Lichen Amyloidosis in an Atopic Patient Treated with Dupilumab: A New Therapeutic Option.

Lichen amyloidosis (LA) is a type of cutaneous amyloidosis characterized by brownish hyperkeratotic and itchy papules on the lower leg, back, forearm, or thigh. It is associated with itching and atopic dermatitis (AD) according to an etiopathogenetic mechanism that has not yet been fully elucidated. Currently, the available therapies for this condition include oral antihistamines, laser, cyclosporine, topical corticosteroids, and phototherapy, but, in light of the overlap with AD, Dupilumab may also be indicated. We report the case of a female, 52 years old, who had been suffering from AD and LA for about 27 years. She had lesions attributable to both diseases on the trunk and lower limbs associated with severe itching and had proved resistant to cyclosporine therapy. It was decided to opt for Dupilumab with the induction of 2 fl of 300 mg and maintenance with 1 fl every other week. The therapy proved to be effective, returning a total resolution of both diseases one year after the beginning of the treatment. Dupilumab demonstrated efficacy and safety in the LA related to AD and led to clinical and quality of life improvements in this patient. Therefore, Dupilumab should be considered when treating LA. Further studies should be conducted focusing on the efficacy of the drug on LA (whether or not related to AD), changes in the skin lesions after discontinuation, and the safety of long-term application.

Clinical Characteristics of Lichen Amyloidosis Associated with Atopic Dermatitis: A Single Center, Retrospective Study.

BACKGROUND: Lichen amyloidosis is a chronic pruritic skin disorder associated with atopic dermatitis, however, the pathogenetic link between these two conditions remains to be elucidated. Only limited research has been performed on patients diagnosed with both pruritic dermatological conditions. OBJECTIVE: This study aimed to analyze the clinical features of lichen amyloidosis associated with atopic dermatitis. METHODS: We conducted a matched case-control study of incident lichen amyloidosis with atopic dermatitis between March 2020 and February 2022. Among the 2,481 patients with atopic dermatitis, 20 patients diagnosed with lichen amyloidosis and atopic dermatitis were included as case patients, and 20 patients diagnosed with atopic dermatitis were enrolled as controls. The controls were matched to cases (1:1) by age and sex. We retrospectively reviewed the medical records of the patients. RESULTS: The prevalence of lichen amyloidosis associated with atopic dermatitis was approximately 0.8%, with a male:female sex ratio of 2.33:1. The recorded onset of lichen amyloidosis associated with atopic dermatitis was more common in adult patients, with moderate-to-severe atopic dermatitis. Lichen amyloidosis lesions in patients with atopic dermatitis were most commonly found on the extremities, sparing the head and neck region. The presence of lichen amyloidosis had no significant impact on severity of atopic dermatitis. CONCLUSION: In patients with lichen amyloidosis associated with atopic dermatitis, the clinical manifestations of lesions are similar to those of conventional lichen amyloidosis lesions in terms of morphology and regional distribution. Further research is required to elucidate the link between the pathogenesis of these two pruritic dermatological conditions.

Primary Localized Cutaneous Amyloidosis in Central Europe: A Retrospective Monocentric Study on Epidemiology and Therapy.

Amyloid deposits can be the cause of many chronic diseases. Primary localized cutaneous amyloidosis (PLCA) is a chronic dermatologic condition with amyloid deposits in the papillary dermis. The most common types of the keratinocyte-derived form of PLCA include macular (MA), lichen (LA), and biphasic (BA) amyloidosis. The estimated prevalence of PLCA in the Asian population is 0.98/10,000, which is higher than in the European population; thus, epidemiologic data on PLCA in the Caucasian population are limited. We performed a retrospective single-center study analyzing epidemiologic characteristics of a Central European PLCA population. Epidemiologic data regarding age, sex, skin phototype (Fitzpatrick scale I-VI), disease duration, comorbidities, history of atopy, and family history of PLCA were collected. Clinical characteristics, localization of PLCA lesions, applied therapies and treatment outcomes were also analyzed. Dermoscopic characteristics were also evaluated. A total of 41 patients diagnosed with PLCA were included, with 22 presenting with macular, 18 with lichen, and 1 with biphasic amyloidosis. The male/female ratio was 16/25, and mean age at diagnosis was 54.6 ± 15.2 years (range 27-87 years). The mean age at the onset of PLCA was 53 ± 16.1 years (range 19-79 years) in MA, 46.7 ± 18.2 years (range 14-73 years) in LA, and 26 years in BA. The interscapular region in MA and the extensor surface of the lower extremities in LA proved to be localization-related areas. In our center, a wide range of therapeutic options was applied, with the most prescribed being topical corticosteroids in all types of PLCA. We presented a retrospective, monocentric study on the epidemiology of PLCA in the Central European region. By examining the medical data of a significant number of PLCA patients, we compared our epidemiologic data with that of the Asian PLCA population. Due to the rarity of the condition, further randomized controlled trials and guidelines are needed to improve therapeutic outcomes.

A Case Report of Generalized Non-pruritic Lichen Amyloidosis.

We evaluate the rare case of a patient who presented with generalized, non-pruritic lichen amyloidosis. There are three reported cases of generalized and non-pruritic lichen amyloidosis. The lichen amyloidosis subtype of primary localized cutaneous amyloidosis is characterized by keratinocyte-derived amyloid deposition in the papillary dermis, classically presenting as pruritic, hyperpigmented macules coalescing into plaques on the lower extremities. While the pathogenesis is likely multifactorial, chronic scratching has been proposed as an inciting factor. The patient's type of lichen amyloidosis challenges the proposed etiology of chronic scratching leading to amyloid deposition.

Successful treatment of lichen amyloidosis coexisting with atopic dermatitis by dupilumab: Four case reports.

BACKGROUND: Lichen amyloidosis (LA) is a chronic, severely pruritic skin disease, which is the most common form of primary cutaneous amyloidosis. The treatment of LA has been considered to be difficult. LA may be associated with atopic dermatitis (AD), and in this setting, the treatment options may be more limited. Herein, we report four cases of LA associated with AD successfully treated by dupilumab. CASE SUMMARY: In this article, we describe four cases of patients who presented with recurrent skin rash accompanied by severe generalized intractable pruritus, diagnosed with refractory LA coexisting with chronic AD. Previous treatments had not produced any apparent improvement. Thus, we administered dupilumab injection subcutaneously at a dose of 600 mg for the first time and 300 mg every 2 wk thereafter. Their lesions all markedly improved. CONCLUSION: Dupilumab may be a new useful treatment for LA coexisting with AD.

Immunoglobulin G4-Related Disease-Associated Dermatitis with Pruritus: A Positive Response to Dupilumab.

Immunoglobulin G4-related disease (IgG4-RD) is a rare fibro-inflammatory condition characterized by IgG4-expressing plasma cell infiltration of the skin and other organs, leading to profound itchiness. Oral corticosteroids are the first-line therapy for IgG4-RD but relapses and potential side effects are common. In this case, we discuss a patient with a hyperpigmented, scaling dermatitis on his arms, back, and chest with lichen amyloidosis (LA) that incompletely responded to corticosteroids. He had reduced quality of life secondary to chronic pruritus. Dupilumab, an IL-4 and IL-13 inhibitor, was initiated. He experienced a transient worsening, followed by complete resolution of his itch with remission of his rash. While the pathogenesis of IgG4-RD is not entirely understood, a T-helper 2 (Th2) immune response has been implicated, with interleukins (IL) 4, 5, 10, and 13 playing a role in IgG4 class switch, resulting in eosinophilia and elevated IgE. The strong response of dupilumab in this case may provide evidence in favor of the involvement of IL-4 and IL-13 in the pathogenesis of cutaneous IgG4-RD. Future clinical studies involving larger patient populations may be warranted.

Coexistent Lichen Amyloidosis and Acquired Reactive Perforating Collagenosis in Type 2 Diabetes Mellitus and Post-Thyroidectomy Hypothyroidism Due to Papillary Thyroid Carcinoma: A Rare Case.

An abundance of endocrine receptors is expressed on the skin and becomes the target of many hormones. This was associated with various skin diseases that might occur in some endocrine diseases eg, lichen amyloidosis (LA) and acquired reactive perforating collagenosis (ARPC). Here, we report a coexistent LA and ARPC in a 55-year-old woman, characterized with multiple pruritic hyperkeratotic papules and plaques on both arms accompanied by pruritic hyperkeratotic papules and nodules on both legs. She had a history of type 2 diabetes mellitus (DM) and post-thyroidectomy hypothyroidism due to papillary thyroid carcinoma. Histopathological examination revealed amyloid deposition in the papillary dermis corresponding with LA and cup-shaped epidermal depression filled with collagen corresponding with ARPC. The hyperkeratotic papules and nodules flattened in one month after application of 0.05% clobetasol propionate ointment with occlusion on both arms and 0.05% retinoic acid gel on both legs. Hyperkeratotic papules, nodules, and plaques in a patient with a history of endocrine diseases, such as type 2 DM and thyroid disorders should undergo histopathological examination to confirm the diagnosis of skin diseases eg, LA or ARPC.

Lichen Amyloidosis on the Scalp: A Case Report.

Lichen amyloidosis (LA) is one form of primary cutaneous amyloidosis, presented as discrete, lichenoid papules with itching, commonly involving the extensor surfaces of extremities. Scalp involvement is rarely reported in the literature. In this study, we reported a case of LA over the crown and vertex areas of the scalp. The lesions subsided with topical clobetasol propionate/all-trans retinoic acid compound ointment treatment for 2 months and achieved significant improvement.

The therapeutic role of lasers in primary localized cutaneous amyloidosis: a systematic review.

With the investigation of the efficacy of laser therapy in primary localized amyloidosis(PLCA) only recently starting to materialize, we aimed to review the currently available studies of laser therapy in the management of the disease. We searched PubMed, Scopus, Embase, Web of Science, Cochrane, and ProQuest online databases with a specified search strategy, assessed the quality of each study, and then extracted the eligible data. Five RCTs(randomized controlled trials), one non-randomized controlled trial, three case series, and nine case reports(18 in total) were included. Overall, carbon dioxide (CO2), neodymium-doped:yttrium aluminum garnet (Nd:YAG), pulsed dye (PDL), Er (Erbium):YAG, and yttrium/erbium fiber were the studied lasers. One hundred fifty-five cases in total underwent laser therapy, with CO2 being the most frequent laser. Almost all studies demonstrated significantly desirable outcomes, while only mild and transient side effects were noted. Even though the studies' results were significant, we noticed that implementing a consistent methodology and a standardized objective assessment method was missing. Therefore, we recommend that future studies be conducted with less heterogeneous data for a more definite conclusion.

Primary Localized Cutaneous Amyloidosis: A Retrospective Study of an Uncommon Skin Disease in the Largest Tertiary Care Center in Switzerland.

BACKGROUND: Primary localized cutaneous amyloidosis (PLCA) is defined by the deposition of amyloid protein in the skin without systemic involvement. There are four subtypes of PLCA: lichen amyloidosis (LA), macular amyloidosis (MA), biphasic amyloidosis (BA), and nodular amyloidosis (NA). PLCA occurs most frequently in Latin Americans and Asians. Treatment is not standardized. OBJECTIVES: To identify subtypes, demographic and clinical features and treatment efficacy in patients with histopathologically confirmed PLCA. MATERIALS AND METHODS: Data of PLCA patients were extracted from the electronic hospital database and included if diagnosis of PLCA was histopathologically confirmed and if sufficient information regarding treatment and follow-up was available. The evaluation of the treatment efficacy was based on a novel score to assess the reduction of itch and skin lesions. RESULTS: In this retrospective, monocentric study, 37 cases of PLCA diagnosed between 2000 and 2020 were included (21 females) with a mean age of 52 years. LA was the most frequent subtype found in 21 patients (56.8%), followed by MA in 10 patients (28%) and BA in 6 patients (16.2%). No cases of NA were included. 22 patients (59.4%) had skin phototype II or III. Regarding treatment, a combination of UVA1 phototherapy with high-potency topical corticosteroids seemed to show the highest efficacy with complete clearance of symptoms in 4 patients (10.8%). A substantial improvement of symptoms was found in 5 patients (12.7%) treated with high-potency topical corticosteroids alone or in combination either with UVA1 or bath PUVA or monotherapy with UVA1 phototherapy or capsaicin (0.075%) cream. Low-/medium-potency topical corticosteroids alone or in combination with UVBnb (311 nm) phototherapy showed a lower efficacy. CONCLUSION: Our data show that PCLA is a rare disease in central Europe but can also be expected in a predominantly Caucasian population. The best treatment response was achieved with a combination of UVA1 phototherapy and high-potency topical corticosteroids.

Dermoscopic Features of Lichen Amyloidosis in Caucasians-A Case Series and Literature Review.

Primary cutaneous amyloidosis (PCA) is characterized by the extracellular deposition of amyloid in the skin without systemic involvement. It comprises several clinical variants, the most common of which are macular amyloidosis (MA) and lichen amyloidosis (LA). PCA is frequently observed in Asians, while it is considered to be very rare in Caucasians. In the latter population, the condition often poses a diagnostic challenge. Dermoscopy has already been proved to be a useful, non-invasive diagnostic tool in various non-neoplastic skin diseases. In the paper, we present three Caucasian patients (skin phototypes I-II) with histologically confirmed LA. Under dermoscopy, central white hubs with grayish-brown dots and globules were observed in all three cases. Vascular structures were present in two cases and had the morphology of red globules and thick, unfocused branching lines intersecting the white hubs. A comprehensive review of the literature retrieved twelve papers presenting the dermoscopic features of PCA, including five articles on the dermoscopy of LA. The vast majority of these studies have been conducted on the Asian population, and there is a lack of data on the dermoscopic findings for patients with skin type I or II. The literature review revealed that MA and LA share several dermoscopic similarities (the presence of a white central hub and grayish dots), but also display distinct features. Compared to the dermoscopic features of LA in darker skin phototypes, our patients presented less pronounced pigmentation and more evident vascular structures. Nevertheless, further studies are needed in order to reliably evaluate the dermoscopic features of PCA in various ethnicities.

Efficacy of 1064-nm Nd:YAG picosecond laser in lichen amyloidosis treatment: clinical and dermoscopic evaluation.

Lichen amyloidosis (LA) is a type of primary localized cutaneous amyloidosis characterized by multiple localized, hyperpigmented, grouped papules, in which the deposition of amyloid materials from altered keratinocytes usually resists to current treatments. We presented two LA patients with non-satisfactory results of topical treatments. After the first treatment using 1064-nm Nd: YAG picosecond (ps-Nd:YAG) laser, there was an improvement with persistence up to 3-month follow up after five sessions of 4-week interval, as well as a decrease in number, thickness, and darkness of lesions from clinical and dermoscopic evaluation. Thus, the ps-Nd:YAG laser could be efficacious for LA treatment.

FotoFinder Dermoscopy Analysis and Histopathological Correlation in Primary Localized Cutaneous Amyloidosis.

BACKGROUND: Primary localized cutaneous amyloidosis (PLCA) causes extracellular proteinaceous deposits in skin. It is clinically divided into macular amyloidosis, lichen amyloidosis and nodular amyloidosis. Atypical presentations of PLCA make the diagnosis challenging, requiring biopsy to confirm amyloid deposition in the upper papillary dermis. OBJECTIVES: This study used FotoFinder dermoscopy to characterize lichen and macular amyloidosis and correlated the dermoscopic features with histopathological findings. METHODS: This cross-sectional study enrolled patients with a clinical and histopathological diagnosis of PLCA. Dermoscopic examination was performed using the FotoFinder dermoscope, which provides a range of magnification from 20× to 140×. RESULTS: A total of 30 patients were included in the study. Common dermoscopic patterns of MA were white or brown central hubs, and common patterns of LA were white structureless, scar-like areas and central hubs. New dermoscopic findings were a day lily appearance in MA and white rosettes in LA. CONCLUSIONS: Dermoscopy plays a pivotal role in demonstrating characteristic findings of PLCA. These findings were well corelated with histopathology, thus avoiding unnecessary biopsy for arriving at an accurate diagnosis of PLCA.

A Rare Presentation of Nodular Amyloidosis on the Lower Back.

Primary localized cutaneous amyloidosis (PLCA) occurs when amyloid is deposited only within the skin and there is no evidence of systemic involvement. Nodular amyloidosis is the rarest subtype of PLCA. It typically involves the acral regions but can sometimes present on the head and neck. The condition usually presents clinically as a single tan or yellow nodule or plaque that may appear waxy. Herein, we present a rare case of a 66-year-old man with nodular amyloidosis on the lower back.

Clinicopathological Study of Primary Cutaneous Amyloidosis in a Tertiary Care Center of Eastern India Reveals Insignificant Association with Friction, Scrubbing, and Photo-Exposure: How valid is the "Keratinocyte Hypothesis"?

INTRODUCTION: Primary cutaneous amyloidosis (PCA) can be classified into four principal categories: macular amyloidosis, lichen amyloidosis, biphasic, and nodular amyloidosis. Some unusual variants such as widespread diffuse hyperpigmentation without papules, poikiloderma like involvement, lesions following Blaschko's line, etc., have also been reported. However, not much data are available regarding the demography, epidemiology, clinical patterns, and distribution and histopathological findings, especially from the eastern part of India. AIMS: We conducted a cross-sectional, institution-based study to evaluate clinicopathological pattern and factors of PCA in eastern India. MATERIALS AND METHODS: We recorded clinical and histopathological findings of 100 consecutive patients of PCA presenting to a tertiary care institution of Kolkata in eastern India. RESULTS: We found female patients of PCA outnumber male (M:F =1:1.9) with majority of patients being young adults (56%) between 20 and 40 years of age. More than half (54%) of the patients were pruritic. The severity of pruritus is significantly more associated with lichenoid and biphasic variants over macular amyloidosis. Positive family history was recorded in 17% of cases. Macular variant was the most common variant constituting 48% of the total PCA. We also found that the association with history of friction and scrubbing and photo-exposure were statistically insignificant. However, duration of the disease has statistically significant association with the disease morphology. Congo red stain showed these deposits as reddish orange substance in 28 patients out of 64 patients' samples on which Congo red could be performed. CONCLUSION: Our study revealed that many concepts of pathogenesis of PCA including friction and photoexposure might have lesser importance. However, morphological types were significantly associated with the duration of the disease and symptom severity.

Lichen amyloidosis successfully treated with fractional ablative laser CO2: A new alternative therapeutic.

Lichen amyloidosis is a primary localized cutaneous amyloidosis. Different types of treatment have been used without complete resolution. Herein, we report a case of patient suffering from lichen amyloidosis successfully treated with fractional ablative laser CO2. He was a 59-year-old man diagnosed lichen amyloidosis localized on the legs 10 years ago. He was treated with topical corticosteroids without any improvement. Then, we started treating the affected area with CO2 laser (limmer*) at a setting of 5-8 J/cm2 and 8 mm laser spot size. A considerable improvement was noticed after the first session. A total healing was reported after four sessions. To the best of our knowledge, only 11 reported cases of lichen amyloidosis have been successfully treated with laser CO2. However, our clinical finding seems to be one of the best reported results.

The Clinical Spectrum of Multiple Endocrine Neoplasia Type 2A with Cutaneous Lichen Amyloidosis in Ethnic Han Chinese.

This study systematically reviewed previous literatures and analyzed the genotype-phenotype relationship between the multiple endocrine neoplasia type 2A (MEN 2A)-cutaneous lichen amyloidosis (CLA) and RET/OSMR/IL31RA mutations. RET/OSMR/IL31RA screening was performed on 8 RET-carriers from 3 independent Chinese MEN 2A families. Besides, 51 MEN 2A-CLA patients in 116 RET carriers from literatures were clustered and analyzed. Our results indicated that almost all MEN 2A-CLA patients exhibited CLA which was located in the scapular region and carried RET mutation at codon 634. Meanwhile, we firstly described MEN 2A-CLA here in Chinese Han patient with RET p.C634F mutation.