(BMJ)—A previously healthy 4-year-old girl presented with recurrent episodes of itchy, red, round plaques that spread from the face to the extremities and trunk. The lesions resolved spontaneously, leaving behind loose, wrinkled skin. Exam: wrinkles on face; red, well-circumscribed atrophic plaques on trunk and limbs. Bx confirmed the dx. What is it?
Progressive lipodystrophy
Cutis laxa
Pseudoxanthoma elasticum
Idiopathic atrophoderma of Pasini and Pierini
Wrinkly skin syndrome
You are correct. Bx results showed decreased number and fragmentation of elastic fibers, consistent with the clinical picture of postinflammatory cutis laxa. It may be inherited or acquired, with both forms being generalized or localized. The acquired form is usually preceded by inflammatory lesions, as in this patient. Cutis laxa can present as a paraneoplastic phenomenon (e.g., lymphoma and multiple myeloma) or follow episodes of urticaria, angioedema, inflammatory dermatoses, infection, or connective tissue disease.

Archives of Disease in Childhood 2021;106:618
By hgreaney@athen…, 11 June, 2021

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