Related Experiment Video
Updated: Jan 30, 2026
06:33
Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
Published on: June 9, 2018
8.1K
Nodular amyloidosis in a patient with systemic scleroderma.
Anne L Marano1, M Angelica Selim, Adela R Cardones
1Duke University Medical Center, Department of Dermatology, Durham, North Carolina. Anne.marano@duke.edu.
Dermatology Online Journal
|January 26, 2019
Summary
Primary cutaneous nodular amyloidosis, a rare skin condition, developed in a patient with systemic scleroderma. Diagnosis involved advanced proteomic analysis, and the patient showed symptomatic improvement with wound care.
Area of Science:
- Dermatology
- Immunopathology
- Proteomics
Background:
- Primary cutaneous amyloidosis presents as macular, lichenoid, or nodular forms.
- Nodular amyloidosis involves immunoglobulin light chain deposition and can rarely be linked to systemic disease.
Observation:
- A patient with systemic scleroderma developed primary cutaneous nodular amyloidosis on the lower leg.
- Diagnosis was confirmed via skin biopsy, Congo red staining, and laser microdissection with mass spectrometry-based proteomic analysis.
Findings:
- The patient's work-up for systemic amyloidosis was negative.
- Symptomatic improvement was achieved with wound care.
Implications:
- This case highlights an unusual presentation of nodular amyloidosis in scleroderma.
- Clinical monitoring for systemic amyloidosis is advised for patients with primary cutaneous nodular amyloidosis, though the risk appears low.
Related Concept Videos
01:18Second Order systems II
406
In an underdamped second-order system, where the damping ratio ζ is between 0 and 1, a unit-step input results in a transfer function that, when transformed using the inverse Laplace method, reveals the output response. The output exhibits a damped sinusoidal oscillation, and the difference between the input and output is termed the error signal. This error signal also demonstrates damped oscillatory behavior. Eventually, as the system reaches a steady state, the error diminishes to zero.
406
01:21First Order Systems
427
First-order systems, such as RC circuits, are foundational in understanding dynamic systems due to their straightforward input-output relationship. Analyzing their responses to different input functions under zero initial conditions reveals significant insights into system behavior.
When a first-order system is subjected to a unit-step input, its response is characterized by its transfer function. By applying the Laplace transform of the unit-step input to the transfer function, expanding the...
When a first-order system is subjected to a unit-step input, its response is characterized by its transfer function. By applying the Laplace transform of the unit-step input to the transfer function, expanding the...
427
01:20Second Order systems I
598
A servo system exemplifies a second-order system, featuring a proportional controller and load elements that ensure the output position aligns with the input position. The relationship between these components is described by a second-order differential equation. Applying the Laplace transform under zero initial conditions yields the transfer function, showing how inputs are converted to outputs in the system.
By reinterpreting the system, one can derive the closed-loop transfer function, which...
By reinterpreting the system, one can derive the closed-loop transfer function, which...
598
01:06Thermodynamic Systems
8.0K
A thermodynamic system is a set of objects whose thermodynamic properties are of interest. The system is considered to be embedded in its surroundings or the environment. The system and its environment can exchange heat and do work on each other through a boundary that separates them. However, the immediate surroundings of the system interact with it directly and therefore have a much stronger influence on its behavior and properties.
Consider an example of tea boiling in a kettle. The...
Consider an example of tea boiling in a kettle. The...
8.0K
01:26Classification of Systems-I
592
Linearity is a system property characterized by a direct input-output relationship, combining homogeneity and additivity.
Homogeneity dictates that if an input x(t) is multiplied by a constant c, the output y(t) is multiplied by the same constant. Mathematically, this is expressed as:
Homogeneity dictates that if an input x(t) is multiplied by a constant c, the output y(t) is multiplied by the same constant. Mathematically, this is expressed as:
592
01:31Classification of Systems-II
501
Continuous-time systems have continuous input and output signals, with time measured continuously. These systems are generally defined by differential or algebraic equations. For instance, in an RC circuit, the relationship between input and output voltage is expressed through a differential equation derived from Ohm's law and the capacitor relation,
501