Is it always blepharitis? Urbach-Wiethe syndrome (lipoid proteinosis)

ABSTRACT A 12-year-old girl presented with recurrent crusty debris and dandruff at the base of both eyelashes despite having completed different medical treatments. She had had a hoarse voice since her early childhood. Upon anterior segment examination of the eyes, we found yellow-white, bead-like papules on the margins of the eyelids. An otolaryngologist detected multiple nodules on the vocal cords and buccal mucosa. Ultrasonography revealed salivary stones in the main parotid ducts. And a dermatological examination revealed thickened skin lesions on the elbows and knees with a biopsy showing histopathological findings of lipoid proteinosis. We diagnosed the patients as having Urbach-Wiethe syndrome or lipoid proteinosis, a rare autosomal recessive multisystem disorder with variable manifestations vary that difficult the diagnosis. The ocular manifestations are not well known among ophthalmologists, but the typical lid lesions are pathognomonic and ophthalmologists should be aware of this presentation to identify patients with Urbach-Wiethe syndrome.RESUMO Uma menina de 12 anos apresentava restos crostosos e caspa recorrente na base de ambos os c ílios, apesar de ter completado diferentes tratamentos médicos. Ela tinha uma voz rouca desde a infância. No exame do segmento anterior dos olhos, encontramos pápulas amarelo-esbranquiçadas nas margens das pálpebras. Um otorrinolaringologista detectou múltiplos nódulos nas corda...
Source: Arquivos Brasileiros de Oftalmologia - Category: Opthalmology Source Type: research

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Conclusions: To date, this is the first report which documents conjunctival neuromas by confocal microscopy in MEN2B.
Source: Cornea - Category: Opthalmology Tags: Case Report Source Type: research
To evaluate the effect blepharitis lid cleansers have on the tear film and ocular surface, and to examine the prevalence of Demodex folliculorum in a young population.
Source: Contact Lens and Anterior Eye - Category: Opthalmology Authors: Source Type: research
Conclusions: Tsukamurella tyrosinosolvens and Tsukamurella pulmonis were found to be the predominant species that caused ocular infection. Ocular manifestation of Tsukamurella has a wider spectrum than that previously reported. A high-level of suspicion and a low threshold for microbiological sampling in cases with prolonged ocular surface infection are recommended to diagnose Tsukamurella infections.
Source: Cornea - Category: Opthalmology Tags: Case Report Source Type: research
Conclusion: Both formulations improved ocular surface parameters. Group 2 showed more reduction in tear cytokines and Demodex count. PMID: 31429629 [PubMed - as supplied by publisher]
Source: Ocular Immunology and Inflammation - Category: Allergy & Immunology Tags: Ocul Immunol Inflamm Source Type: research
AbstractPurpose of ReviewThe human body lives in a symbiotic relationship with the bacteria, viruses, fungi, and protozoa that make up the microbiome. In this review, we discuss the compositions of the gut and ocular surface microbiomes in relationship to health and disease.Recent FindingsThe gut microbiome is dominated by Firmicutes, whereas the ocular surface is dominated by Proteobacteria. The compositions of the microbiome are similar between individuals at the phyla level, but differ at the genus level. Alterations in the microbiome have been associated with disease. For example, ocular diseases such as uveitis, dry e...
Source: Current Ophthalmology Reports - Category: Opthalmology Source Type: research
CONCLUSION: Coagulase-negative Staphylococcus and Staphylococcus aureus were the most isolated bacteria in patients affected by blepharitis in our tertiary eye centre. Both bacteria were resistant to erythromycin. Through the years, it seems that coagulase-negative Staphylococcus gained resistance to penicillin, erythromycin, ciprofloxacin and rifampicin. PMID: 31232091 [PubMed - as supplied by publisher]
Source: European Journal of Ophthalmology - Category: Opthalmology Authors: Tags: Eur J Ophthalmol Source Type: research
Demodex mites are commonly found on the periocular skin and eyelashes, with increasing rates observed with age. [1,2] However, these microscopic ectoparasites found on the skin of healthy asymptomatic humans have also been shown to have pathogenic potential [3,4]. Demodex have been associated with anterior and posterior blepharitis, disorders of the eyelashes, keratoconjunctivitis and contact lens wear. [1,5] Furthermore, ocular discomfort has been also been associated with higher Demodex counts.
Source: Contact Lens and Anterior Eye - Category: Opthalmology Authors: Source Type: research
CONCLUSION: We reported the efficiency of the different treatments of Demodex blepharitis. Because of less systemic side effects, local treatments seem promising molecules in the treatment of Demodex blepharitis. PMID: 31229586 [PubMed - as supplied by publisher]
Source: The Ocular Surface - Category: Opthalmology Authors: Tags: Ocul Surf Source Type: research
Conclusion: Microbial keratitis can affect those with OSD. In our setting, CoNS were the main organisms identified. Furthermore, patients prescribed a combination therapy of fortified antibiotics had poorer outcomes compared to monotherapy fluoroquinolones. PMID: 31189397 [PubMed - as supplied by publisher]
Source: Current Eye Research - Category: Opthalmology Authors: Tags: Curr Eye Res Source Type: research
ConclusionOcular manifestations are significant in patients with RA. The most common ocular manifestation is dry eye. Ocular manifestations are significantly associated with the disease duration and should be expected regardless of disease activity in RA patients.
Source: The Egyptian Rheumatologist - Category: Rheumatology Source Type: research
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