Amyloidosis - Amyloidose
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Klassiske ansigtstræk af Amyloidose (Amyloidosis)
References
En 26-årig kvinde kom til vores klinik og klagede over et kløende udslæt på hendes ben, som hun havde haft i 10 år. På trods af brug af steroidcremer og tazarotencreme blev udslættet ikke bedre. Hun havde ikke nogen relevant familiehistorie. Da vi undersøgte hende, fandt vi hævede, ru pletter på forsiden af hendes ben, som matchede en sygdom kaldet 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.
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 er en sjælden hudlidelse forbundet med vedvarende kløe af ukendt årsag. Det viser sig typisk som klynger af hævede, misfarvede pletter på hudens ydre overflader. Lichen Amyloidosis optræder normalt hos personer i alderen 50 til 60 år, og der er desværre ingen kur mod det. Behandlinger, der er tilgængelige nu, virker normalt ikke godt.
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 er en langvarig kløende hudtilstand. Det er kendt for klynger af fortykkede knopper, der hovedsageligt findes på ryggen, skinnebenene, lårene og armene. Når det undersøges under et mikroskop, viser Lichen amyloidosis en ophobning af amyloid i det øverste hudlag sammen med fortykkelse og forstørrelse af det ydre hudlag. Selvom den nøjagtige årsag til Lichen amyloidosis ikke er fuldt ud forstået endnu, har tidligere undersøgelser knyttet det til faktorer som gnidning eller friktion på huden, celledød, virusinfektioner, blandt andre. Lichen amyloidosis ser ud til at være forbundet med flere andre hudsygdomme såsom atopic dermatitis, 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.
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.
○ Diagnose og behandling
#Electrophoresis of blood or urine
#Skin biopsy