cutaneous amyloidosis

BACKGROUND: Lichenoid amyloidosis (LA) is a subtype of primary cutaneous amyloidosis characterized by persistent multiple groups of hyperkeratotic papules, usually on the lower leg, back, forearm, or thigh. LA may be associated with several skin diseases, including atopic dermatitis (AD). The treatment of LA is considered to be difficult. However, as there is some overlap in the etiopathogenesis of LA and AD, AD treatment may also be effective for LA. CASE SUMMARY: Case 1: A 70-year-old man was diagnosed with severe AD with LA based on large dark erythema and papules on the trunk and buttocks and dense hemispherical millet-shaped papules with pruritus on the extensor side of the lower limbs...

Primary localized cutaneous nodular amyloidosis (PLCNA) is a rare condition attributed to plasma cell proliferation and the deposition of immunoglobulin light chains in the skin without association with systemic amyloidosis or hematological dyscrasias. It is not uncommon for patients diagnosed with PLCNA to also suffer from other auto-immune connective tissue diseases, with Sjögren's syndrome (SjS) showing the strongest association. This article provides a literature review and descriptive analysis to better understand the unique relationship between these two entities...

BACKGROUND: Primary localized cutaneous amyloidosis (PLCA) is a chronic skin disease characterized by aberrant keratinocyte differentiation, epidermal hyperproliferation, and amyloid deposits. Previously, we demonstrated OSMR loss-function mutants enhanced basal keratinocyte differentiation through the OSMR/STAT5/KLF7 signaling in PLCA patients. OBJECTIVE: To investigate the underlying mechanisms involved in basal keratinocyte proliferation in PLCA patients that remain unclear...

No abstract text is available yet for this article.

Immunoglobulin G4-related disease (IgG4-RD) is a rare fibro-inflammatory condition characterized by IgG4-expressing plasma cell infiltration of the skin and other organs, leading to profound itchiness. Oral corticosteroids are the first-line therapy for IgG4-RD but relapses and potential side effects are common. In this case, we discuss a patient with a hyperpigmented, scaling dermatitis on his arms, back, and chest with lichen amyloidosis (LA) that incompletely responded to corticosteroids. He had reduced quality of life secondary to chronic pruritus...

The diagnosis and follow-up of peripheral neuropathies involving small-diameter nerve fibers require specific examinations beyond conventional nerve conduction studies which only concern large-diameter nerve fibers. Among these tests, some are dedicated to the investigation of cutaneous innervation by the autonomic nervous system, mainly by unmyelinated sympathetic C fibers. To this end, various laboratory tests have been proposed, but the measurement of electrochemical skin conductance (ESC) by Sudoscan® is increasingly becoming the most widely used technique, because it allows a quick and simple assessment of the sudomotor function of the limb extremities...

Trichoscopy is a diagnostic tool for hair and scalp diseases. It was recently shown that it also allows the identification of features associated with disorders that typically do not affect the scalp. The aim of this article was to analyse and outline the usefulness of trichoscopy in suspecting such diseases. Connective tissue diseases were the most investigated systemic disorders in regard to trichoscopy. The most common features of systemic lupus erythematosus, systemic sclerosis and dermatomyositis are thick arborizing and tortuous vessels...

No abstract text is available yet for this article.

A collision tumor is a neoplastic lesion comprised of two or more tumors consisting of distinct cell populations in the concurrent location. Multiple skin neoplasms at one site (MUSK IN A NEST) is a term recently coined to describe two or more cutaneous benign or malignant tumors occurring at the same anatomic site. In retrospective studies, seborrheic keratosis and cutaneous amyloidosis have both individually been documented as a component of a MUSK IN A NEST. This report describes a 42-year-old woman who presented with a pruritic skin condition on her arms and legs of 13 years' duration...

Amyloidosis cutis dyschromica is a rare variant of primary cutaneous amyloidosis characterized by hyper- and hypopigmented macules. In this paper, we reported a case of a 16-year-old Filipino girl with hyper- and hypopigmented to depigmented macules on the upper and lower extremities, which started when she was 9 years of age.

BACKGROUND: Apart 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 PRESENTATION: A 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...

Dimethyl sulfoxide (DMSO) is a clear, odorless liquid, inexpensively produced as a by-product of the wood pulp industry. DMSO's unique chemical properties allow for its broad applications in a wide variety of cutaneous challenges. Widely available in the USA as a solvent, DMSO is FDA-approved only for the treatment of interstitial cystitis and for use as a preservative for organ transplant. DMSO readily penetrates and diffuses through biological membranes. At low concentrations, DMSO exhibits anti-inflammatory, analgesic, diuretic, vasodilator, anti-platelet aggregation, radio-protective, and muscle-relaxing properties...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

Lichen amyloidosis is believed to be caused by damage to keratinocytes, often by chronic scratching. It has also been associated with autoimmune conditions, including thyroid disease. Dermatologic manifestations of poorly controlled thyroid disease are well described within the medical literature, within both hypothyroid and hyperthyroid states. Myxedema is a rare complication of Graves disease. We report a unique case of concurrent myxedema and lichen amyloidosis in a 63-year-old patient with uncontrolled hypothyroidism in the setting of post-ablative Graves disease...

BACKGROUND: Amyloidosis is a rare disease characterized by extracellular deposition of misfolded protein aggregated into insoluble fibrils. Gastrointestinal involvement in systemic amyloidosis is common, but is often subclinical or presents as vague and nonspecific symptoms. It is rare for gastrointestinal symptoms to be the main presenting symptom in patients with systemic amyloidosis, causing it to be undiagnosed until late-stage disease. CASE SUMMARY: A 53 year-old man with diarrhea, hematochezia, and weight loss presented to a community hospital...

Keratinic primary localized cutaneous amyloidosis is a disease in humans; however, no similar condition has been reported in animals. This study aimed to investigate cutaneous keratinic amyloid deposition in dogs and elucidate its etiology. Canine hair follicle tumor tissues were histopathologically analyzed. Immunohistochemistry and mass spectrometry-based proteomic analyses were performed to identify precursor protein candidates. Structural prediction and in vitro fibrillization analyses were conducted to determine the amyloidogenic region and gene sequencing analysis was performed to assess mutations...

No abstract text is available yet for this article.

Insulin-derived amyloidosis (AIns) is a rare iatrogenic subtype of cutaneous amyloidosis occurring at frequent insulin injection sites. Here, we describe 2 cases of AIns accompanied by acanthosis nigricans (AN)-like changes, a rare finding which has been reported fewer than 5 times in the literature. We also report the first case of an AIns nodule being misdiagnosed as a keloid. Both of our patients presented with asymptomatic, hyperkeratotic, pigmented plaques at frequent insulin injection sites, and histopathologic examination showed (1) nodular aggregates of amyloid demonstrating apple-green birefringence with Congo red staining and (2) AN-like features, such as epidermal papillomatosis, hyperkeratosis, and hyperpigmentation...

BACKGROUND: Transthyretin-mediated amyloidosis (ATTR) is a rare multisystemic disease involving the peripheral nervous system and heart. Autonomic and small fiber involvement is one of the hallmarks of ATTR, and many tools have been proposed to assess this aspect. AIM: The aim of this study was to investigate cutaneous and mixed nerve silent periods (CSP and MnSP) as instruments for small fiber assessment. METHODS: A total of 21 ATTR patients, 20 healthy controls, and 18 asymptomatic carriers underwent a sensory conduction study from the right sural and non-dominant ulnar nerves...