Miller Devin, Manci Rachel, Patel Jay, Guo William, Lozeau Daniel, Briley James
Department of Dermatology, Stony Brook University Hospital, 1320 Stony Brook Road, Building F Suite 200, Stony Brook, NY, 11790, United States, 1 (631) 444-4200.
Lewis Katz School of Medicine at Temple University, Philadelphia, PA, United States.
JMIR Dermatol. 2025 Sep 16;8:e77714. doi: 10.2196/77714.
Acral persistent papular mucinosis (APPM) is a localized variant of lichen myxedematosus (LM) characterized by asymptomatic, flesh-colored papules primarily distributed on the hands and forearms. This chronic dermatosis, distinct from generalized mucinosis due to its lack of systemic involvement, remains underreported in medical literature.
In this study, we present two cases of APPM to the limited pool of documented cases in the United States, highlighting its emerging recognition.
This is a case series of two patients presenting with asymptomatic papular eruptions on the hands and wrists, consistent with the typical presentation of APPM. Diagnostic confirmation via biopsy revealed focal cutaneous mucinosis. Comprehensive laboratory evaluations, including serum and urine protein electrophoresis, showed no evidence of underlying gammopathy in either patient.
Treatment modalities for APPM are limited and often ineffective. Unlike other forms of LM, APPM features are confined to skin lesions, posing primarily as a cosmetic concern with a favorable prognosis. Accurate diagnosis of this localized LM is crucial to differentiate it from the more severe, generalized form, scleromyxedema, which can have organ involvement and may become fatal. Notably, while spontaneous resolution is reported in LM, including discrete papular mucinosis, APPM typically persists without resolution even after extended follow-up.
These cases underscore the importance of recognizing APPM and advocating for broader awareness and exploration of its clinical variability, etiology, and management strategies. With increasing recognition, the understanding of APPM can be enhanced, paving the way for optimized management and improved outcomes for affected individuals.
肢端持久性丘疹性黏蛋白病（APPM）是黏液性苔藓（LM）的一种局限性变体，其特征为无症状的肉色丘疹，主要分布于手部和前臂。这种慢性皮肤病由于缺乏全身受累情况，有别于全身性黏蛋白病，在医学文献中的报道仍然较少。
在本研究中，我们向美国有限的已记录病例库中新增了两例APPM病例，以凸显其日益受到关注。
这是一个病例系列，包含两名手部和腕部出现无症状丘疹性皮疹的患者，临床表现符合APPM的典型特征。通过活检确诊为局限性皮肤黏蛋白病。包括血清和尿蛋白电泳在内的全面实验室评估显示，两名患者均无潜在丙种球蛋白病的证据。
APPM的治疗方法有限且往往效果不佳。与其他形式的LM不同，APPM的特征仅限于皮肤病变，主要是美容方面的问题，预后良好。准确诊断这种局限性LM对于将其与更严重的全身性形式硬肿性黏液水肿区分开来至关重要，后者可能累及器官并可能致命。值得注意的是，虽然在LM（包括离散性丘疹性黏蛋白病）中有自发缓解的报道，但APPM即使经过长期随访通常也不会消退。
这些病例强调了认识APPM的重要性，并提倡更广泛地了解其临床变异性、病因和管理策略。随着认识的不断提高，可以加深对APPM的理解，为优化管理和改善患者预后铺平道路。
