Clinicopathologic Conference Clinicopathologic Conference|Articles in Press


Published:March 17, 2023DOI:
      A 26-year-old white female was referred to the Oral Medicine Clinic of the University for evaluation of symptomatic oral submucosal nodules. Past medical history taken from the patient's mother revealed an uneventful pregnancy and delivery, and normal crying at birth. However, hoarseness was noted when the patient started saying her first words. At the age of 13 the patient started to experience severe headaches that were centered in the orbital region and were diagnosed as migraines. The headaches did not improve with the proposed treatment at the time (Topiramate 50 mg), so the medication was discontinued at age 16. Fainting and vomiting during the headache crises and frequent general malaise were mentioned. Moreover, endometriosis was diagnosed at age 20 and has been treated with dienogest 2 mg daily. Depression and occurrence of two epileptic seizures were also reported during adulthood. No consanguinity between parents was reported, and family history was negative for similar clinical manifestations. The patient showed generally dry skin, as well as several scars on her upper limbs, and scaling on hands and feet (Figure 1A and B). The skin lesions progressively worsened over the last five years. The extraoral examination showed moniliform blepharosis characterized by multiple yellowish circular intact small papules covered by a smooth surface along the edges of her eyelids (Figure 1C and D); this clinical finding was noticed to had been present ever since birth.
      Figure 1
      Figure 1Extraoral manifestations of Lipoid Proteinosis. (A) and (B) Extensive scaling on hands and feet. (C) and (D) Multiple papules covered by a smooth surface along the edges of the eyelids and characterizing moniliform blepharosis.
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'