Familial Lichen Amyloidosis.

Rajagopalan, K.V.; Ch, Tay

doi:10.1111/j.1365-2133.1972.tb16186.x

Cited by 50 publications

(3 citation statements)

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“…[28] Although most cases of PLCA are sporadic, 10% of cases have been reported to be familial. [323] Female preponderance has been consistently reported in the literature,[3614] except for a study by Black et al . which reported more males to be affected.…”

Section: Discussionmentioning

confidence: 99%

A clinico-epidemiological study of macular amyloidosis from North India

2012

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Background:Macular amyloidosis (MA) is the most subtle form of cutaneous amyloidosis, characterized by brownish macules in a rippled pattern, distributed predominantly over the trunk and extremities. MA has a high incidence in Asia, Middle East, and South America. Its etiology has yet to be fully elucidated though various risk factors such as sex, race, genetic predisposition, exposure to sunlight, atopy and friction and even auto-immunity have been implicated.Aim:This study attempts to evaluate the epidemiology and risk factors in the etiology of MA.Materials and: Methods:Clinical history and risk factors of 50 patients with a clinical diagnosis of MA were evaluated. Skin biopsies of 26 randomly selected patients were studied for the deposition of amyloid.Results:We observed a characteristic female preponderance (88%) with a female to male ratio of 7.3:1, with a mean age of onset of MA being earlier in females. Upper back was involved in 80% of patients and sun-exposed sites were involved in 64% cases. Incidence of MA was high in patients with skin phototype III. Role of friction was inconclusiveConclusion:Lack of clear-cut etiological factors makes it difficult to suggest a reasonable therapeutic modality. Histopathology is not specific and amyloid deposits can be demonstrated only in a small number of patients. For want of the requisite information on the natural course and definitive etiology, the disease MA remains an enigma and a source of concern for the suffering patients.

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Section: Discussionmentioning

confidence: 99%

A clinico-epidemiological study of macular amyloidosis from North India

2012

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“…found cutaneous amyloidosis in members of four successive generations, suggesting an autosomal dominant mode of transmission. [16] Desouza reported four siblings with localized cutaneous amyloidosis. [17] However, Looi LM reported that most cases of primary cutaneous amyloidosis did not have a familial basis.…”

Section: Discussionmentioning

confidence: 99%

Study on epidemiology of cutaneous amyloidosis in northern India and effectiveness of dimethylsulphoxide in cutaneous amyloidosis

Krishna

Nath

Dhir

et al. 2012

Indian Dermatol Online J

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Context:Amyloidosis, which is characterized by the extracellular deposition of a proteinaceous substance, is usually associated with considerable tissue dysfunction. However, the etiology of the disease remains uncertain and the treatment disappointing.Aim:1. To know the epidemiology of cutaneous amyloidosis 2. To evaluate the effect of dimethylsulphoxide on cutaneous amyloidosis.Settings and Design:Data was collected from patients attending the Outpatient Department (OPD) over a period of one year.Material and Methods:Patients were screened on the basis of signs and symptoms and then confirmed histologically. A total of 62 patients who were suspected to be suffering from amyloidosis on the basis of clinical signs and symptoms and 38 patients who were further confirmed histopathologically underwent the treatment.Statistical Analysis Used:Chi-square test was used for testing the significance of proportions.Results:63.15 percent of the patients had macular amyloidosis and the interscapular area was the most common area involved (52.63%). Pruritus, pigmentation, and papules responded excellently to dimethylsulphoxide after one month of treatment.Conclusions:Cutaneous amyloidosis is a disease found in middle-aged persons, with a female preponderance, and dimethylsulphoxide seems to be an effective therapy.

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“…[2021] The overall positive family history of 17% in this study was similar to other studies, an observation that showed genetic predisposition and racial factor might contribute to a minor role in the occurrence of PCA. [222324]…”

Section: Discussionmentioning

confidence: 99%

Clinicopathological study of primary cutaneous amyloidosis in a tertiary care center of Eastern India reveals insignificant association with friction, scrubbing, and photo-exposure: How valid is the “keratinocyte hypothesis”?

Biswas

Pal

et al. 2019

Indian J Dermatol

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Introduction:Primary cutaneous amyloidosis (PCA) can be classified into four principal categories: macular amyloidosis, lichen amyloidosis, biphasic, and nodular amyloidosis. Some unusual variants such as widespread diffuse hyperpigmentation without papules, poikiloderma like involvement, lesions following Blaschko's line, etc., have also been reported. However, not much data are available regarding the demography, epidemiology, clinical patterns, and distribution and histopathological findings, especially from the eastern part of India.Aims:We conducted a cross-sectional, institution-based study to evaluate clinicopathological pattern and factors of PCA in eastern India.Materials and Methods:We recorded clinical and histopathological findings of 100 consecutive patients of PCA presenting to a tertiary care institution of Kolkata in eastern India.Results:We found female patients of PCA outnumber male (M:F =1:1.9) with majority of patients being young adults (56%) between 20 and 40 years of age. More than half (54%) of the patients were pruritic. The severity of pruritus is significantly more associated with lichenoid and biphasic variants over macular amyloidosis. Positive family history was recorded in 17% of cases. Macular variant was the most common variant constituting 48% of the total PCA. We also found that the association with history of friction and scrubbing and photo-exposure were statistically insignificant. However, duration of the disease has statistically significant association with the disease morphology. Congo red stain showed these deposits as reddish orange substance in 28 patients out of 64 patients’ samples on which Congo red could be performed.Conclusion:Our study revealed that many concepts of pathogenesis of PCA including friction and photoexposure might have lesser importance. However, morphological types were significantly associated with the duration of the disease and symptom severity.

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Familial Lichen Amyloidosis.

Cited by 50 publications

References 20 publications

A clinico-epidemiological study of macular amyloidosis from North India

A clinico-epidemiological study of macular amyloidosis from North India

Study on epidemiology of cutaneous amyloidosis in northern India and effectiveness of dimethylsulphoxide in cutaneous amyloidosis

Clinicopathological study of primary cutaneous amyloidosis in a tertiary care center of Eastern India reveals insignificant association with friction, scrubbing, and photo-exposure: How valid is the “keratinocyte hypothesis”?

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