Last update 01 Nov 2024

Amyloidosis, Primary Cutaneous

Last update 01 Nov 2024

Basic Info

Synonyms

AD type amyloidosis, Amyloid lichen, Amyloidosis cutis

+ [35]

Introduction

A rare chronic form of cutaneous amyloidosis, a skin disease with characteristics of the accumulation of amyloid deposits in the dermis. Clinical manifestations include the development of pruritic, often pigmented hyperkeratotic papules on trunk and extremities, especially on the shins. Histological findings include the deposition of amyloid or amyloid-like proteins in the papillary dermis.

Drugs associated with Amyloidosis, Primary Cutaneous

Flurandrenolide

Target

Mechanism

GR agonists

Active Org.

Sumitomo Pharma Co., Ltd.

Originator Org.

Almirall SA

Active Indication

Amyloidosis, Primary Cutaneous [+12]

Inactive Indication

Inflammation [+1]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

First Approval Date19 Mar 1963

Clinical Trials associated with Amyloidosis, Primary Cutaneous

IRCT20240612062098N1 / RecruitingPhase 3IIT

Comparison of efficacy of carboxytherapy and topical fluocinolone acetonide 0.025% in treatment of macular amyloidosis

Start Date27 Aug 2024

Sponsor / Collaborator

Arak University of Medical Sciences

ChiCTR2300068837 / SuspendedPhase 1IIT

A clinical study of Tofactibu citrate in the treatment of primary cuntaneous amyloidosis

Start Date01 Mar 2023

Sponsor / Collaborator-

TCTR20191213001 / Enrolling by invitationPhase 2IIT

A pilot study, The efficacy of topical 0.025% capsaicin gel in the treatment of lichen amyloidosis at Institute of dermatology, Thailand

Start Date02 Dec 2019

Sponsor / Collaborator-

100 Clinical Results associated with Amyloidosis, Primary Cutaneous

100 Translational Medicine associated with Amyloidosis, Primary Cutaneous

0 Patents (Medical) associated with Amyloidosis, Primary Cutaneous

1,204

Literatures (Medical) associated with Amyloidosis, Primary Cutaneous

01 Nov 2024·Journal of the European Academy of Dermatology and Venereology

Primary localized cutaneous amyloidosis associated with atopic dermatitis treated successfully with nemolizumab

Letter

Author: Tanaka, Rio ; Kubo, Akiharu ; Tanaka, Ryo ; Yoshioka, Ai ; Fukumoto, Takeshi ; Natsuaki, Satoshi ; Hashimoto, Shinya

A 67-yr-old Japanese man with paediatric-onset AD had a 20-yr history of PLCA and severe itch.Phys. examination revealed pruritic erythema with lichenification throughout his body and multiple firm hyperpigmented verruciform papules on the extremities.Although an IL-31 signature appears to play a role in pathogenesis in PLCA associated with AD, further studies are required.Our patient exhibited rapid relief from itching 1month after the administration of nemolizumab by inhibiting IL-31 signalling, followed by a gradual improvement in the EASI score, indicating a break in the itch-scratch cycle.

01 Oct 2024·Annales de Pathologie

Surcharges cutanées endogènes

Article

Author: Ortonne, Nicolas

As in other organs, the diagnosis of endogenous cutaneous overload diseases is based on histopathological analysis of the lesions using special stainings, even if the clinical appearance is sometimes very suggestive. The lesions are sometimes very subtle and can be included in the group of "invisible" dermatoses, such as primary macular cutaneous amyloidosis or calciphylaxis. Superficial dermal melanosis or pigmentary incontinence generally reflects the post-inflammatory stage of a chronic or recurrent interface dermatitis. Section levels should be systematically performed to look for active lesions of diagnostic interest: Alcian blue staining to identify dermal mucinosis (connectivitis) and pan-T markers (fixed pigmented erythema, lichenoid mycosis fungoides, and vitiligo). Some pathologies have a prognostic impact, either because they reflect an underlying disease, monoclonal gammopathies, in particular myeloma, being one of the most common conditions in this context (AL amyloidosis, xanthoma and xanthogranuloma, scleromyxedema), or because they can be associated with visceral damage (AL amyloidosis, scleromyxedema). The clinical-pathological comparison is mandatory to rule out differential diagnoses, especially for life-threatening diseases: nodular amyloidosis and primary cutaneous amyloidosis versus systemic AL amyloidosis, papular mucinosis versus scleromyxedema and calcific panniculitis versus calciphylaxis.

10 Sep 2024·International journal of molecular sciences

Thyroid Malignancy and Cutaneous Lichen Amyloidosis: Key Points Amid RET Pathogenic Variants in Medullary Thyroid Cancer/Multiple Endocrine Neoplasia Type 2 (MEN2).

Review

Author: Sandru, Florica ; Ghemigian, Adina ; Carsote, Mara ; Ciuche, Adrian ; Radu, Andreea-Maria ; Ciobica, Mihai-Lucian ; Stanescu, Laura-Semonia ; Nistor, Claudiu

We aimed to provide an updated narrative review with respect to the RET pathogenic variants and their implications at the clinical and molecular level in the diagnosis of medullary thyroid cancer (MTC)/multiple endocrine neoplasia (MEN) type 2, particularly with respect to the presence of cutaneous lichen amyloidosis (CLA). We searched English-language, in extenso original articles with no timeline nor study design restriction that were published on PubMed. A traditional interplay stands for CLA and MTC in MEN2 (not MEN3) confirmation. While the connection has been reported for more than three decades, there is still a large gap in understanding and addressing it. The majority of patients with MEN2A-CLA have RET pathogenic variants at codon 634; hence, it suggests an involvement of this specific cysteine residue in both disorders (most data agree that one-third of C634-positive subjects have CLA, but the ranges are between 9% and 50%). Females seem more prone to MEN2-CLA than males. Non-C634 germline RET pathogenic variants included (at a low level of statistical evidence) the following: RET V804M mutation in exon 14 for MTC-CLA (CLA at upper back); RET S891A mutation in exon 15 binding OSMR variant G513D (familial MTC and CLA comprising the lower legs to thighs, upper back, shoulders, arms, and forearms); and C611Y (CLA at interscapular region), respectively. Typically, CLA is detected at an early age (from childhood until young adulthood) before the actual MTC identification unless RET screening protocols are already applied. The time frame between CLA diagnosis and the identification of RET pathogenic variants was between 5 and 60 years according to one study. The same RET mutation in one family is not necessarily associated with the same CLA presentation. In MTC/MEN2 subjects, the most affected CLA area was the scapular region of the upper back. Alternatively, another hypothesis highlighted the fact that CLA is secondary to long-term prurit/notalgia paresthetica (NP) in MTC/MEN2. OSMR p. G513D may play a role in modifying the evolutionary processes of CLA in subjects co-harboring RET mutations (further studies are necessary to sustain this aspect). Awareness in CLA-positive patients is essential, including the decision of RET testing in selected cases.

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Amyloidosis, Primary Cutaneous

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