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Amyloidosis is characterized by extracellular amyloid protein deposition. When amyloidosis intersects with basal cell carcinoma (BCC), it introduces complex diagnostic challenges. This study explored the overlap between primary localized cutaneous amyloidosis (PLCA) and BCC, examining amyloid deposits in BCC, systemic amyloidosis risk in PLCA, and various treatment methods. Two case studies were discussed, followed by a literature review, in which PubMed, Web of Science, EMBASE, and the Cochrane Library databases were utilized. The search, covering studies from infinity up to January 2024, focused on "cutaneous amyloidosis," "basal cell carcinoma," and related terms. Articles in English detailing the clinical presentation, diagnostic methods, treatment, and outcomes of cutaneous amyloidosis mimicking BCC were included. Data extraction and synthesis were performed by two independent reviewers. This study highlighted two cases exemplifying the complexity of diagnosing BCC and PLCA. The first case (a 64-year-old with a nodule on the cheek) and the second (a 67-year-old with a nodular lesion on the upper lip cheek) were initially suspected as BCC and were later identified as PLCA upon histopathological examination. The diagnosis of amyloidosis within BCC nodules remains a diagnostic challenge. Although their coexistence is relatively prevalent, their local recurrence rates remain debatable. Various diagnostic and therapeutic approaches have been suggested, such as topical creams and phototherapy. However, none have garnered conclusive and consistent evidence to establish reliable clinical application. The findings emphasized the importance of considering alternative pathologies in differential diagnoses. Future research should focus on understanding systemic amyloidosis risks and optimizing care for both conditions. [ABSTRACT FROM AUTHOR]

This article discusses a pilot study that explores the efficacy of topical 0.025% capsaicin gel for the treatment of lichen amyloidosis, a common form of primary localized cutaneous amyloidosis found in Southeast Asia. The study found that the capsaicin gel significantly reduced papule induration scores, but did not significantly improve hyperpigmentation. The treatment was well-tolerated by participants and had minimal adverse effects. The authors suggest that topical capsaicin could be an affordable and effective treatment option for lichen amyloidosis. However, further research with larger sample sizes and longer treatment durations is needed. [Extracted from the article]

Amyloidosis cutis dyschromica is a very rare form of primary cutaneous amyloidosis characterized by prepubertal onset of hyper and hypopigmented spots and amyloid deposits in the papillary dermis. We report two cases of this condition highlighting its dermoscopic findings. [ABSTRACT FROM AUTHOR]

The article presents a case study of a rare subtype of primary cutaneous amyloidosis known as amyloidosis cutis dyschromica (ACD) involving three patients aged 24, 28, and 34. Topics discussed include the clinical presentation and diagnostic challenges of ACD, its dermoscopic and histopathological features, and the various treatment options and their effectiveness.

This article discusses a rare form of cutaneous amyloidosis called amyloid elastosis (AE). The article presents a case study of a 58-year-old man with AE, along with a review of existing literature on the topic. The patient had three lesions, with the most recent one appearing on his right arm. Dermoscopic examination revealed different characteristics for each lesion. The article also provides information on the pathogenesis, clinical features, and treatment options for AE. The authors emphasize the importance of considering AE in the differential diagnosis of anetodermic lesions and highlight the differences between primary localized cutaneous AE and AE associated with systemic amyloidosis. The article concludes by stating that although AE is a rare condition, the findings from this case study contribute valuable clinical insights for managing AE patients. [Extracted from the article]

Fujuan Huang,1,&ast; Yuling Zhang,2,&ast; Junyi Guo,3 Hongju Pan,4 Zhigang Liao,4 Bin Yang,1 Ping Lu1 1Department of Dermatology, Dermatology Hospital of Southern Medical University, Guangzhou, People’s Republic of China; 2Department of Dermatology, The First Hospital of Jilin University, Changchun, People’s Republic of China; 3Department of Dermatology, Guangdong Provincial Dermatology Hospital, Guangzhou, People’s Republic of China; 4Guangdong Provincial Engineering Technology Research and Development Center for External Drugs, Foshan, People’s Republic of China&ast;These authors contributed equally to this workCorrespondence: Ping Lu; Bin Yang, Department of Dermatology, Dermatology Hospital of Southern Medical University, Guangzhou, People’s Republic of China, Email lvping75@163.com; yangbin101@hotmail.comPurpose: To compare epidermal biophysical properties, indicators of epidermal function, in individuals with and without primary cutaneous amyloidosis (PCA).Patients and Methods: This study incorporated 189 patients with PCA and 166 healthy individuals. The GPSkin Barrier was employed to measure transepidermal water loss (TEWL) rates and hydration levels of the stratum corneum. The Sebumeter and the Skin pH Meter were utilized to determine the skin surface’s sebum content and pH, respectively. The severity of pruritus in participants was evaluated using the visual analog scale (VAS).Results: Compared to the control group without PCA, individuals with PCA displayed a notable increase in skin surface pH and TEWL and a decrease in the hydration levels of the stratum corneum (p< 0.0001 for all parameters). Additionally, the sebum content was markedly lower in those with PCA than in the controls (p< 0.0001). Of particular note, both TEWL and skin surface pH at the lesion sites on the back and the shin were more elevated in lichenoid amyloidosis (LA) and in macular amyloidosis (MA), whereas hydration levels of the stratum corneum and sebum levels were diminished in LA compared to MA (p< 0.05). In conclusion, both hydration levels of the stratum corneum and sebum content exhibited an inverse relationship with pruritus severity, whereas TEWL and skin surface pH demonstrated a positive correlation with pruritus intensity.Conclusion: The function of the epidermis is compromised in individuals diagnosed with PCA. However, the mechanisms underlying these changes await further investigation.Keywords: cutaneous amyloidosis, epidermis, biophysical properties, pruritus

Background: Macular amyloidosis (MA) represents a common variant of primary localized cutaneous amyloidosis (PLCA). Many therapeutic modalities, including topical and systemic medications, phototherapy, photochemotherapy, dermabrasion, and lasers have been suggested for PLCA with variable efficacy. Over the past decade, several studies have reported successful treatment of PLCA with different types of lasers. Aim: To evaluate the efficacy and safety profile of fractional carbon dioxide laser (FrCO2) in primary cutaneous MA. Methods: Using the consecutive sampling technique, 30 clinically confirmed cases of MA aged 18 years and above attending the dermatology out-patient department were recruited for a prospective, single group, interventional study. Each patient received three sessions with 4 weeks interval and then subsequently monitored for 20 weeks. The degree of pigmentation, rippling, itching, overall progress, and patient satisfaction were graded and compared for each patient. SPSS software version 23.0 was used for data analysis. Results: There was a statistically significant (P < 0.001) improvement in the mean percentage of the degree of pigmentation, itching (P < 0.001), and rippling scores (P= 0.03). Marked patient satisfaction was noted at 20 weeks. The excellent safety profile was noted. Conclusion: Our study has shown a positive response to treating MA with FrCO2 laser. Patients were highly satisfied, as there was a significant reduction in itching, pigmentation, and area of the skin lesion with no significant side effects. Our study points towards better efficacy of FrCO2 laser in MA with a good safety profile and better patient satisfaction. [ABSTRACT FROM AUTHOR]

Cutaneous manifestations in systemic amyloidosis secondary to multiple myeloma or AL amyloidosis are seen in approximately 15%-40% of patients with systemic amyloidosis. Cutaneous involvement has a preference for skin folds, retroauricular region, eyelids, neck and the axilla and may present in the form of purpura, domed papules or nodes resembling isolated nodular amyloidosis. Congo red staining remains the gold standard for diagnosis, revealing amyloid fibrils with apple-green birefringence in the polarized microscopy. It is noteworthy that patients may sometimes present with non-specific skin changes, suggestive of depositional disease, such as alopecia or nail dystrophy. Herein, we present a patient with systemic amyloidosis, who exhibited red-brown macules coalescing into a rippled pattern in the extremities, amyloid nodules in the peri-auricular region and dystrophic nails, findings that were attricuted to cutaneous amyloidosis in the context of systemic amyloidosis. [ABSTRACT FROM AUTHOR]

Cutaneous amyloidosis is a disease characterized by the abnormal deposition of fibrous proteins in the skin arranged in a folded sheet in a beta spatial configuration that tends to invade and destroy it. Amyloid fibrils are derived from immunoglobulin and are both kappa and lambda light chains. They are classified in accordance with their morphology as papular or lichenoid, muscular and nodular. Due to its nature as a primary fibrillary protein, amyloidosis may also be classified as primary or secondary. We made a review of literature by means of the PubMed (MEDLINE), EMBASE (Elsevier), Cochrane, EBSCO (Dynamed) y Ovid, including the most relevant articles for our review. The diagnostic approach should include a complete clinical history and physical examination, as well as serum protein electrophoresis and urine protein electrophoresis, and biopsies of the gingival, rectal, or abdominal fat pad to rule out the presence of extracutaneous amyloid deposits. Treatment of amyloidosis is ineligible and is controversial; however, it depends on the type, as well as the patient's prognosis. [ABSTRACT FROM AUTHOR]

The purpose of this study was to apply functionalized magnetic nanoparticles for the treatment of amyloidosis, a disease characterized by the accumulation of aberrant protein forms with an insoluble amyloid structure. The dissolution and clearance of these extremely stable fibrils from lesions is very complicated. For this purpose, we examined the possibility of using magnetic nanoparticles that generate heat in an external alternating magnetic field with a frequency of 3.5 MHz. As a convenient model system, we used lysozyme fibrils. For the quantification of fibrillar status, we used Thioflavin T and Congo red, specific dyes which change their spectroscopic properties upon binding with the cross-beta structure of fibrils. We found that by using fluorescence, and polarization microscopy, as well as absorption spectrophotometry, the amyloid-like fibrils can be almost completely dissolved. The obtained results suggest that the application of magnetic nanoparticles could be a possible therapeutic intervention in cutaneous amyloidosis. [ABSTRACT FROM AUTHOR]

BACKGROUND: Amyloidosis is an uncommon disorder characterized by the extracellular deposition of amorphous and insoluble proteins in an abnormal fibrillary configuration. Mammary amyloidosis is an unusual and easily overlooked diagnosis with an ambivalent presentation that could mimic breast cancer. CASE REPORT: We here report the case of 60-year-old Caucasian woman who presented to our surgical oncology department for clinically and radiologically suspicious breast mass. A fine needle biopsy was irrelevant, so we performed a lumpectomy. Final histology revealed an amyloid deposit and further workup was consistent with nodular cutaneous and breast AL amyloidosis associated with Sjögren's syndrome. CONCLUSION: Although rare, an awareness of the clinicopathologic characteristics of this easily overlooked entity is of great importance for breast surgeons. [ABSTRACT FROM AUTHOR]

Amyloidosis describes a group of diseases characterized by abnormal extracellular deposition of a fibrillar protein in tissues. It comprises of a spectrum of diseases ranging from a mild asymptomatic cutaneous involvement to a progressive life-threatening multisystem disorder. Primary localized cutaneous amyloidosis (PLCA) is characterized by pruritus, hyperpigmentation, and lichenification. Demonstration of amyloid in dermal papillae by various techniques confirms the diagnosis. Three types are described, macular, lichen/papular, and nodular. Majority of cases of PLCA are sporadic but an autosomal dominant family history may be present in up to 10% of cases. Exact pathogenesis is not elucidated yet, but several factors like friction, sunlight, genetics, atopy, immune dysregulation have been implicated. Pathogenic mutations in oncostatin M receptor and the interleukin (IL)-31 receptor A gene (IL31RA) have an important role to play in the pathogenesis of both familial and sporadic PLCA. Nodular amyloidosis, the rarest type of PLCA, is known to progress to systemic amyloidosis in 7% to 15% of cases, hence a long-term follow up is recommended. The plethora of treatment options reported to be beneficial in cutaneous amyloidosis underscores the fact that none of the modality is universally effective. [ABSTRACT FROM AUTHOR]

Background: Primary cutaneous amyloidosis (PCA) and post‐inflammatory hyperpigmentation (PIH) are common causes of cutaneous hyperpigmentation that are usually diagnosed clinically. However, their presentations are sometimes atypical, and their differentiation is difficult. Dermoscopy is a valuable diagnostic tool for pigmented diseases that might aid in their diagnosis. Objective: To describe the characteristic dermoscopic features of PCA and PIH together with histopathological correlation, and to differentiate between these conditions in a non‐invasive way. Methods: Fifty‐two patients with PCA (n = 26) and PIH (n = 26) were enrolled. A detailed history, skin examination, dermoscopic examination using handheld and video dermoscopy, and histopathological evaluation were performed. Results: A statistically significant difference could be detected between PCA and PIH in terms of the duration of the disease (p = 0.027), symmetry (p = 0.044), rippling (p < 0.001), and back affection (p = 0.048). On dermoscopic examination, central hubs were seen more in the PCA group (p < 0.001) with different patterns of peripheral pigmentation. Histopathologically, the number of melanophages per high‐power field was significantly higher in the PCA group (p = 0.013). Conclusion: The results of this study shed the light on the potential of dermoscopy as a non‐invasive diagnostic tool in differentiating between doubtful cases of PCA and PIH. [ABSTRACT FROM AUTHOR]

Frictional melanosis is a condition that is often encountered in dermatology practice. The acquired pigmentary disorder has clinical similarity to the condition termed macular amyloidosis. These two entities have etiological-epidemiological and clinical overlap. Dermoscopy and histopathology can demonstrate subtle differences. The review is an attempt to understand the different etiological aspects proposed for these conditions. We discuss the clinical features of the two conditions, dermoscopic features and histopathological changes. It is imperative for dermatologists to be aware of the similarities and the differences between the two entities for their better management.

Secondary localized cutaneous amyloidosis is a histopathological finding seen in the dermis, in various benign, premalignant, and malignant skin conditions, without clinical significance. The real incidence is not known. We aimed to investigate the phenomenon of secondary localized cutaneous amyloidosis in Bowen�s disease and Bowenoid papulosis. We retrospectively evaluated the data of all cases with histopathological confirmation of Bowen�s disease and Bowenoid papulosis between 2006 and 2017 in our Dermatovenereology and/or Pathology departments. Secondary localized cutaneous amyloidosis was observed in three patients with Bowen�s disease (3/52; 5.8%) and in three patients with Bowenoid papulosis (3/18; 16.7%). Herein, we present the demographic, clinical and histopathological features of these six cases of secondary localized cutaneous amyloidosis in detail. Although the occurrence of secondary localized cutaneous amyloidosis in epithelial tumors is a well-known phenomenon, its incidence has not been previously reported in Bowen�s disease and Bowenoid papulosis. Therefore, our results indicating a high incidence may be particularly important for Bowenoid papulosis, as its association with secondary localized cutaneous amyloidosis has only been shown in one case before. Moreover, in three of six cases, we histologically observed areas of regression with a marked prominence of amyloid deposition. Remarkably, two of these patients had a history of topical application of destructive agents which reveals a possible etiologic relationship between secondary localized cutaneous amyloidosis and cellular apoptosis/necrosis induced by these external agents.

BackgroundApart from myasthenia gravis (MG), thymoma is associated with a wide spectrum of autoimmune paraneoplastic syndromes (PNSs). Here, we report on a rare case presenting with four different PNSs, namely, MG, membranous nephropathy, cutaneous amyloidosis, and Morvan’s syndrome associated with thymoma.Case presentationA middle-aged man was frequently hospitalized because of nephrotic syndrome (stage I membranous nephropathy), cutaneous amyloidosis, and MG with acetylcholine receptor (AChR) antibody and titin antibody positivity. Chest CT showed a thymic mass in the left anterior mediastinum, and he received intravenous immunoglobulin (IVIG), methylprednisolone pulse therapy, thoracoscopic thymoma resection, and radiotherapy. Postoperative pathological examination revealed a type B2 thymoma. During the perioperative stage, his electrocardiogram (ECG) showed myocardial infarction-like ECG changes; however, his levels of cardiac enzymes and troponin were normal, and he had no symptoms of precardiac discomfort. Six months after thymectomy, his nephrotic syndrome and MG symptoms were relieved; however, he presented with typical manifestations of Morvan’s syndrome, including neuromyotonia, severe insomnia, abnormal ECG activity, and antibodies against leucine-rich glioma-inactivated 1 (LGI1) and γ-amino-butyric acid-B receptor (GABABR). His symptoms did not improve after repeated IVIG and steroid therapies. Finally, he received low-dose rituximab, and his symptoms gradually resolved.ConclusionThis case serves to remind us that apart from MG, thymoma is also associated with other autoimmune PNSs such as membranous nephropathy, cutaneous amyloidosis, and Morvan’s syndrome. Autoimmune PNSs can present concurrently with or after surgical or medical therapy for thymoma. For Morvan’s syndrome post-thymectomy with LGI1 antibody positivity, B-cell depletion therapy such as intravenous rituximab is an effective treatment.

The article informs about a 52-year-old female with primary atrophic cutaneous amyloidosis, presenting as multiple, discrete, itchy, skin-colored to slightly hyperpigmented lesions over her trunk for the past 18 years. Topic include the clinical examination revealed sharply demarcated, atrophic areas with amyloid deposition confirmed by histopathology, and the study highlights the rarity of atrophic lesions as part of cutaneous amyloidosis.

Frictional melanosis is a condition that is often encountered in dermatology practice. The acquired pigmentary disorder has clinical similarity to the condition termed macular amyloidosis. These two entities have etiologicalepidemiological and clinical overlap. Dermoscopy and histopathology can demonstrate subtle differences. The review is an attempt to understand the different etiological aspects proposed for these conditions. We discuss the clinical features of the two conditions, dermoscopic features and histopathological changes. It is imperative for dermatologists to be aware of the similarities and the differences between the two entities for their better management. [ABSTRACT FROM AUTHOR]

As in other organs, the diagnosis of endogenous cutaneous overload diseases is based on histopathological analysis of the lesions using special stainings, even if the clinical appearance is sometimes very suggestive. The lesions are sometimes very subtle and can be included in the group of "invisible" dermatoses, such as primary macular cutaneous amyloidosis or calciphylaxis. Superficial dermal melanosis or pigmentary incontinence generally reflects the post-inflammatory stage of a chronic or recurrent interface dermatitis. Section levels should be systematically performed to look for active lesions of diagnostic interest: Alcian blue staining to identify dermal mucinosis (connectivitis) and pan-T markers (fixed pigmented erythema, lichenoid mycosis fungoides, and vitiligo). Some pathologies have a prognostic impact, either because they reflect an underlying disease, monoclonal gammopathies, in particular myeloma, being one of the most common conditions in this context (AL amyloidosis, xanthoma and xanthogranuloma, scleromyxedema), or because they can be associated with visceral damage (AL amyloidosis, scleromyxedema). The clinical-pathological comparison is mandatory to rule out differential diagnoses, especially for life-threatening diseases: nodular amyloidosis and primary cutaneous amyloidosis versus systemic AL amyloidosis, papular mucinosis versus scleromyxedema and calcific panniculitis versus calciphylaxis., (Copyright © 2024 The Author. Published by Elsevier Masson SAS.. All rights reserved.)

Nodular cutaneous amyloidosis represents the rarest variant of primary localized cutaneous amyloidosis. The proposed management ranges from topical or systemic agents to surgical treatment. Complete surgical excision is advisable due to its potential progression to systemic amyloidosis due to dermis and subcutaneous tissue infiltration. However, in particular locations, the risk of functional complications is high, so an alternative treatment option should be considered. We report a case of a large primary nodular cutaneous amyloidosis of the leg involving the joint capsule which was successfully treated by incomplete surgical removal, without recurrences at 7-year follow-up.

The purpose of this study was to apply functionalized magnetic nanoparticles for the treatment of amyloidosis, a disease characterized by the accumulation of aberrant protein forms with an insoluble amyloid structure. The dissolution and clearance of these extremely stable fibrils from lesions is very complicated. For this purpose, we examined the possibility of using magnetic nanoparticles that generate heat in an external alternating magnetic field with a frequency of 3.5 MHz. As a convenient model system, we used lysozyme fibrils. For the quantification of fibrillar status, we used Thioflavin T and Congo red, specific dyes which change their spectroscopic properties upon binding with the cross-beta structure of fibrils. We found that by using fluorescence, and polarization microscopy, as well as absorption spectrophotometry, the amyloid-like fibrils can be almost completely dissolved. The obtained results suggest that the application of magnetic nanoparticles could be a possible therapeutic intervention in cutaneous amyloidosis.

With the investigation of the efficacy of laser therapy in primary localized amyloidosis(PLCA) only recently starting to materialize, we aimed to review the currently available studies of laser therapy in the management of the disease. We searched PubMed, Scopus, Embase, Web of Science, Cochrane, and ProQuest online databases with a specified search strategy, assessed the quality of each study, and then extracted the eligible data. Five RCTs(randomized controlled trials), one non-randomized controlled trial, three case series, and nine case reports(18 in total) were included. Overall, carbon dioxide (CO2), neodymium-doped:yttrium aluminum garnet (Nd:YAG), pulsed dye (PDL), Er (Erbium):YAG, and yttrium/erbium fiber were the studied lasers. One hundred fifty-five cases in total underwent laser therapy, with CO2 being the most frequent laser. Almost all studies demonstrated significantly desirable outcomes, while only mild and transient side effects were noted. Even though the studies' results were significant, we noticed that implementing a consistent methodology and a standardized objective assessment method was missing. Therefore, we recommend that future studies be conducted with less heterogeneous data for a more definite conclusion. [ABSTRACT FROM AUTHOR]

Poikiloderma-like cutaneous amyloidosis (PCA) is a rare variant of primary cutaneous amyloidosis. It was first described in 1929 and there are two clinical forms of PCA, the ordinary type and PCA syndrome. The characteristics of PCA include poikiloderma-like skin changes, lichenoid papules, blister formation, and cutaneous amyloid deposits on histological examination. These skin lesions usually occur at the extremities, consistent with the few cases that have been reported. We present a case of a 62-year-old man who presented with the features of poikiloderma-like cutaneous amyloidosis. Diagnosis of this unique condition is a challenge and a skin biopsy is necessary in such instances. A discussion of the differential diagnosis of this condition is also included.

Macular amyloidosis (MA) is a common form of cutaneous amyloidosis that manifests as dark spots consisting of brown pigments with a rippled pattern on the skin, and the treatment of this condition is highly challenging. The aim of this study was to compare the efficacy and safety of intralesional injection of tranexamic acid (TXA) and topical application of Kligman combination drug in the treatment of macular amyloidosis. In this double‐blind clinical trial, a total of 43 patients, who were diagnosed with MA, were treated with two different methods of intralesional injection of tranexamic acid and topical application of Kligman combination drug. Both therapeutic methods were effective in improving MA and significantly reduced hyperpigmentation of the treated areas, but tranexamic acid was significantly more effective than the Kligman combination drug. Significantly, greater improvements were observed in the group of patients treated with tranexamic acid. In the tranexamic acid treatment group, ΔE was reduced from 11.39 in the first session to 8.53 in the third session, and in the Kligman treatment group, it was reduced from 8.79 in the first session to 6.32 in the third session (p < 0.05). In addition, the pruritus score in patients treated with topical tranexamic acid injection was lower compared to the patients treated with the topical application of the Kligman combination drug. The results of this study demonstrated the significant positive effects of both treatment methods, but in terms of reducing melanin content, intralesional injection of tranexamic acid was a more effective method. Both treatments considered safe for MA. In tranexamic acid group, patients logically experienced a tolerable pain during injection but they significantly had significantly lower local pruritic discomfort during study. So, based on the positive findings of this study we suggest to use tranexamic acid in combination with other effective therapeutic methods for treatment of MA especially use of its topically applied form in combination with non‐aggressive needling that results in better drug delivery without the experience of injection pain. Selection of the best administration route of tranexamic acid for hyperpigmented lesions depends on the each patient characteristic and their previous theraputic results that may vary case by case. [ABSTRACT FROM AUTHOR]

Secondary localized cutaneous amyloidosis is a histopathological finding seen in the dermis, in various benign, premalignant, and malignant skin conditions, without clinical significance. The real incidence is not known. We aimed to investigate the phenomenon of secondary localized cutaneous amyloidosis in Bowen's disease and Bowenoid papulosis. We retrospectively evaluated the data of all cases with histopathological confirmation of Bowen's disease and Bowenoid papulosis between 2006 and 2017 in our Dermatovenereology and/or Pathology departments. Secondary localized cutaneous amyloidosis was observed in three patients with Bowen's disease (3/52; 5.8%) and in three patients with Bowenoid papulosis (3/18; 16.7%). Herein, we present the demographic, clinical and histopathological features of these six cases of secondary localized cutaneous amyloidosis in detail. Although the occurrence of secondary localized cutaneous amyloidosis in epithelial tumors is a well-known phenomenon, its incidence has not been previously reported in Bowen's disease and Bowenoid papulosis. Therefore, our results indicating a high incidence may be particularly important for Bowenoid papulosis, as its association with secondary localized cutaneous amyloidosis has only been shown in one case before. Moreover, in three of six cases, we histologically observed areas of regression with a marked prominence of amyloid deposition. Remarkably, two of these patients had a history of topical application of destructive agents which reveals a possible etiologic relationship between secondary localized cutaneous amyloidosis and cellular apoptosis/necrosis induced by these external agents. [ABSTRACT FROM AUTHOR]

Nodular localized cutaneous amyloidosis is a rare form of cutaneous amyloidosis and is characterized by an extracellular deposition of insoluble amyloid fibrils which are either primarily cutaneous or a manifestation of an underlying systemic amyloidosis. Biopsy of the lesion is mandatory for the diagnosis, and histopathology shows diffuse amyloid deposits with plasmacytic infiltration. Apple-green birefringence characteristic of amyloidosis is observed when stained with Congo red and viewed under polarized light. Amyloid subtyping is done with laser microdissection followed by mass spectrometry. Majority of these lesions do not require any treatment but surgical excision, shave excision, laser therapy, and radiotherapy can be considered for symptomatic nodular localized primary cutaneous amyloidosis (NLPCA). We present a case of recurrent NLPCA in a 64-year-old woman who was treated with bortezomib and dexamethasone after failing several local therapies with excellent response. [ABSTRACT FROM AUTHOR]

Amyloid is a protein derived from at least 20 different substances. Once misfolded, it results in a group of cutaneous and systemic conditions. Primary localized cutaneous amyloidosis of keratinocyte origin is a very common subtype that can manifest either as lichen or macular amyloidosis, lacking systemic involvement. Lichen amyloidosis often presents as multiple hyperpigmented papules on the lower extremities whereas macular amyloidosis is classically characterized by dark brown rippled macules on the interscapular area. Review of the literature reveals that in addition to the classical presentation of primary localized cutaneous amyloidosis there exists a plethora of various manifestations that can be grouped into either geographic or morphologic categories. This review provides clinicians with the intimate knowledge of these presentations and summarizes the available treatment modalities. [ABSTRACT FROM AUTHOR]

Nodular cutaneous amyloidosis represents the rarest variant of primary localized cutaneous amyloidosis. The proposed management ranges from topical or systemic agents to surgical treatment. Complete surgical excision is advisable due to its potential progression to systemic amyloidosis due to dermis and subcutaneous tissue infiltration. However, in particular locations, the risk of functional complications is high, so an alternative treatment option should be considered. We report a case of a large primary nodular cutaneous amyloidosis of the leg involving the joint capsule which was successfully treated by incomplete surgical removal, without recurrences at 7-year follow-up. [ABSTRACT FROM AUTHOR]

The article describes the case of an 80-year-old hypertensive Taiwanese male who presented with chronic scalp pruritus. Physical examination showed a bald scalp with overlying multiple hyperkeratotic and scaly papules and plaques in the inflamed background. Clinical presentation and histological findings led to a diagnosis of poikiloderma-like primary cutaneous amyloidosis in the scalp. The patient was treated with potent topical steroid and cryotherapy.

The article focuses on the Libre 2 acts as a continuous glucose monitor (CGM) rather than a flash monitor and continuously sends readings to the reader by Bluetooth. It mentions that Medtronic has launched their new hybrid closed loop system. It also mentions that people with a higher level of exercise had a lower risk of dying during the study period.

Background: Localized nodular deposits of AL amyloid are seen in different tissues/organs; however, the pathogenesis of this form of amyloidosis remains unclear. Recently, Sjögren syndrome combined with localized nodular AL amyloidosis has been noted. Here, we report Sjögren syndrome cases showing multifocal nodular AL amyloidosis and the followed benign course. Materials and methods: We investigated the clinical pictures and histopathological findings of three cases with both presence of Sjögren syndrome and localized nodular AL amyloidosis, paying a special attention to the distribution of amyloidoma. Results: All three cases were middle-aged females. In two of three cases localized deposits of AL amyloid preceded Sjögren syndrome. Amyloidoma was detected in scalp, eyelid, cheek, larynx, trachea, lung and breast, and around these amyloid-deposited lesions infiltration of plasma cells was seen. Pulmonary amyloidosis was consistently accompanied with parenchymal cystic lesions, but this amyloidosis did not produce any significant respiratory symptoms. Some of large pulmonary amyloidomas showed cavity formation and subsequent shrinkage. In two cases amyloid deposition was found on gastric mucosa. Two cases received small doses of oral prednisone, with no further appearance of amyloidoma. Conclusion: Sjögren syndrome-related plasma cell disorder may be responsible for the formation of this unique multifocal nodular AL amyloidosis. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Lichen amyloidosis is the most common form of primary cutaneous amyloidosis. It is characterized by the presence of hyperkeratotic, pruritic and persistent papules, which may coalesce forming red brownish plaques, found mostly on the extensor surface of the extremities. Histopathological findings are represented by the deposits of insoluble amyloid fibres in the papillary dermis. We describe the case of a 47 year-old female patient, who was admitted to our clinic for the presence of infiltrated patches and plaques, composed of light brown, grouped papules, involving the anterior and posterior trunk, arms and thighs, of 15 years duration. The diagnosis of lichen amyloidosis was established based on clinical and histopathological examination. We aim to discuss the main clinical, paraclinical and treatment aspects in lichen amyloidosis. [ABSTRACT FROM AUTHOR]

Background: Waldenstrom macroglobulinemia is a lymphoplasmacytic lymphoma with elevated serum immunoglobulin M and multi-organ involvement. Primary systemic amyloidosis usually develops due to immunoglobulin light chains depositions in different organs due to an underlying gammopathy. Case presentation: Our patient was an 86-year-old man with macroglossia, ecchymotic patches and bullous lesions associated with a skin laxity on the periorbital, palmar, and glans penis areas. Skin biopsy confirmed dermal amyloid depositions. In serum immunofixation electrophoresis, prominent monoclonal immunoglobulin-M lambda light chains were detected associated with prominent lymphoplasmacytic infiltration in bone marrow biopsy which was diagnosed as Waldenstrom macroglobulinemia. Conclusion: Skin involvement presenting as cutaneous amyloidosis could be the first manifestation of Waldenstrom macroglobulinemia. We should think about an underlying gammopathy in an old patient with skin laxity and ecchymosis. [ABSTRACT FROM AUTHOR]

Aims: Interobserver reliability of histopathological features in differentiation between cutaneous polyarteritis nodosa (cPAN) and superficial thrombophlebitis (ST) by assessment of inter‐rater agreement of five histological features was investigated. Methods and results: All sections of cPAN and ST were evaluated independently by three experienced pathologists and one resident of pathology. The histopathological features studied included elastic fibre distribution in the vascular wall, a smooth muscle arrangement pattern, an internal elastic lamina pattern, fibrinoid necrosis and luminal thrombosis. Agreement analysis was performed using the kappa coefficient. Sensitivity, specificity, positive predictive value (PPV), positive likelihood ratio (PLR) and 95% confidence interval (95% CI) of the useful histopathological features were analysed. Of all 62 biopsies, 28 were cPAN and 34 were ST. Reproducibility between four observers was in substantial agreement (κ = 0.73). Elastic fibre distribution in the vascular wall (κ = 0.68), fibrinoid necrosis (κ = 0.63), an internal elastic lamina pattern (κ = 0.51) and a smooth muscle arrangement pattern (κ = 0.46) showed high specificity and PPV for differentiating between cPAN and ST. The smooth muscle arrangement pattern, internal elastic lamina pattern and elastic fibre distribution in the vascular wall may be obscured when extensive inflammation and necrosis occurs. Conclusions: These aforementioned histopathological features are useful in differentiation between cPAN and ST. The Verhoeff–van Gieson (VVG) elastic stain is an important histochemical study for differentiating between cPAN and ST, particularly in cases with extensive inflammation and necrosis. [ABSTRACT FROM AUTHOR]

Amyloidosis cutis dyschromica (ACD) is a distinct form of primary cutaneous amyloidosis characterized by generalized hyperpigmentation mottled with small hypopigmented macules on the trunks and limbs. Affected families and sporadic case subjects have been reported predominantly in East and Southeast Asian ethnicities; however, the genetic cause has not been elucidated. We report here that the compound heterozygosity or homozygosity of GPNMB truncating alleles is the cause of autosomal-recessive ACD. Six nonsense or frameshift mutations were identified in nine individuals diagnosed with ACD. Immunofluorescence analysis of skin biopsies showed that GPNMB is expressed in all epidermal cells, with the highest staining observed in melanocytes. GPNMB staining is significantly reduced in the lesional skin of affected individuals. Hyperpigmented lesions exhibited significantly increased amounts of DNA/keratin-positive amyloid deposits in the papillary dermis and infiltrating macrophages compared with hypo- or depigmented macules. Depigmentation of the lesions was attributable to loss of melanocytes. Intracytoplasmic fibrillary aggregates were observed in keratinocytes scattered in the lesional epidermis. Thus, our analysis indicates that loss of GPNMB, which has been implicated in melanosome formation, autophagy, phagocytosis, tissue repair, and negative regulation of inflammation, underlies autosomal-recessive ACD and provides insights into the etiology of amyloidosis and pigment dyschromia. [ABSTRACT FROM AUTHOR]

Abstract: Cutaneous pigmented viral plaques is a disorder of epidermal growth caused by canine papillomavirus type 4 (CPV‐4). There is currently no standard of care for managing this condition and it has not been reported in the Hungarian Vizsla. This case series documents the clinical features of canine pigmented viral plaques in Hungarian Vizsla dogs and the treatment of a severe case using a novel topical agent tigilanol tiglate (EBC‐46). A 4‐year‐old spayed Hungarian Vizsla in Australia was presented for multiple cutaneous pigmented plaques extending from the ventral cervical region. Lesions were neither painful nor pruritic. The number and size of these sessile plaques increased over time, with the largest lesions eventually taking on an exophytic (wart‐like) appearance. These lesions did not affect the dog's wellbeing. Two much less severe cases in a 5‐year‐old Vizsla from the UK and a 7‐year‐old Vizsla from New Zealand were also diagnosed. Histology was consistent with papillomavirus‐induced pigmented plaques and CPV‐4 DNA sequences were amplified from paraffin‐embedded formalin‐fixed tissue using the polymerase chain reaction from the most severely affected patient. Topical imiquimod was ineffective although used for only a short time. Two topical applications of novel anti‐neoplastic diterpene ester tigilanol tiglate as a gel, 9 days apart, greatly reduced the size and number of lesions in a limited portion of skin treated, over the lateral hock. While CPV‐4 has been previously reported to cause pigmented plaques, most commonly on pug dogs, but sporadically on other breeds, this is the first report of this virus causing plaques in Hungarian Vizslas. The cases illustrate some of the difficulties in diagnosing papillomavirus‐induced disease in dogs, especially in its early stages. Topical tigilanol tiglate is a potentially useful topical therapy for this viral‐induced disorder of cell growth and represents a treatment deserving of further investigation. [ABSTRACT FROM AUTHOR]

Background: Histoplasma capsulatum is a dimorphic fungus predominately found in soils enriched with bird and bat excreta. Although several cases of histoplasmosis have been reported in India, diagnosis using cytology has been done in very few cases.Case Presentation: We report here a case of disseminated histoplasmosis in a 46-year-old Indian man.Conclusion: Skin scrape cytology is a simple, safe, and rapid technique to establish the initial diagnosis, thus promoting early treatment and favorable outcome, in cutaneous fungal infections. [ABSTRACT FROM AUTHOR]

Primary cutaneous amyloidosis (PCA) is a form of localized amyloidosis. It is characterized by the deposition of a fibrillar material in the superficial dermis, without affecting other systems or organs. The diagnosis can be made clinically, but usually a skin biopsy is performed in order to exclude other skin diseases with similar appearance. Reflectance confocal microscopy (RCM) is a novel imaging tool that enables in vivo characterization of various skin changes with a high, quasi-microscopic resolution. This technique might have an important role in the differential diagnosis of cutaneous amyloidosis, by the in vivo assessment of epidermal changes and dermal amyloid deposition. Moreover, it is completely non-invasive and can be safely repeated on the same skin area. However, to date, there is only one published paper presenting the confocal features of primary cutaneous amyloidosis. Hereby, we describe the in vivo RCM features of PCA lesions from a patient with diabetes and correlate them with histologic findings. This strengthens the clinical usefulness of in vivo RCM examination for the non-invasive diagnosis of cutaneous amyloidosis, especially in patients that might associate diseases with impaired wound healing.

Background: Primary localized cutaneous amyloidosis (PLCA) is characterized by extracellular deposition of heterogenic amyloid proteins in the skin without systemic involvement. Lichen amyloidosis, macular amyloidosis, and (primary localized cutaneous) nodular amyloidosis are different subtypes of PLCA. Objective: The aim of this study was to review the current reported treatment options for PLCA. Methods: This systematic review was based on a search in the PubMed database for English and German articles from 1985 to 2016. Results: Reports on the treatment of PLCA were limited predominantly to case reports or small case series. There were a few clinical trials but these lacked control groups. A variety of treatment options for PLCA were reported including retinoids, corticosteroids, cyclophosphamide, cyclosporine, amitriptyline, colchicine, cepharanthin, tacrolimus, dimethyl sulfoxide, vitamin D analogs, capsaicin, menthol, hydrocolloid dressings, surgical modalities, laser treatment, and phototherapy. Conclusion: No definitive recommendation of preferable treatment procedures can be made based on the analyzed literature. Randomized controlled trials are needed to offer patients an evidence-based therapy with high-quality standardized treatment regimens for PLCA. [ABSTRACT FROM AUTHOR]

Background: Leishmaniasis manifests as visceral (VL), cutaneous (CL) or a dermal sequel of VL, known as Post kala-azar dermal leishmaniasis (PKDL). The aim of the study was to analyze the clinical and laboratory features of cases diagnosed with leishmaniasis. Methods: This hospital-based retrospective study included all cases of VL, PKDL, and CL diagnosed between Jan 2011 to Jan 2016 at All India Institute of Medical Sciences, New Delhi. Clinical and laboratory profile of the diagnosed cases were analyzed in detail. All diagnosed cases were mapped according to the state and the district from which the cases originated. Results: A total of 91 VL cases and 4 PKDL cases were reviewed. Only one case of CL (1 female) and mucocutaneous leishmaniasis (1 female) were observed during the study period. Majority of the cases of VL (75/91) originated from Bihar. The most common presenting symptoms in all our patients were fever (97.8%), weight loss (40.6%) and abdominal discomfort (17.6%) while the most common presenting signs were hepatosplenomegaly (45.8%), isolated splenomegaly (23.1%) and skin pigmentation (11%). The most common laboratory abnormality was anaemia followed by thrombocytopenia and leucopenia. Conclusion: VL is globally recognized as a neglected tropical disease. Even after continued effort to bring down its transmission in India, it continues to affect the endemic states with reports from new pockets. [ABSTRACT FROM AUTHOR]

Soft tissue amyloidoma is a rare condition that presents primarily in the abdomen and/or mediastinum and more uncommonly on the extremities. Soft tissue amyloidomas on the extremities have been associated with chronic inflammation, particularly when accompanied by AA-type amyloid deposition as seen in local trauma, surgery, hypertension and diabetes. To our knowledge, this is the first reported case of nodular cutaneous amyloidoma in the setting of systemic and cutaneous sarcoidosis. A 65-year-old woman presented with an asymptomatic subcutaneous nodule above her left lateral malleolus. Histopathology of the lesion showed an inconspicuous epidermis with amorphous eosinophilic material deposited in masses within the entire dermis. Congo red and crystal violet stains were positive. Based on the clinical and pathologic findings she was diagnosed with nodular cutaneous amyloidoma. We hypothesize that this process developed secondary to the chronic granulomatous inflammation of sarcoidosis. [ABSTRACT FROM AUTHOR]

Introduction: Primary Localized Cutaneous Amyloidosis (PLCA) is a relatively rare chronic condition characterized by amyloid deposition in dermis without associated deposits in internal organs. Histopathology of cutaneous amyloidosis using Haematoxylin and Eosin (H&E) stain shows eosinophilic hyaline material in papillary dermis, which can be further confirmed by Congo Red (CR) staining or Direct Immunofluorescence (DIF) Test or immunohistochemistry. Aim: To assess the concordance between the clinical, histopathological and DIF findings in various subtypes of (PLCA). Materials and Methods: Data was collected from patients attend ing the Outpatient Department (OPD) at a tertiary care centre in Karnataka, India, over a period of one and half years. A total of 50 patients with clinical features suggestive of cutaneous amyloidosis were subjected to histopathological examination with H&E, CR stain and immunofluorescence. Results: Among 50 clinically suspected patients, the most common subtype was macular amyloidosis (70%) and lichen amyloidosis seen only in 16%. A biphasic pattern comprising of both macular and lichen amyloidosis was seen in 14% cases. Extensor aspect of the arm was the most frequently (76%) involved area. All the cases had multiple site involvement. Immunofluorescence positivity was 88% as compared to 86% on histopathology using CR stain. Amyloid deposits were detected in 80% of clinically diagnosed macular amyloidosis cases by histopathology using CR stain and in 85.7% by DIF, whereas in 5.7% cases, it was not detectable by both CR stain and DIF. Both immunofluorescence and CR staining were able to detect amyloid in all the cases of lichen amyloidosis. In biphasic amyloidosis, amyloid was detected in 100% cases on histopathology versus 85.7% cases on immunofluorescence. Conclusion: CR stain and DIF are complimentary to each other for detection of macular amyloidosis. In case of lichen and biphasic amyloidosis, both CR and DIF are comparable modalities. [ABSTRACT FROM AUTHOR]