Hyper IgE syndrome
An 18-year-old boy was referred to us because of widely disseminated erythematous plaque with scaling and lichenification since the age of one year. Growth retardation and mild psychomotor delay were observed during his development. At the six year of age, a history of intermittent epilepsy was recorded. The seizures consisted of loss of consciousness, deviation of eyes and head along with tonic posturing of arm or leg of either side or associated flushing. The seizures usually lasted for 10 minutes. The initial frequency was 5-6 times per year. He had been treated with sodium valproate. Improvement was noted after six years with frequency decreased to once per year. He was born healthy (weight = 3.59 kg, height = 49 cm) and followed the recommended schedule for vaccination. There was no history of asthma, epilepsy, trauma or atopic dermatitis among family members.\nPhysical examination revealed growth retardation for his age with height 150 cm and weight 40 kg. His facial abnormalities was obvious including a prominent forehead, a broad nasal bridge, deep set eyes, broad outer canthal distance []. Chronic eczematous dermatitis including a diffuse erythematous with scaling and lichenification spread through his face, entire trunk and extremities [Figure , ]. No obvious abscesses were noted on skin but seldom atrophic scars. Hyperextensible joints including scoliosis and talipes cavus [] were also seen. There was no abnormality of the hair or nails. Neurological examination showed limb muscle strength of grade V, normal muscular tension of upper limb, increasing active achilles tendon reflex of lower limb. The findings of the rest of the physical examination were unremarkable.\nHis laboratory work up included excessively elevated serum level of IgE 20800 IU/ml (normal range 0-100 IU/ml), IgA 441 mg/dl (normal range 69-382 mg/dl) and eosinophils 8.6% with absolute eosinophils\n0.8×109/L (normal range). Levels of IgM, IgG, and number of CD4+ and CD8+ T lymphocyte in serum were in normal range. The skin biopsy demonstrated mild epidermal hyperkeratosis with intermittent parakeratosis, exocytosis of neutrophils and intercellular edema. In the dermis, papillary edema, infiltration of lymphocytes and dilated capillaries in superficial dermis were prominent []. Maxillofacial X-ray showed primary dentition of the left maxillary first molar was retained [] and X-ray of feet showed features of talipes cavus. EEG, CT of the brain, and chest X-ray was normal. Intelligence assessment showed decreased calculation ability.\nBased on the typical clinical features, excessively increased IgE levels and the histopathology findings, the diagnosis of HIES was confirmed. Moreover, neurological complication was prominent in this case and other related neurological diseases were differentiated. The patient was treated with moisturizer for the rash and oral antihistamine. Improvement was noted at week three post-treatment and the treatment was well tolerated.