Erythroderma desquamativa of Leiner


Erythroderma desquamativa of Leiner: A rare skin disorder characterized by a reddish, thickened skin rash that spreads to various parts of the body and is usually accompanied by diarrhea, failure to thrive, anemia and recurring local and systemic infections.


The list of signs and symptoms mentioned in various sources for Erythroderma desquamativa of Leiner includes the 11 symptoms listed below: * Localized infection * Recurring systemic infections * Anemia * Diarrhea * Failure to thrive * Reddish skin rash * Thickened skin rash * Spreading rash * Peeling rash * Scaling rash * Itching


o Psychosocial (non-organic) –Insufficient caloric intake –Most common etiology –Cause accounts for 1/3–1/2 of cases investigated in tertiary settings * Gastrointestinal disorders –Gastroesophageal reflux –Celiac disease –Milk protein allergy –Pancreatic insufficiency –Inflammatory bowel disease * Endocrine disorders –Hypothyroidism –Hyperthyroidism –Diabetes mellitus –Diabetes insipidus –Growth hormone deficiency * Cardiac disorders –Congestive heart failure –Congenital anomalies * Pulmonary disorders –Brochopulmonary dysplasia –Asthma –Cystic fibrosis * Infectious –HIV –Parasites –Tuberculosis * Neurologic –Hypotonia –Cerebral hemorrhage –Diencephalic syndrome * Metabolic –Galactosemia –Methylmalonic acidemia –Tyrosinemia * Renal –Renal tubular acidosis –Chronic urinary tract infections –Chronic renal insufficiency * Syndromes –Down syndrome –Turner syndrome –Russell-Silver dwarfism –Fetal alcohol syndrome * Anatomic –Cleft lip/palate –Malrotation –Pyloric stenosis * Lead poisoning


The routine diagnostic workup should include a CBC, sedimentation rate, urinalysis, urine culture, chemistry panel, thyroid profile, sweat test, stool for quantitative fat, chest x-ray, and an EKG. Bone age x-rays are often helpful in indicating a growth delay. If there are focal neurologic signs or a pituitary tumor is suspected, a CT scan of the brain may be necessary. Additional endocrinologic tests include serum growth hormone level before and after exercise, somatomedin-C level, and overnight dexamethasone suppression test. However, an endocrinologist, pediatrician, or orthopedic surgeon should be consulted before ordering expensive diagnostic tests.


* Hospitalization unnecessary unless severe malnutrition or abuse * Psychosocial causes require team approach with physician, family, social worker, dieticians o Goal of refeeding to allow for catch-up growth at 1.25–1.5 times normal caloric intake for age –Monitor for refeeding syndrome with electrolyte imbalances (e.g., phosphorus, potassium, glucose) * Structured and scheduled feeding crucial in appropriate feeding atmosphere * Consider nasogastric feedings if weight gain by other methods is insufficient within 4–6 weeks * Treat organic causes –Diet restriction for food allergy, metabolic disease –Correct electrolyte disturbances –Treat endocrine disease –Remove environmental exposures