Localized Pseudoxanthoma Elasticum-like Changes on the Chin: DISCUSSION part 2
Until now, more than 20 reports involving PXE have been published in Korea, most of which present cases with typical clinical symptoms, asso¬ciated systemic abnormalities, or perforating PXE. Unlike previous reports, our patient complained of severe pruritus on a normal appearing chin without other systemic problems or a family history. Recently, Lebwohl et al reported a previously unde- scribed cutaneous finding in a patient with PXE. As a marker of PXE, mental creases are sensitive and highly specific under the age of 30 years, affecting two-thirds of patients, but not in age-matched control patients. However, there was no further description of clinical findings related to PXE. Our patient also had horizontal and oblique mental creases; however, the creases may not be specific because the development of a horizontal mental crease is not rare in individuals with advanced age. Taylor et al reported tumefactive lipedema with PXE-like microscopic changes. The patient could have either a subclinical form of PXE, perhaps predisposing to lipedema, or secondary elastic tissue changes resulting from the massive edema. They explained if the latter was the case, this could represent an unusual manifestation of localized acquired cutaneous PXE. Similarly, there is a possibility that PXE-like changes in our case might be secondary changes resulting from the scratching.
In summary, we present a rare case of patient who only had an itching sensation localized to the chin without typical skin surface changes of PXE or a family history, showing PXE-like histologic changes. Patients with PXE typically have a normal life span, but morbidity and mortality depend on the extent of systemic involvement. It is important to recognize the disease early in order to minimize the risk of systemic complications. Patients with PXE should receive ophthalmology and cardiology assess¬ments on a regular basis. Since there may be an unusual case of PXE-like conditions with only localized itching sensation, we recommend a skin biopsy in similar cases. kamagra soft tablets