Objective: Lipoid proteinosis (LP) is a rare autosomal recessive genodermatosis characterized by the accumulation of amorphous hyaline substance in the skin and mucous membranes. In this study, the histopathological findings of the patients who were admitted to our clinic and diagnosed with LP were examined. Materials and Methods: This prospective study included 18 patients who presented to our clinic between January 2014 and December 2018 and were confirmed by histopathological examination. A punch biopsy including epidermis, dermis, and subcutaneous tissues was obtained from the lesional skin of each patient evaluated clinically, and the material was stained with hematoxylin and eosin stain and periodic acid–Schiff (PAS) stain. These preparations were evaluated by a pathologist experienced in dermatopathology. Results: The most common histopathological findings in the epidermis were hyperkeratosis (88.8%) and pigmentary incontinence (83.3%) in the basal layer. The most common histopathological findings in the dermis were amorphous substance accumulation (100%), perivascular PAS positivity (33.3%), and PAS positivity around eccrine glands (11.1%). Conclusion: The findings of our study were similar to the histopathological findings of late-term skin lesions in LP patients previously described in the literature. In order to better understand the histopathological findings of skin lesions of LP patients, studies with a large number of patients including early skin lesions of LP are needed.

Histopathological findings in patients with lipoid proteinosis

Lipoid proteinosis (LP) is a rare metabolic storage disease characterized by the storage of hyaline-like substances in the skin, mucosa, and many other organs. The aim of this study was to compare the neutrophil/lymphocyte ratio (NLR), platelets/lymphocyte ratio (PLR), and mean platelet volume (MPV) values of LP patients with healthy control group and to determine the usefulness of these parameters in determining the inflammatory status in LP patients. This study included 14 patients who were admitted to our clinic between March 2013 and January 2017 and diagnosed as LP with clinical, radiological, and histopathological examinations with 14 individuals who had no systemic inflammatory disease or malignancy and has a hemogram result. There was no statistically significant difference between the two groups in comparison to the MPV and PLR values of the patients with LP and healthy controls (P values 0.666 and 0.654, respectively). When the NLR values of the patients with LP and healthy controls were compared, it was found that the LP-diagnosed NLR values were statistically higher. (P: 0.019). In conclusion, it can be said that NLR is an appropriate parameter in demonstrating inflammation in LP patients, but PLR and MPV are not a suitable parameter in demonstrating inflammation in LP patients. Prospective studies with more patients are needed.

Evaluation of inflammatory parameters in lipoid proteinosis patients.

SUMMARY OBJECTIVE: Lipoid proteinosis is a rare autosomal recessive genetic dermatological disease that occurs due to the accumulation of hyaline material in the skin and mucous membranes. This study aimed to investigate whether dynamic thiol-disulfide homeostasis is a new marker of oxidative stress in patients suffering from lipoid proteinosis. METHODS: The study group involved 17 patients with lipoid proteinosis and 17 healthy controls with same gender and age. Native thiol, total thiol, disulfide levels, and thiol-disulfide indexes were measured with the fully automated spectrophotometric method described by Erel and Neselioglu, and the results of the two groups were statistically analyzed. RESULTS: Serum total thiol and native thiol levels were significantly lower in lipoid proteinosis group compared to the control group (p=0.020 and p=0.014, respectively). The disulfide levels were found to be higher in lipoid proteinosis group, but there was no significant difference between two groups. CONCLUSIONS: Impaired dynamic thiol-disulfide homeostasis was observed in lipoid proteinosis patients, suggesting that thiol-disulfide homeostasis may have a role in the pathogenesis of this disease.

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Assessment of dynamic thiol-disulfide homeostasis in patients with lipoid proteinosis (Urbach-Wiethe syndrome)

OBJECTIVE The aim of this study was to evaluate serum thiol disulphide levels in patients with cutaneous leishmaniasis (CL) compared with a healthy control group and to investigate whether thiol/disulphide balance can be used as a new marker of oxidative stress in CL patients. MATERIAL AND METHODS This prospective study included 47 patients diagnosed with CL and 46 healthy individuals without any disease. Native thiol, disulphide and total thiol levels were used in plasma. Disulphide/total thiol, disulphide/native thiol and native thiol/total thiol levels were measured. RESULTS Disulphide, native thiol disulphide and total thiol disulphide levels were significantly lower in CL patients compared with the control group. However, there was no statistically significant difference between CL patients and the control group in terms of disulphide/native thiol, disulphide/total thiol and native thiol/total thiol values. CONCLUSION Based on the data in our study, it can be said that the use of thiol/disulphide balance as a new oxidative stress marker in CL patients is not appropriate, but studies with a larger number of patients are needed.

Evaluation of thiol/disulphide balance in patients with cutaneous leishmaniasis.

AIM Lipoid proteinosis (LP) is a systemic, progressive, rare genodermatosis that manifests in early life with mucocutaneous lesions. Kidney involvement has not been described before, hence we aimed to investigate kidney findings in LP patients. MATERIALS AND METHODS In this cross-sectional study, LP patients who were followed up and diagnosed with clinical, histopathological and radiological findings in the Dermatology outpatient clinic were invited to the Pediatric Nephrology Clinic. Biochemical, spot urine and 24-hour urine collection tests and ultrasound scans were scanned for the functional and anatomical status of the kidneys. Healthy controls who visited the Pediatric Nephrology Clinic during study periods for other reasons were invited to participate as the control group. RESULTS Data of 44 participants (22 LP patients, mean age 14.5 years and 22 healthy controls, mean age 14.3 years) were available for the analysis. Ultrasound scans revealed simple renal cysts in 68% of LP patients. The cysts were bilateral in 4 patients, while no cyst was detected in healthy controls. Biochemical parameters, proteinuria, and albuminuria were all within the normal reference range in both LP patients and healthy controls, whereas LP patients had statistically significant lower serum albumin and higher C-reactive protein levels than healthy controls. CONCLUSION Multiple simple renal cysts were detected in the majority of LP patients, and this finding could be part of the systemic involvement of the disease. Renal functions and biochemical parameters were within the normal reference range, but low serum albumin and high C-reactive protein levels in LP patients may be the signs of elevated systemic inflammation. Although more studies are needed to better define these findings, the presence of many simple renal cysts in LP patients was found to be the most important finding of this study.

Are the kidneys involved in lipoid proteinosis? Study of 22 patients

Amac: Bu calismadaki amacimiz, total antioksidan seviye (TAS), ferrik iyon indirgeyici antioksidan guc (FRAP) ve toplam serbest sulfhidril gruplari (Total-SH) gibi antioksidan parametreler ile total oksidan seviye (TOS), ileri oksidasyon protein urunleri (AOPP), oksidatif stres indeksi (OSI) ve lipid hidroperoksit (LOOH) gibi oksidasyon parametrelerini olcerek Lipoid Proteinozlu (LP) hastalarda oksidatif stresin rolunu arastirmaktir. Materyal ve Metod: Bu prospektif vaka kontrol calismasina klinik, radyolojik ve histopatolojik olarak tani konulan 14 LP hastasi ve cinsiyet, yas ve vucut kitle indeksi benzer 14 saglikli gonullu katilimci dahil edildi. Serum TAS, TOS, AOPP, FRAP, LOOH ve Total-SH seviyeleri spektrofotometrik olarak olculdu ve OSI degeri hesaplandi. Bulgular: LP'li hastalarda serum TAS, FRAP ve Total –SH (p< 0,001; her biri icin) seviyeleri daha yuksek bulunurken OSI, AOPP ve LOOH duzeyleri daha dusuk (sirasiyla, p= 0,001, p= 0,018 ve p= 0,047) bulundu. LP hastalarinda serum TOS seviyeleri kontrol grubuna gore daha yuksekti, ancak bu yukseklik istatistiksel olarak anlamli degildi (p= 0,081). Yapilan ROC analizinde TAS, FRAP, Total-SH ve OSI seviyelerinin LP hastaligini guclu bir duyarlilik ve yuksek bir ozgulluk ile ongordugunu gosterdi. Sonuc: LP hastaliginin tanisinda, antioksidan parametrelerindeki azalma ve oksidan parametrelerindeki artmanin yardimci laboratuvar testleri olarak kullanilabilecegini gostermektedir.

/pdf/the-investigation-of-oxidative-stress-parameters-in-patients-jr2tbo6cpn.pdf

The Investigation of Oxidative Stress Parameters in Patients with Lipoid Proteinosis

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The Investigation of Oxidative Stress Parameters in Patients with Lipoid Proteinosis