A Case of Periumbilical Perforating Pseudoxanthoma Elasticum: DISCUSSION

PPPXE, also referred as perforating calcific elasto- sis, is an acquired disorder of connective tissue, characterized by localized degeneration and calcifi­cation of elastic fibers. It occurs predominantly in middle-aged, multiparous, obese, black women. Patients typically present as an occasionally pruritic, slowly enlarging, hyperpigmented plaque with dis­crete keratotic papules at the periphery of the lesion. The skin lesion usually locates in the periumbilical region, especially in the supraumbilical area. It is difficult to explain the periumbilical localization of PPPXE, however, it is suggested that the repeated trauma of pregnancy, obesity, abdominal surgery, or massive ascites may act as the initiating factors and result in localized elastic fiber degeneration.

Clinically, hereditary pseudoxanthoma elasticum (PXE) should be differentiated. Whereas PXE is an inherited disorder of elastic tissue in the ocular, cutaneous and cardiovascular systems as a systemic process, PPPXE is a non-inherited, localized cutane­ous disease, which can be distinguished by lack of family history, late onset and absence of other systemic manifestations. Both PXE and PPPXE are characterized histologically by degeneration of elastic fibers with deposition of calcium in the mid-dermis. However, transepidermal elimination of these altered elastic fibers is only found in PPPXE and it rarely occurs in PXE.

An important histopathological entity in the differential diagnosis is elastosis perforans serpi- ginosa, which consists of the transepidermal elimin­ation of abnormally large, eosinophilic noncalcified elastic fibers in the upper dermis. In contrast, PPPXE is characterized by the transepidermal elimination of short, fragmented, basophilic calcified elastic fibers located primarily in the mid-dermis. With the von Kossa stain, calcification of elastic tissue in PPPXE is easily demonstrable.
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While there are no effective therapeutic options presently available, patients can be reassured that the eruption is a benign process limited to the skin. Up to now, 12 cases of PPPXE have been reported in English literature and 4 cases have been found in Korea. In summary, a case of PPPXE with no signs of hereditary pseudoxanthoma elasticum is presented.


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