Karl Leiner (1871-1930) was a world-renowned Austrian pediatrician (Ruhrah,
1935). Among his great contributions to pediatric, he is credited for describing
children with a similar clinical phenotype (Leiner, 1908a,
b) which was later known as Leiners syndrome (http://www.whonamedit.com/synd.cfm/2369.html).
Leiners syndrome: Leiners syndrome is a systemic disease
diagnosed in infancy. The main cardinal features of this syndrome are (1) generalized
dermatitis, (2) intractable diarrhea and (3) marked wasting. The other features
that might be associated with this syndrome are recurrent local and systemic
infections, usually of gram-negative etiology, central nervous system deficiency
and failure to thrive (http://www.whonamedit.com/synd.cfm/2369.html).
In 1908, Leiner described 43 children, over five years in children hospital
Vienna. Those were affected with similar clinical features (Leiner,
In his first description, Leiner noticed that the breast-fed infants are most
frequently affected and the condition of the children improved clinically when
they fed cow milk (Leiner, 1908a, b).
The syndrome may be present at birth but more commonly develops within the first
few months of life. It has rapid onset in 2nd to 4th month of life. It begins
as seborrhoic eczematoid lesions of the scalp and face or the gluteal region,
eventually spreading to other areas. The affected area is bright red and may
look swollen. Infants appear uncomfortable but do not itch. In addition, the
affected children will have fever, diarrhea, generalized lymphadenopathy, edema,
albuminuria and local skin infections and may develop more severe infections
that may lead to pneumonia, meningitis and septicemia which may ends in death.
Cases with Leiner syndrome were treated with hospitalization, to manage fluid
and heat loss, Bland emollients and adequate nutrition. Biotin, a water-soluble
vitamin that is found naturally in foods such as liver, kidney, meat, milk,
egg yolks and vegetables and Essential Fatty Acids (EFAs) were also, found to
be useful in treating Leiner disease. This syndrome was thought to be caused
by complement component 5 deficiency (OMIM No. 609536). However, case reports
have described it in relation to deficits in either C3 or C4.
It seems that, deficiency with hypogammaglobulinemia or other lymphoid deficiency,
are also required for its expression. The current understanding is that, Leiner
syndrome is a separate entity and it is merely, a clinical phenotype that can
be caused by several nutritional and immunodeficiency's disorders (http://www.whonamedit.com/synd.cfm/2369.html).
Therefore many authors recommend that this term should be avoided as it is a
description phenotype caused by many causes. On other hand, some authors suggest
reserving this term, Leiners disease to erythrodermic seborrheic dermatitis
in infants which associated with immunodeficiency disorders, diarrhea and failure
Karl Leiner (1871-1930): Carl Leiner, is an Austrian pediatrician, born
on January 23, 1871, Flohau, Bohemia (http://www.whonamedit.com/doctor.cfm/2095.html).
He was an unusual combination of clinician, practitioner, consultant and scientific
investigator. He combined the skills of a consultant physician with that of
a laboratory worker and scientific investigator (http://www.whonamedit.com/doctor.cfm/2095.html).
He was educated in Praha and Vienna (http://www.whonamedit.com/doctor.cfm/2095.html)
and Ruhrah (1935). He was graduated in Vienna in 1898
and began at once to devote his time to pediatrics (Ruhrah,
1935). He study in the skin clinic as well as the pathological institute
at Vienna. He became professor of Pediatric in 1922 and from 1920 was director
of the Mautner-Markhof Children's Hospital in Vienna (http://www.whonamedit.com/doctor.cfm/2095.html)
and (Ruhrah, 1935). Leiner took a special interest in
infectious diseases of children and was the first to use an intracutaneous technique
for the vaccination of smallpox and was the first to recognize its relationship
to post-vaccinal encephalitis. He investigated poliomyelitis and other viruses
and bacteria at the Weichselbaum Institute (http://www.whonamedit.com/doctor.cfm/2095.html).
He was the first to call the attention to the annular erythema observed in cases
of rheumatic endocarditis (Ruhrah, 1935). Leiner died
on April 24, 1930, in Vienna (http://www.whonamedit.com/doctor.cfm/2095.html).