An Unusual Overlap of Lipoid Proteinosis and Cutaneous Amyloidosis – A Case Report

Sheron Christin Armel, Trishna Vaishali Muthurathinam
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Abstract

Lipoid proteinosis is a rare genodermatosis that primarily affects the skin and mucous membranes of the respiratory and digestive systems. Cutaneous amyloidosis is characterised by deposition of amyloid protein in the upper papillary dermis. The present case is a 32-year-old male who presented with asymptomatic multiple raised skin coloured lesions and hoarseness of voice since childhood. On examination, he had multiple skin coloured and yellow waxy papules, some discrete and others coalescing to form plaques over the trunk, neck, upper and lower limbs. He also had multiple hyperpigmented papules and plaques on the back and neck, and ovoid shaped scars on the back. Examination of the eyelids revealed moniliform blepharosis, and there were pock like scars on the face. Indentations were present on the lateral and anterior borders of the tongue. Biopsy confirmed the same with few areas positive for Congo red staining. Overlap of lipoid proteinosis and macular amyloidosis was diagnosed. We report this overlap condition for its rarity.
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类脂样蛋白病与皮肤淀粉样变性的不寻常重叠--病例报告
类脂样蛋白病是一种罕见的遗传性皮肤病,主要影响皮肤以及呼吸系统和消化系统的粘膜。皮肤淀粉样变性的特点是淀粉样蛋白沉积在真皮乳头上部。本病例是一名 32 岁的男性,从小就出现无症状的多发性隆起皮损和声音嘶哑。经检查,他的躯干、颈部、上肢和下肢有多处皮肤色素沉着和黄色蜡样丘疹,有些是离散的,有些则凝聚成斑块。他的背部和颈部还有多处色素沉着丘疹和斑块,背部还有卵圆形疤痕。眼睑检查发现了单形眼睑病,面部有痘状疤痕。舌头的外侧和前缘有凹痕。活组织检查证实了同样的情况,少数区域刚果红染色呈阳性。确诊为类脂样蛋白病和黄斑淀粉样变性重叠症。我们报告的这种重叠病症非常罕见。
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