymphocytes and histiocytes may be present. Collagen bundles show varying degrees of homogenization and clumping in the mid and reticular dermis. When compared with adjacent normal skin, dermal thickness is reduced. The sweat glands, pilosebaceous units, and appendages are not affected.
In most series, no abnormalities in elastic fibers have been observed with either elastic tissue staining or electron microscopy. However, a 2008 study described a spectrum of histopathological findings ranging from normal to severe diminution and fragmentation of elastic fibers, with 35.3% of cases showing moderate-to-severe reduction and fragmentation.
If preexisting patches show sclerodermatous changes, histology may reveal varying degrees of collagen sclerosis resembling morphea. Direct immunofluorescence of early lesions may show nonspecific immunoglobulin M and C3 staining in the dermal papillary blood vessels or at the dermoepidermal junction. CD34 dermal dendrocytes are reduced, just as in morphea.
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