Devred I, Sfecci A, Cardot-Leccia N, Lacour J-P, Passeron T
Service de dermatologie, université Côte d'Azur, CHU, Nice, France.
Service d'anatomo-pathologie, université Côte d'Azur, CHU, Nice, France.
Ann Dermatol Venereol. 2019 Mar;146(3):215-218. doi: 10.1016/j.annder.2018.07.006. Epub 2018 Aug 11.
Prurigo pigmentosa is a rare inflammatory dermatosis characterized by pruritic and reticulate papules on the trunk leaving hyperpigmentation. This dermatosis has been rarely described outside Asia. The pathophysiology remains obscure.
We report the case of a 21-year-old North-African woman presenting with a highly pruritic eruption with numerous erythematous macules and papules coalescing in a reticular pattern on the trunk. The eruption occurred during the first trimester of pregnancy, which was marked by severe vomiting resulting in weight loss of 13kg with ketonemia and ketonuria. Taking into account the characteristic pattern of the eruption, the absence of differential diagnosis, and the histological examination, we concluded on a diagnosis of prurigo pigmentosa. Progression of the disease exhibited phases of decreased inflammation and of pruritus alternating with episodes of inflammatory flares triggered by relapses of ketonemia. The symptoms finally resolved during the 2nd trimester of pregnancy after vomiting ceased. Secondary reticulated hyperpigmentation was observed.
Although rare, the highly evocative clinical presentation of the eruption should help clinicians in diagnosing prurigo pigmentosa. While pathophysiology remains undetermined, prurigo pigmentosa was reported in cases of intense fasting, anorexia, type-1 diabetes, and in two other cases of pregnancy with severe vomiting. Our case underlines the need to screen for and treat underlying ketonemia to achieve control of the inflammatory flares of prurigo pigmentosa.
色素性痒疹是一种罕见的炎症性皮肤病,其特征为躯干出现瘙痒性网状丘疹并遗留色素沉着。这种皮肤病在亚洲以外地区鲜有报道。其病理生理学仍不清楚。
我们报告一例21岁的北非女性病例,该患者躯干出现剧烈瘙痒性皮疹,有大量红斑和丘疹融合成网状。皮疹发生在妊娠早期,其特征为严重呕吐导致体重减轻13kg,伴有酮血症和酮尿症。考虑到皮疹的特征性表现、缺乏鉴别诊断以及组织学检查结果,我们诊断为色素性痒疹。疾病进展呈现炎症和瘙痒减轻阶段,与酮血症复发引发的炎症发作交替出现。呕吐停止后,症状最终在妊娠中期缓解。观察到继发性网状色素沉着。
尽管色素性痒疹罕见,但其皮疹高度提示性的临床表现应有助于临床医生进行诊断。虽然病理生理学仍未明确,但色素性痒疹在剧烈禁食、厌食、1型糖尿病病例中以及另外两例伴有严重呕吐的妊娠病例中均有报道。我们的病例强调需要筛查和治疗潜在的酮血症,以控制色素性痒疹的炎症发作。