In the condition known as familial cutaneous amyloidosis, amyloid-containing abnormal protein clusters build up in the skin, particularly in the wave-like projections (dermal papillae) that divide the top two layers of skin (the dermis and the epidermis). Familial cutaneous amyloidosis is characterized by areas of skin that have an aberrant texture or color.
TYPES
Familial cutaneous amyloidosis is categorized into the following types based on its appearance and histology:
SYMPTOMS
Depending on the type you have, familial cutaneous amyloidosis symptoms can vary. A prominent feature of familial cutaneous amyloidosis is the presence of many tiny bumps and very irritating regions of thickened skin. The patches are reddish brown in hue and scaly. Usually seen on the shins, these patches may also be found on the forearms, other areas of the legs, and other portions of the body.
The flat, dark-brown spots are indicative of familial cutaneous amyloidosis. Although it is frequently uniform, the coloring can appear reticulated or rippled. Patches of amyloidosis may appear anywhere on the body, although they are most often detected on the upper back. These blotches have a little itching.
DIAGNOSIS
When there is an itch in usual places, a PCA subtype can be diagnosed based on its distinctive clinical features. On H&E-stained skin biopsy specimens, Congo-red-stained skin samples illuminated with polarized light (apple-green birefringence), and immunohistochemistry, amyloid deposits in the skin may be detected.
TREATMENT
There are no approved treatment plans since there aren’t enough controlled studies. Recalcitrance and recurrences occur with all treatments. The goal of treatment is to relieve pruritus and cosmetic deformity.
Topical treatment
Depending on the severity of the lesion and tolerability, intralesional corticosteroid injection or cryosurgery with or without topical steroids may be used.
Oral/systemic treatment
When topical therapies are ineffective or impracticable for large lesions, this medication may be administered (off-label):
Physical treatment
Overview and FactsTypes and SymptomsDiagnosis & MedicationsOverview and Facts Tricuspid atresia is a congenital heart defect characterized by the absence [...]
Overview and FactsTypes and SymptomsDiagnosis & MedicationsOverview and Facts Toxic megacolon is a rare but serious complication of inflammatory bowel [...]
Overview and FactsTypes and SymptomsDiagnosis & MedicationsOverview and Facts Toxic Epidermal Necrolysis is a rare but severe and potentially life-threatening [...]
