enteropathic acrodermatitis
Introduction
Introduction to enteric acral dermatitis Acrodermatitisenteropathica is a rare autosomal recessive hereditary disease characterized by dermatitis around the mouth and extremities, diarrhea, and alopecia. The disease usually begins to develop around 1 year old, and severe cases can cause death if not treated. basic knowledge Sickness ratio: 0.05% Susceptible people: good in early childhood Mode of infection: non-infectious complication:
Cause
Causes of enteropathy apical dermatitis
(1) Causes of the disease
It is believed that this disease is a hereditary zinc deficiency disease, and there is a dysfunction of zinc absorption in the gastrointestinal tract. The reason is that the level of zinc-binding factor (probably prostaglandin E2) secreted by a pancreas in the intestine is insufficient.
(two) pathogenesis
Zinc is an important factor in many enzymes. Its content and activity are reduced, leading to a series of clinical manifestations. It is treated with oral zinc, and its curative effect is remarkable, and life can be saved.
Prevention
Enteropathic acral dermatitis prevention
Prevention: The disease of enteric acral dermatitis is fluctuating. In order to prevent aggravation of symptoms in a special period such as pregnancy, the symptoms of adolescence are relieved, and experts remind patients to prevent it purposefully.
Complication
Enteropathic acral dermatitis complications Complication
Fingers, toenails are deformed, and even missing. Secondary lactose intolerance, malnutrition, stagnant growth and development, can be combined with bacterial or fungal infections.
Symptom
Enteropathic cutaneous dermatitis symptoms Common symptoms Hair loss, diarrhea, corneal opacity, photophobia, blushing
Onset in infants and young children, mainly in infants within 1 year after birth, the highest incidence before and after weaning, for chronic polymorphic dermatitis, the beginning of the lesion is a small blister, the blister fluid is transparent, and later secondary bacteria Or fungi, especially Candida albicans infection and turbidity, skin lesions merge with each other to form scars of different sizes, surrounded by blush, some skin lesions with psoriasis or mossy appearance, vesicular Nie's sign negative, The lesions are mainly around the mouth (oral, periocular, nostril, perianal, perineal, genital, etc.), extremities (finger, toe, heel) and limb friction (elbow joint, knee) (Figure 1), In all cases, the occurrence of de-discovery can be seen, usually before the appearance of rash, first after the mechanical stimulation of the head, then on both sides of the head, the hair is diffusely rare, soft and dull, eyebrows, eyelashes can also fall off, gastrointestinal tract Symptoms are mainly diarrhea, which discharges acid-smelling watery stools, but about 10% of patients have no intestinal symptoms. Most patients have nail deformation, atrophy and shedding, paronychia, etc., severe cases of sputum erosion, stomatitis, combined Memitis, rhinitis , photophobia, corneal opacity, mental dysfunction when the rash deteriorates, irritability, facial tilt, bow, mental retardation.
The course of the disease is fluctuating, some symptoms improve during puberty, and the pregnancy is intensified. When the childbirth or abortion, the symptoms can disappear. It may be due to the change of zinc content in the food, and when the body is in the growth stage, during pregnancy. Or caused by changes in the amount of zinc required for infectious diseases, in more serious cases, physical growth and sexual maturity disorders, accompanied by mental depression, loss of personality, severe cases can cause death.
Examine
Examination of enteropathy apical dermatitis
Plasma zinc levels are reduced.
Histopathology: skin biopsy is non-specific, showing sub-acute dermatitis histopathological changes, with epithelial spongiform edema, epidermal blister, sometimes hyperkeratosis or parakeratosis and stratum corneum hypertrophy, upper dermis and perivascular lymphocytes With inflammatory infiltration, jejunal biopsy by Loembeck et al showed impaired ultrastructure of intestinal eosinophils.
Diagnosis
Diagnosis and identification of enteropathy apical dermatitis
Diagnosis can be made based on the location of the disease, diffuse alopecia, recurrent diarrhea, and a specific mental state of aggravation.
It is often necessary to distinguish from candidiasis, bullous epidermolysis and severe malnutrition, and the determination of plasma zinc value is of great value.
