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

Abstract

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.

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

“…Lichen amyloidosis (LA) is the most common form of Primary Localized Cutaneous Amyloidosis (PLCA) [1]. The latter is characterized by the extracellular amyloid deposition exclusively in the skin [2][3][4][5]. Primary localized cutaneous amyloidosis is divided into four subtypes, namely: lichen amyloidosis, macular amyloidosis (MA), biphasic amyloidosis (BA), and nodular amyloidosis (NA).…”

“…Clinically, LA presents with clusters of multiple brownish hyperkeratotic papules coalescing in plaques. They may be localized or generalized and are typically distributed on the lower leg, back, forearm or thigh [2][3][4][5]. At the onset, lesions are usually unilateral, but a bilateral symmetric distribution pattern can develop over time.…”

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

“…Lichen amyloidosis (LA) is the most common form of primary localized cutaneous amyloidosis (PLCA) [1]. It is characterized by an extracellular amyloid deposition exclusively in the skin [2][3][4][5]. Primary localized cutaneous amyloidosis is divided into four subtypes, namely lichen amyloidosis, macular amyloidosis (MA), biphasic amyloidosis (BA), and nodular amyloidosis (NA).…”

“…Clinically, LA presents with clusters of multiple brownish hyperkeratotic papules coalescing in plaques. They may be localized or generalized and are typically distributed on the lower leg, back, forearm, or thigh [2][3][4][5]. At the onset, the lesions are usually unilateral, but a bilateral symmetric distribution pattern can develop over time.…”