PDF(Pubmed)
Abstract：
Pityriasis rubra pilaris (PRP) is a rare inflammatory papulosquamous skin disease that has six distinct types. Type 5 PRP is called atypical juvenile PRP. Here we report the case of a 17-year-old boy with insignificant past medical history presenting with a history of persistent slowly progressing very itchy skin lesions since the age of seven years. The lesions were photoaggravated. No similar cases in the family were observed and the parents were not consanguint. Skin examination revealed scaly erythematous patches, papules and plaques all over his body. There were also ichthyosiform-like scales covering the whole body. Hair, nails, and mucus membranes examinations were normal. A 4-mm punch skin biopsy was taken. The dermis revealed hyperkeratosis with checkerboard pattern of orthokeratosis and parakeratosis, the granular layer was preserved and acanthosis with thick and short rete ridges. The dermis showed mild perivascular lymphocytic infiltrates. On the basis of the above clinicopathological findings, the diagnosis of pityriasis rubra pilaris (atypical juvenile type) (type 5) was made. The patient was started on isotretinoin capsule 20 mg twice a day and placed under periodic follow-up.
摘要：
发毛糠疹（PRP）是一种罕见的炎症性丘疹鳞状皮肤病，有六种不同类型。5型PRP称为非典型少年PRP。在这里，我们报告了一个17岁男孩的病例，该男孩的既往病史微不足道，自7岁以来一直存在缓慢进展的非常发痒的皮肤病变。病变轻度加重。在家庭中未观察到类似的病例，父母也没有血缘关系。皮肤检查显示有鳞状红斑，他全身都有丘疹和斑块.全身也有鱼鳞状鳞片。头发,指甲,粘膜检查正常。取4-mm穿孔皮肤活检。真皮显示角化过度，呈棋盘状角膜角化和角化不全，颗粒层保留，棘皮有厚短的网纹脊。真皮显示轻度血管周围淋巴细胞浸润。根据上述临床病理发现，诊断为毛发红色糠疹（非典型青少年型）（5型）。患者开始服用异维A酸胶囊20mg，每天两次，并进行定期随访。
