⑴ Erythema: Erythema, blisters and wheals are often arranged in rows and distributed on the trunk and limbs.
⑵ Verrucous hyperplasia stage: smooth red or blue-purple nodules or plaques are arranged in irregular lines, and there are linear verrucous lesions on the back of hands and feet, especially on the back of fingers and toes.
⑶ Pigmentation stage: It is a widespread irregular capsicum-like or fountain-like pigmentation with colors ranging from blue-gray to brown, especially in the trunk.
The appearance of rashes at different stages can be irregular or overlapping.
2. Pigmentation can last for many years until the age of 20 ~ 30.
3. Generally, patients are in good health, without fever, and may be accompanied by pseudoalopecia areata, tooth defect, abnormal nails, eye and retinal diseases (possibly blindness), abnormal skeletal system, central nervous system and other organs outside the skin, which are complications of the disease.
4. Laboratory examination: During erythema, blood eosinophils increased significantly.
5. Genetic genetic examination: Detection of NEMO gene sequence on X chromosome can provide reference for diagnosis. It is reported that more than 50% patients can detect the abnormality of NEMO gene on X chromosome.