Leiner's disease is a systemic disease, a skin disorder and extends to erythroma, typically diagnosed in early infancy. Leiner’s disease is characterized by a long lasting seborrhea dermatitis associated with increased likelihood to infection. Other characterizations found on newborns with Leiner’s disease are a patch or a large patch of red skin normally on the bum and spreads to the rest of the body.
This disease is also listed as a "rare disease", meaning that a small percent of the population , less than 200,000 people in the United States, will have this disorder.

Symptoms

Symptoms include severe seborrheic dermatitis of the scalp, severe diarrhea, recurrent local and systemic infection, central nervous system problems, and failure to thrive

Failure to thrive

Failure to thrive is a medical term which is used in both pediatric and adult human medicine, as well as veterinary medicine ....

. Other symptoms also include scaling on the trunk and limbs, red patches of skin on parts of the body that bend, fevers, reduced blood protein levels, thick red skin patches, peeling of the skin, itching, corneal ulcers. wasting of the lymph nodes, underdeveloped lymphatics, anemia, wasting, and nervous system deficiency. The disease may then spread to the rest of the epidermis with the appearance of crusty, dry, moist or greasy scaling on the scalp. Scaling could also appear behind the ears, nose or eyebrows, or around the mouth; peeling of the skin may also happen in these areas. If left untreated, the skin infections will cause loss of protein or electrolytes. Leiner’s Disease may also be accompanied by a systemic reaction that is most evident in its gastrointestinal manifestation.

It is caused by a deficit of the complement protein, C5

Complement component 5

Complement component 5 is a protein that in humans is encoded by the C5 gene.Complement component 5 is involved in the complement system. It is cleaved into C5a and C5b:* C5a plays an important role in chemotaxis....

; however, case reports have described it in relation to deficits in either C3 or C4

Complement component 4

Complement component 4 is a protein involved in the complement system.It is cleaved into proteins 4a and 4b.* C4a is an anaphylatoxin.* C4b forms part of C3-convertase, in conjunction with 2a:* C4b can bind CR1....

.

Diagnosis

This disease is often found during the first two months of an infants life, breast-fed infants with a higher chance. Male and female infants are affected equally.

Treatment

Hospitalization for the diseased person is suggested because of the controlled environment because it may prevent nutritional deficiencies and skin infections. A decrease in severity of symptoms usually happens after a few weeks when treated redness and scaliness usually do not recur. In 10 percent of cases, the result of uncontrolled infections or severe electrolyte loss may be fatal.

Epidemiology

The etiology of this disease is unknown, some infants may have a severe case, others may have immunodeficiency.

See also

• List of cutaneous conditions
• Erythrodermia Desquamativa Leiner
• Leiner-Moussous Desquamative Erythroderma
• Severe Infantile Dermatitis
• Ritter disease
• dermatitis exfoliatva neonatorum