Primary localized cutaneous amyloidosis
Primary Localized Cutaneous Amyloidosis[edit | edit source]
Primary Localized Cutaneous Amyloidosis (PLCA) is a rare skin condition characterized by the deposition of amyloid proteins in the skin without systemic involvement. This condition is part of a group of disorders known as cutaneous amyloidosis, which are distinguished by the presence of amyloid deposits in the skin.
Types of Primary Localized Cutaneous Amyloidosis[edit | edit source]
PLCA can be classified into several types based on clinical presentation:
- Lichen Amyloidosis: This is the most common form of PLCA, characterized by pruritic, hyperkeratotic papules, usually on the shins.
- Macular Amyloidosis: This type presents as hyperpigmented macules, often on the upper back, and is less pruritic than lichen amyloidosis.
- Nodular Amyloidosis: A rarer form, presenting as nodules, which can occur anywhere on the body.
Pathophysiology[edit | edit source]
The exact pathogenesis of PLCA is not fully understood. However, it is believed to involve the deposition of amyloid derived from keratinocyte-derived proteins. The amyloid deposits are localized to the papillary dermis and are thought to result from chronic friction or trauma to the skin.
Clinical Features[edit | edit source]
Patients with PLCA typically present with skin lesions that are pruritic and can be cosmetically concerning. The lesions are usually persistent and can be exacerbated by scratching or rubbing.
Diagnosis[edit | edit source]
Diagnosis of PLCA is primarily clinical, supported by histopathological examination. A skin biopsy will reveal amyloid deposits in the dermis, which can be confirmed by special staining techniques such as Congo red staining, which shows apple-green birefringence under polarized light.
Treatment[edit | edit source]
Treatment options for PLCA are limited and primarily aimed at symptom relief. These may include:
- Topical corticosteroids to reduce inflammation and pruritus.
- Antihistamines to alleviate itching.
- Laser therapy or dermabrasion for cosmetic improvement.
Prognosis[edit | edit source]
PLCA is a chronic condition with no known cure. However, it is not life-threatening and does not progress to systemic amyloidosis. Management focuses on controlling symptoms and improving quality of life.
Epidemiology[edit | edit source]
PLCA is more common in certain populations, such as those of Asian and South American descent. It can occur at any age but is most frequently seen in middle-aged adults.
See Also[edit | edit source]
==
NIH genetic and rare disease info[edit source]
Primary localized cutaneous amyloidosis is a rare disease.
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Contributors: Prab R. Tumpati, MD
