Netherton’s syndrome

Li, a 10-year-old female student, was admitted to the hospital on November 12, 2008 with the chief complaint of “recurrent erythema, blistering, crusting, and flaking all over the body for 10 years”. History: On the 6th day after birth, the child’s skin was flushed and moist with dense milky papules and blisters, with thick scabs forming a few days later. After the scabs came off, thin-walled flaccid blisters appeared on the flushed skin, which soon broke down into vesicles, and then blisters appeared again after the scabs and debridement, and so on. He had a lumbar puncture examination at the county people’s hospital, but no results. At the age of 1 year, he was treated at a city Chinese medicine hospital (diagnosis unknown, treatment plan unknown), then at a tertiary hospital where he was given internal and external medicine for “eczema” (specific medicine unknown), and at the age of 8 years, he was treated at a city hospital in Fujian (diagnosis unknown, treatment plan unknown). Dexamethasone tablets (dosage unknown) were included in the medication given at each hospital, and later injected medication at a private clinic (specific details unknown), but the efficacy was not satisfactory, and the condition was slightly relieved during the treatment period and then recurred. Today, he came to our hospital for a clear diagnosis and systematic treatment, and was admitted to the outpatient clinic as “atopic dermatitis”. He was admitted to our hospital with “atopic dermatitis”. His symptoms were: large erythema and desquamation of the head, mild ectropion of the lid margin, congestion of the lid conjunctiva, dry skin all over the body, large erythema with irregular circular distribution and scattered flaccid thin-walled blisters at the edges, extensive crusting and desquamation all over the body, slightly high skin temperature, short and easily folded hair, obvious roughness of the skin lines on both hands, intense itching, sleepiness, dry stool and normal urine. The tongue is red with little coating and the pulse is fine. Specialized examination: large erythematous and desquamated head and face, short and easily folded hair, mild ectropion of the lid margin, lid conjunctiva congestion, generalized dry skin, large erythematous patches with irregular circular distribution, massive crusting and desquamating on the erythematous patches, scattered flaccid thin-walled blisters on the edges, positive Nixon’s sign, slightly high skin temperature, sweating is not obvious, skin lines on the palm surface of both hands are obviously rough, Zhang Xiaojing, Department of Dermatology, The First Affiliated Hospital of Henan College of Traditional Chinese Medicine, was admitted to diagnose. Netherton’s syndrome. Laboratory tests showed: platelet pressure 0.29%, neutrophil ratio 35.2%, eosinophil ratio 25.4%, eosinophil 2.08*109/L; total protein 58.4g/L, albumin 37.1g/L, creatinine 39.5umol/L, phosphorus 1.75mmol/L; urobilinogen (±); pathological section of right lower limb medial femur showed: epidermis The pathological section showed: mild hyperkeratosis of the epidermis, no obvious atrophy, a small number of intracutaneous vacuoles liquefaction, epithelial foot extension, a small number of lymphocytes infiltration in the subdermis and around small vessels, the lesion may be consistent with herpes-like dermatitis or aspergillosis. After admission, he was given compound glycyrrhizin injection 40ml, influnomide injection 160mg sedation, anti-wind and anti-itch granules 6g Tidpo, Chinese herbal medicine treatment to nourish yin, replenish blood and clear deficiency heat, formula: raw Radix Rehmanniae 10g, Radix et Rhizoma Ginseng 10g, Radix et Rhizoma Dioscoreae 10g, Fructus Lycii 10g, Radix et Rhizoma Nanshashen 10g, Radix Rehmanniae 10g, Radix Salviae Miltiorrhizae 10g, Radix Angelicae Sinensis 10g, Radix Paeoniae Alba 10g, Radix et Rhizoma Gastrodiae 15g, Radix Aconiti 6g, Radix Glycyrrhizae 6g. g, Glycyrrhiza glabra 6 g. Afterwards, erythromycin ointment was given topical Bid to relieve dry skin symptoms. After 10 days of treatment, the patient’s skin lesions on the trunk significantly subsided, and the head and face also reduced, without complaining of any discomfort.