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School of Medicine, Federal University of Alfenas, Alfenas, MG, BrazilSchool of Medicine, Universidade Professor Edson Antônio Velano - UNIFENAS, Alfenas, MG, Brazil
Corresponding author: Marina Lara de Carli, DDS, PhD – Phone number: 55 35 99122-6277, School of Dentistry, Federal University of Alfenas, Rua Gabriel Monteiro da Silva, 700 – Centro – Alfenas – Minas Gerais – Brazil, Zip Code 37130 – 001
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 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.