Jangid Shivani D, Saoji Vikrant, Madke Bhushan, Bhatt Drishti M
Dermatology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND.
Cureus. 2024 Apr 4;16(4):e57574. doi: 10.7759/cureus.57574. eCollection 2024 Apr.
Pseudoporphyria is an uncommon dermatosis resembling porphyria cutanea tarda (PCT). The exclusion of true porphyria, especially PCT, is critically essential for diagnosing pseudoporphyria. It has an unknown underlying pathophysiology with a normal or near-normal porphyrin profile. Pseudoporphyria has been associated with chronic renal failure and hemodialysis, medications, and tanning beds. In drug-induced pseudoporphyria cases, eliminating the suspected photosensitizing drug improves the disease typically within weeks to months (on average eight weeks). In genetically predisposed individuals, phototoxic metabolites may trigger the development of skin fragility, bullae, milia, and scarring on the dorsum of the hands and other sun-exposed areas. Wearing a broad-spectrum sunscreen and maintaining strict ultraviolet protection is essential in cases of pseudoporphyria. We report the case of a 20-year-old male who presented to us with complaints of photosensitivity and multiple erosions with irregular scars over photo-exposed areas involving the dorsum of the hands and face predominantly. The patient was evaluated further to determine the underlying cause. A wood's lamp examination of the urine was done, which did not show fluorescence. Based on clinical and laboratory findings, the diagnosis of pseudoporphyria was made, and the patient was started on the oral antimalarial agent hydroxychloroquine sulfate with strict sun protection.
假性卟啉症是一种罕见的皮肤病,类似于迟发性皮肤卟啉症(PCT)。排除真正的卟啉症,尤其是PCT,对于诊断假性卟啉症至关重要。其潜在病理生理学尚不清楚,卟啉谱正常或接近正常。假性卟啉症与慢性肾衰竭、血液透析、药物以及晒黑床有关。在药物性假性卟啉症病例中,停用可疑的光敏药物通常可在数周内至数月内(平均八周)改善病情。在具有遗传易感性的个体中,光毒性代谢产物可能引发手部背部和其他暴露于阳光下区域的皮肤脆性增加、大疱、粟丘疹和瘢痕形成。对于假性卟啉症患者,涂抹广谱防晒霜并严格进行紫外线防护至关重要。我们报告了一名20岁男性的病例,该患者因光敏性以及手部背部和面部等主要暴露于阳光下区域出现多处糜烂并伴有不规则瘢痕而前来就诊。对该患者进行了进一步评估以确定潜在病因。进行了尿液的伍德灯检查,结果未显示荧光。根据临床和实验室检查结果,诊断为假性卟啉症,并让患者开始服用口服抗疟药硫酸羟氯喹,并严格进行防晒。