Amyloidosis - 아밀로이드증
아밀로이드증 (Amyloidosis)의 고전적인 얼굴 특징
References
26세 여성이 10년 전부터 다리에 가려운 발진이 생겼다고 주소로 내원했습니다. 스테로이드 크림과 타자로틴 크림을 사용해도 발진은 호전되지 않았습니다. 그녀는 관련 가족력이 없었습니다. 우리가 그녀를 검사했을 때, 우리는 그녀의 다리 앞부분에 lichen amyloidosis 이라는 질병과 일치하는 융기되고 거친 반점을 발견했습니다.
A 26-year-old woman presented to our clinic with an itchy rash on her legs that had persisted for 10 years. The rash had previously been treated with topical steroids and tazarotene cream, with no improvement. The patient’s family history was noncontributory. A physical examination showed discrete and coalescing hyperkeratotic tan-brown papules on the pretibial surfaces, consistent with lichen amyloidosis.
A 26-year-old woman presented to our clinic with an itchy rash on her legs that had persisted for 10 years. The rash had previously been treated with topical steroids and tazarotene cream, with no improvement. The patient’s family history was noncontributory. A physical examination showed discrete and coalescing hyperkeratotic tan-brown papules on the pretibial surfaces, consistent with lichen amyloidosis.
Lichen Amyloidosis 은 원인을 알 수 없는 지속적인 가려움증과 관련된 드문 피부 상태입니다. 이는 일반적으로 피부 외부 표면에 융기된 변색된 반점 덩어리로 나타납니다. Lichen Amyloidosis 은 주로 50~60세에 나타나며 안타깝게도 치료법은 없습니다. 현재 이용 가능한 치료법은 일반적으로 잘 작동하지 않습니다.
Lichen Amyloidosis (LA) is an uncommon, primary cutaneous amyloidosis associated with chronic, idiopathic pruritus. Clinical presentation of LA includes skin colored to hyperpigmented, papules coalescing into plaques with a rippled appearance on the extensors.1 LA most commonly presents in the fifth to sixth decade of life and has no curative treatments. Overall response to current therapies is poor.
Lichen Amyloidosis (LA) is an uncommon, primary cutaneous amyloidosis associated with chronic, idiopathic pruritus. Clinical presentation of LA includes skin colored to hyperpigmented, papules coalescing into plaques with a rippled appearance on the extensors.1 LA most commonly presents in the fifth to sixth decade of life and has no curative treatments. Overall response to current therapies is poor.
Lichen amyloidosis 은 오래 지속되는 가려운 피부 상태입니다. 주로 등, 정강이, 허벅지, 팔에서 발견되는 두꺼워진 돌기 집단으로 알려져 있습니다. 현미경으로 검사하면 Lichen amyloidosis 은 피부 표피층에 아밀로이드가 축적되고 피부 외층이 두꺼워지고 커지는 것을 보여줍니다. Lichen amyloidosis 의 정확한 원인은 아직 완전히 이해되지 않았지만 이전 연구에서는 피부 마찰이나 마찰, 세포 사멸, 바이러스 감염 등과 같은 요인과 관련이 있었습니다. Lichen amyloidosis 은 atopic dermatitis, lichen planus, and mycosis fungoides 과 같은 여러 다른 피부 질환과 연관이 있는 것으로 보입니다.
Lichen amyloidosis (LA) is a chronic pruritic skin disorder characterized by multiple grouped hyperkeratotic papules, predominantly located on the back, shins, thighs, and arms. Histological analysis of LA shows amyloid deposition in the papillary dermis and hyperkeratosis and acanthosis of the epidermis. The exact pathogenesis of LA has not yet been elucidated; however, prior reports have implicated frictional epidermal damage, apoptosis, viral infection, and many other triggers. LA is reportedly associated with several skin disorders, including atopic dermatitis (AD), lichen planus, and mycosis fungoides.
Lichen amyloidosis (LA) is a chronic pruritic skin disorder characterized by multiple grouped hyperkeratotic papules, predominantly located on the back, shins, thighs, and arms. Histological analysis of LA shows amyloid deposition in the papillary dermis and hyperkeratosis and acanthosis of the epidermis. The exact pathogenesis of LA has not yet been elucidated; however, prior reports have implicated frictional epidermal damage, apoptosis, viral infection, and many other triggers. LA is reportedly associated with several skin disorders, including atopic dermatitis (AD), lichen planus, and mycosis fungoides.
○ 진단 및 치료
#Electrophoresis of blood or urine
#Skin biopsy