primary localized cutaneous amyloidosis 2

Primary Cutaneous Amyloidosis (PCA) Description

Primary cutaneous amyloidosis (PCA) encompasses a group of skin conditions characterized by the deposition of amyloid protein in the skin, without internal organ involvement. This condition is also known as primary localized cutaneous amyloidosis (PLCA).

Key Features:

• Deposition of amyloid protein in the skin
• No systemic involvement or internal organ involvement
• Three main forms of PLCA:
  • Macular
  • Lichen (papular)
  • Nodular (the rarest form)

References:

• [2] Primary cutaneous amyloidosis (PCA) encompasses a group of skin conditions characterised by deposition of amyloid protein, without internal organ ...
• [4] Primary localised cutaneous amyloidosis (PLCA) mainly affects the skin. There are three main forms of PLCA: macular, lichen (papular) and nodular (the rarest ...

Symptoms of Primary Localized Cutaneous Amyloidosis

Primary localized cutaneous amyloidosis (PLCA) is a rare condition characterized by the deposition of abnormal proteins called amyloids in the skin. The symptoms of PLCA can vary from person to person, but here are some common signs and symptoms:

• Skin lesions: The primary feature of PLCA is patches of skin with abnormal texture or color. These lesions can be firm, pink to brown or red nodules that may coalesce.
• Itching (pruritus): Some people with PLCA experience itching in the affected areas.
• Asymptomatic: In some cases, the lesions may be asymptomatic, meaning they do not cause any discomfort or pain.

According to a study published in 2011 [9], the most common form of primary cutaneous amyloidosis is lichen amyloidosus, which presents with small discrete, often pruritic waxy papules typically on the skin. Another study found that NLCA lesions are usually asymptomatic and may vary from a few millimeters to a few centimeters in size [8].

It's worth noting that primary localized cutaneous amyloidosis is a relatively rare condition, and more research is needed to determine its exact prevalence and characteristics.

References: [8] Jun 17, 2024 — NLCA lesions usually are asymptomatic. The firm, pink to brown or red nodules vary from a few millimeters to a few centimeters and may coalesce. [9] by DY Lee · 2011 · Cited by 25 — Primary localized cutaneous amyloidosis is a metabolic disease of the skin that is characterized by extracellular deposition of amyloid proteins in the skin ...

Diagnostic Tests for Primary Localized Cutaneous Amyloidosis

Primary localized cutaneous amyloidosis (PLCA) can be diagnosed through various tests, including:

• Skin biopsy: A skin sample is taken and examined under a microscope to check for the presence of amyloid deposits. This is considered the definitive diagnostic test [4].
• Gene rearrangement studies: These studies can help identify specific genetic mutations associated with PLCA.
• Blood and urine tests: These may be carried out to rule out systemic manifestations of amyloidosis, such as proteinuria (excess protein in the urine) [2].

Other Diagnostic Methods

In some cases, additional diagnostic methods may be used, including:

• Total body computed tomography scan: This can help identify any systemic manifestations of amyloidosis.
• Laboratory results: These can also help rule out systemic manifestations of amyloidosis.

It's worth noting that the differential diagnosis for systemic amyloidoses depends on the syndrome, but AL amyloidosis should always be considered in the differential diagnosis of PLCA [9].

References: [1] Not applicable [2] Jun 17, 2024 - Urinalysis or 24-hour urine testing can be performed to check for protein. [3] Not applicable [4] Jun 17, 2024 - The definitive diagnostic test is skin ... [5] Pathologists can do special tests on the skin sample to check for the ... [6] Sep 15, 2021 - Objectives: To identify subtypes, demographic and clinical features and treatment efficacy in patients with histopathologically confirmed PLCA. [7] Not applicable [8] Not applicable [9] The differential diagnosis for systemic amyloidoses depends on the syndrome, but AL amyloidosis should always be considered in the differential diagnosis of ...

Treatment Options for Primary Localized Cutaneous Amyloidosis

Primary localized cutaneous amyloidosis (PLCA) can be effectively treated with various medications, including:

• Cyclophosphamide: This medication has been shown to be effective in treating PLCA, particularly in reducing the size and number of lesions. A study involving 36 patients found that most patients experienced marked improvement after taking cyclophosphamide for six months [3].
• High-potency topical corticosteroids: These creams or ointments can help reduce inflammation and itching associated with PLCA.
• UVA1 phototherapy: This treatment involves exposure to UVA light, which can help reduce the size and number of lesions.

Other medications that may be used to treat PLCA include:

• Acitretin: A systemic retinoid that can help reduce the production of amyloid proteins.
• Calcipotriene: A topical medication that can help reduce inflammation and itching.
• Menthol: A topical medication that can help reduce itching.

It's essential to note that while these medications can be effective, they may not completely eliminate the condition. In some cases, a combination of treatments may be necessary to achieve optimal results [7].

References:

[3] Thirty-six patients of primary localised cutaneous amyloidosis were treated with cyclophosphomide 50 mg. tablets orally daily for a period of six months. Most of the patients following the therapy showed marked improvement in respect of itching, pigmentation and sizes of the popular lesions. [7] Topical therapies for PLCA include high potency steroids, calcipotriene, and menthol (4). Systemic therapies include acitretin, cyclophosphamide and ...

Differential Diagnoses for Primary Localized Cutaneous Amyloidosis

Primary localized cutaneous amyloidosis (PLCA) is a condition in which clumps of abnormal proteins called amyloids build up in the skin. When considering the differential diagnosis for PLCA, several conditions should be taken into account.

• Acalculous Cholecystopathy: This is a rare condition characterized by inflammation of the gallbladder without any visible stones or other obstructions.
• Colloid Milium: A benign condition that presents as small, yellowish or whitish papules on the skin, often on the face and neck.
• Cutaneous Pseudolymphoma: A type of skin cancer that mimics lymphoma, characterized by abnormal growths in the skin.
• Leiomyoma: A rare tumor that arises from smooth muscle cells, which can occur anywhere in the body but is most commonly found on the skin.

These conditions should be considered as part of the differential diagnosis for primary localized cutaneous amyloidosis. It's essential to consult with a healthcare professional for an accurate diagnosis and treatment plan.

References: * [3] Primary localized cutaneous amyloidosis (PLCA) is a condition in which clumps of abnormal proteins called amyloids build up in the skin. * [2] Jun 17, 2024 — Differential Diagnoses · Acalculous Cholecystopathy · Colloid Milium · Cutaneous Pseudolymphoma · Leiomyoma.