Diagnosis and treatment of porcupine-like ichthyosis: a case report
10.3760/cma.j.issn.0412-4030.2010.11.018
- VernacularTitle:豪猪状鱼鳞病一例诊断与治疗观察
- Author:
Xibao ZHANG
;
Xuemei LI
;
Jianfang SUN
;
Yuqing HE
;
Xin TIAN
;
Quan LUO
;
Xiao XU
;
Changxing LI
;
Xin ZHOU
;
Wei LI
;
Li CUI
;
Gengyun TANG
;
Xinjing GAO
- Publication Type:Journal Article
- Keywords:
Ichthyosis hystrix;
Acitretin
- From:
Chinese Journal of Dermatology
2010;43(11):801-804
- CountryChina
- Language:Chinese
-
Abstract:
A 10-year-old boy presented with a 3-year history of erythematous flat keratotic papules and brown-yellow, nail-like prominent keratotic plaques all over the body surface. Dermatological examination showed verrucous or nail-like prominence over multiple erythematous keratotic plaques on the head, face,trunk and limbs. The lesions, most of which confluenced, were covered with brown-yellow and greasy crusts,and gave a porcupine-like appearance. Skin biopsy of lesions from the back revealed epidermal hyperkeratosis,focal columnar parakeratosis, acanthosis, few acantholytic and dyskeratotic cells in stratum corneum, irregular upward proliferation of dermal papilla cells, and a superficial perivascular lymphocytic infiltration. A diagnosis of ichthyosis hystrix was established based on the histopathological findings. The boy was treated with oral acitretin and topical 0.1% acitretin cream for 8 years. The initial and maximum dose of oral acitretin was 0.5 mg·kg-1·d-1 and 1 mg·kg-1·d-1, respectively. Liver and kidney function, body height and weight were examined during the treatment, and no side effect was observed except for skin xerosis.
