ABSTRACT
A 58-year-old, gravida 6, obese woman presented with a pruritic yellowish plaque around the umbilicus, which first appeared about 3 years ago. She also had flat yellow papules on the axilla and neck. After a burn from a heating device, a few perforating papules and a violaceous hue occurred on the plaque. Histological examination revealed pathological elastic fibers with a keratotic plug and perforation in the deep dermis, which was consistent with perforating pseudoxanthoma elasticum. This case indicates that perforating pseudoxanthoma elasticum, a variant of hereditary pseudoxanthoma elasticum may perforate due to mechanical factors.
ABSTRACT
Pseudoxanthoma elasticum (PXE) is a rare, inherited, multisystem disorder characterized by progressive calcification and fragmentation of elastic fibers. Typically, small, yellowish papules are located on the neck and flexural areas; however, localized acquired cutaneous PXE has been proposed as a non-heritable form of the disorder. We report a patient who had normal appearing skin on the chin with an itching sensation and had PXE-like changes by skin biopsy.