Presentation
It is easily diagnosed when presented in its typical manifestation. Atypical or rare clinical presentations can pose diagnostic difficulties. [ncbi.nlm.nih.gov]
Entire Body System
-
Recurrent Respiratory Infections
These included neonatal colitis, infantile diarrhea, recurrent respiratory infections, corneal dystrophy, photophobia, unruly hair with a frontal upsweep, dry skin, and mottled, muddy-brown pigmentation seen first on the inner thighs and spreading diffusely [ncbi.nlm.nih.gov]
-
Leg Edema
Side effects included fatigue, drowsiness, numbness, and facial and leg edema in some of the patients. From the present observation, it seems that thalidomide is a promising drug to treat PCA. 2013 Wiley Periodicals, Inc. [ncbi.nlm.nih.gov]
-
Renal Artery Stenosis
artery stenosis · Hypertensive nephropathy · Renovascular hypertension Other Analgesic nephropathy · Renal osteodystrophy · Nephroptosis · Abderhalden-Kaufmann-Lignac syndrome Ureter Ureteritis · Ureterocele · Megaureter Pelvic Bladder Cystitis ( Interstitial [wikidoc.org]
Gastrointestinal
-
Regurgitation
Echocardiography revealed left ventricular hypertrophy and aortic regurgitation. [medicaljournals.se]
Skin
-
Papule on the Upper Back
Detailed physical examination revealed dotted pigmented papules on the upper back (Fig. 1b). [medicaljournals.se]
Eyes
-
Photophobia
These included neonatal colitis, infantile diarrhea, recurrent respiratory infections, corneal dystrophy, photophobia, unruly hair with a frontal upsweep, dry skin, and mottled, muddy-brown pigmentation seen first on the inner thighs and spreading diffusely [ncbi.nlm.nih.gov]
Psychiatrical
-
Suggestibility
CONCLUSIONS: Both parametric and nonparametric linkage evidence suggested that a possible susceptibility locus for a subset of FPCA might exist on chromosome 1q23. [ncbi.nlm.nih.gov]
Neurologic
-
Paresthesia
Some of the more common findings during a review of systems include the following: weight loss, weakness/fatigue, dyspnea, edema, paresthesias, light-headedness, syncope, constipation/diarrhea, and hoarseness/change of voice. [dermatologyadvisor.com]
Workup
Other Pathologies
-
Lymphocytic Infiltrate
Histopathological features showed orthokeratosis, thinning of epidermis, basal cell vacuolar degeneration and diffuse mild degree of lymphocytic infiltration in the upper dermis with perivascular accentuation. [ijpmonline.org]
In the dermis, a dense lymphocytic infiltrate constituted the major finding (50%), followed by pigment incontinence (22.72%) and increased dermal collagen (18.18%). [pigmentinternational.com]
Hypertrophy
-
Ventricular Hypertrophy
Echocardiography revealed left ventricular hypertrophy and aortic regurgitation. [medicaljournals.se]
Treatment
[…] dressings, surgical modalities, laser treatment, and phototherapy. [ncbi.nlm.nih.gov]
A comprehensive review of the literature on the treatment options for PCA showed no controlled trials available. However, case series indicated phototherapy (UVB, narrow band UVB, photochemotherapy) as the most successful treatment. [clinicaldermatology.eu]
Prognosis
Primary systemic amyloidosis, which carries a poorer prognosis than does nodular amyloidosis, also may give rise to ecchymoses and many other cutaneous and extracutaneous findings. [ncbi.nlm.nih.gov]
Dialysis-related amyloidosis Pathophysiology Systemic Amyloidosis Organ-specific Amyloidosis Microscopic Pathology Causes Differentiating Amyloidosis from other Diseases Epidemiology and Demographics Risk Factors Screening Natural History, Complications and Prognosis [wikidoc.org]
Topics covered include classification/epidemiology, pathogenesis, clinical features (with classic images), diagnosis, prognosis and treatment. [dermatologyadvisor.com]
Nodular amyloidosis is extremely rare, and there are notable differences in clinical presentation, prognosis, histology, and pathogenesis between this entity and the macular and lichenoid variants. [actasdermo.org]
Etiology
Amyloidosis cutis dyschromica is mostly a congenital disorder with hypersensitivity to UVB and DNA repair defects due to repeated UVB damage as possible etiological factors. [e-ijd.org]
Herrera, Ahmet Dogan Humana Press, ١٧/٠٨/٢٠١٥ - 542 من الصفحات The second edition of this text presents an overview of the most recent developments in this area including clinical presentation, etiology, pathogenesis, and differential diagnosis. [books.google.com]
However, the etiology of amyloidosis remains unclear and population-based studies would prove to be of great benefit in evaluating the exact etiology of amyloidosis. [idoj.in]
Whether there exists an association with regard to female sex hormones in the etiology of PCA still requires elaboration. [pigmentinternational.com]
Its etiology is unknown but chronic irritation to the skin has been proposed as an etiological factor. [6] However, there are examples where in cutaneous amyloidosis was not associated with scratching and chronic irritation was not the causal factor. [ijpmonline.org]
Epidemiology
However, no large-scale epidemiological study of PCA and its associations with other diseases has been conducted so far. [ncbi.nlm.nih.gov]
The present study was an attempt to study the epidemiology of PLCA and the effectiveness of DMSO on the lesions. [idoj.in]
“The epidemiology of AL and AA amyloidosis”. Baillieres Clin Rheumatol. vol. 8. 1994. pp. 627-34. [dermatologyadvisor.com]
Epidemiology of primary cutaneous amyloidoses in southeast Asia. Clin Dermatol 1990;8:20-4. 13. Ollague W, Ollague J, Ferretti H. Epidemiology of primary cutaneous amyloidoses in South America. Clin Dermatol 1990;8:25-9. 14. [pigmentinternational.com]
In one epidemiological study, Chinese women were found to be predominantly affected and familial cases were not uncommon ( 9 – 11 ). [clinicaldermatology.eu]
Pathophysiology
We present the case of a patient with an autoimmune hepatitis-primary biliary cirrhosis overlap syndrome with concomitant cutaneous amyloidosis, a very unusual association, and discuss similar cases and possible pathophysiological implications. [ncbi.nlm.nih.gov]
Amyloidosis Main page Overview Historical Perspective Classification AL amyloidosis AA amyloidosis Heritable amyloidosis Age-related (senile) systemic amyloidosis Dialysis-related amyloidosis Pathophysiology Systemic Amyloidosis Organ-specific Amyloidosis [wikidoc.org]
Pathophysiology As a term, "amyloid" was used historically to define proteins that shared similar microscopic characteristics and affinity for certain stains. Research has revealed that "amyloid" proteins are heterogeneous. [emedicine.medscape.com]
Prevention
While this treatment can achieve therapeutic success, it cannot prevent complications from cirrhosis. This patient remains alive but experienced an emergent gastrointestinal hemorrhage. [ncbi.nlm.nih.gov]
Secondary Prevention Cost-Effectiveness of Therapy Future or Investigational Therapies v t e Urinary system · Pathology · Urologic disease / Uropathy ( N00-N39, 580-599 ) Abdominal Nephropathy / ( nephritis nephrosis ) Glomerulopathy / glomerulitis / [wikidoc.org]
Other treatments to help with symptoms include: Diuretic medicine to remove extra water from your body Thickeners to add to fluids to prevent choking if you have trouble swallowing Compression stockings to relieve swelling in your legs or feet Changes [webmd.com]