Cutis laxa‐like calcinosis cutis secondary to asfotase alfa in juvenile‐onset hypophosphatasia

Abstract

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Cutaneous lesions in the setting of hypophosphatasia

“… 8 , 9 Importantly, there are 2 documented cases of calcinosis cutis after AA therapy. Charalambides et al 10 reported a case of severe injection site reaction to subcutaneous injections of AA in a patient with juvenile-onset HPP receiving 60 mg AA for 6 days per week for 6 months alternating in the abdomen and thigh. 10 Skin biopsies at these injection sites showed calcium deposition in the dermis, subcutis, vessel walls, and sweat ducts with a multinucleate giant cell response.…”

“…Charalambides et al 10 reported a case of severe injection site reaction to subcutaneous injections of AA in a patient with juvenile-onset HPP receiving 60 mg AA for 6 days per week for 6 months alternating in the abdomen and thigh. 10 Skin biopsies at these injection sites showed calcium deposition in the dermis, subcutis, vessel walls, and sweat ducts with a multinucleate giant cell response. The pathophysiology of this reaction is due to degradation of PPi by synthetic ALP found in AA; degraded PPi produces higher levels of inorganic phosphate, which promote calcification.…”

“…The pathophysiology of this reaction is due to degradation of PPi by synthetic ALP found in AA; degraded PPi produces higher levels of inorganic phosphate, which promote calcification. 10 …”