Lipoid Proteinosis (Urbach-Wiethe Disease): A Case Report from India

Batra, Kadambari; Safaya, A.; Aggarwal, Kiran

doi:10.1177/014556130808700914

Cited by 6 publications

(4 citation statements)

References 8 publications

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“…Fourteen patients (70%) had a better voice; but unfortunately, in most cases, nothing is known about the duration of the improvement. 1,[25][26][27][28][29] In two patients (10%) (one laser and one cold instruments), the voice was better for several months, but recurrence was documented. 17,30 One patient (5%) had no improvement, 31 and one study described improved of stridor, but not of hoarseness after laser excision.…”

Section: Surgerymentioning

confidence: 99%

Lipoid Proteinosis: A Rare Cause of Hoarseness

2019

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Lipoid proteinosis is a rare cause of voice problems. Hoarseness is often the first clinical manifestation of this disorder and can present years before any other symptom. Therefore, it is very important as an otorhinolaryngologist to be familiar with the main characteristics of this disease. We present a case report and a review of current literature to provide a concise overview of this frequently missed diagnosis.

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Section: Surgerymentioning

confidence: 99%

Lipoid Proteinosis: A Rare Cause of Hoarseness

2019

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“…Hence, review of 51 cases of LP reported in India from 1969 to 2021 is done along with the present case [ Table 1a – c ]. [ 1 4 5 6 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 ] LP is more reported in patients in the age group of 11-20 (51%). More prevalence of LP is reported in males (70.6%).…”

Section: Review and Discussionmentioning

confidence: 99%

“…On review of oral manifestations, 42 cases were found as shown in Table 1c . [ 1 4 5 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 25 26 27 28 29 30 31 32 33 34 35 37 38 39 42 43 44 ] The most common involved site is tongue with typical thick woody appearance followed by restricted tongue movements. In the present case, also similar findings were present.…”

Section: Review and Discussionmentioning

confidence: 99%

Lipoid proteinosis

Shah

2022

Journal of Oral and Maxillofacial Pathology

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Lipoid proteinosis (LP) is a rare autosomal recessive disorder characterized by the deposition of amorphous hyaline material in the dermis and submucosal connective tissue. To date <500 cases of LP have been described and oral manifestations described in a very few reports. Indian cases are much less reported and reviewed. Hence, here review of 51 Indian LP cases along with a case of histologically proven LP in 12-year-old male patient with typical skin, ocular, laryngeal, oral and radiographic features is done. Cases from 1969 to 2021 were collected using keyword LP on google and google scholar and Indian cases were analyzed afterward. Review with case presentation regarding oral manifestations will help the oral physician to diagnose LP in early stage.

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“…Lipoid proteinosis (LP) is known as Urbach-Wiethe syndrome or hyalinosis cutis et mucosae and was first reported by dermatologist and otolaryngologist duo Urbach and Wiethe in 1929 [1]. LP is a rare entity with autosomal recessive inheritance and multiple system involvement causing dermatological, otorhinolaryngological, ocular, and neurological manifestations.…”

Section: Introductionmentioning

confidence: 99%

Lipoid Proteinosis Masquerading as Seborrheic Dermatitis

Tripathi¹,

Gupta²

2021

Cureus

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We report a case of lipoid proteinosis (LP) masquerading as seborrheic dermatitis. A 35-year-old female presented to our outpatient department with complaints of itching and crust-like formation on eyelids for five years. She was treated as a case of seborrheic dermatitis elsewhere and got intermittent relief in itching with medications. Beaded lesions were found along the upper and lower eyelids involving the lash line and caruncle on removing the crust. The verrucous lesions were pathognomonic, moniliform blepharosis of LP. Systemic examination revealed hoarseness of voice, and hyperkeratosis was seen on the dorsum of both of her hands. She has been advised lid hygiene, artificial tears, and antihistaminics for itching. Skin emollients were also advised by dermatologists to decrease the chances of abrasion and bleeding from minor trauma. She was well explained about the danger signs as well as neurological and psychiatric implications of the disease. Although ophthalmologists have a rare encounter with this disease, LP is a well-known entity to dermatologists and otorhinolaryngologists, and thus it may sometimes go undiagnosed. An ophthalmologist should be well aware of the life-threatening complications associated with LP, and patients should be sensitized regarding the chronic nature, supportive measures, and danger signs of the disease.

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Lipoid Proteinosis (Urbach-Wiethe Disease): A Case Report from India

Cited by 6 publications

References 8 publications

Lipoid Proteinosis: A Rare Cause of Hoarseness

Lipoid Proteinosis: A Rare Cause of Hoarseness

Lipoid proteinosis

Lipoid Proteinosis Masquerading as Seborrheic Dermatitis

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