Clinical case of desquamative erythroderma (Leiner’s disease) in a young child

We present a clinical case based on our observation of an infant with rare disease, desquamative erythroderma (Leiner’s disease). This disease is characterized by dermatitis in infants, usually found during the period of one week to four months of an infant’s life. The causes of desquamative erythro...

Full description

Bibliographic Details
Main Authors: О.М. Shulhai, І.Y. Shostak, А.B. Kabakova, О.І. Khara
Format: Article
Language:English
Published: Zaslavsky O.Yu. 2017-03-01
Series:Zdorovʹe Rebenka
Subjects:
Online Access:http://childshealth.zaslavsky.com.ua/article/view/100998
_version_ 1818974373562810368
author О.М. Shulhai
І.Y. Shostak
А.B. Kabakova
О.І. Khara
author_facet О.М. Shulhai
І.Y. Shostak
А.B. Kabakova
О.І. Khara
author_sort О.М. Shulhai
collection DOAJ
description We present a clinical case based on our observation of an infant with rare disease, desquamative erythroderma (Leiner’s disease). This disease is characterized by dermatitis in infants, usually found during the period of one week to four months of an infant’s life. The causes of desquamative erythroderma are: action of allergens in breast milk; sensitizing effect of pathogenic microflora (cocci and fungi); metabolic disorders; vitamin deficiencies, especially a significant deficiency of biotin (vitamin B7), vitamin A and carotenoids in breast milk; enzymopathies. It was also suggested that compromised immune system, such as a deficit of the system complement proteins C4 and C5, play a role in development of this disorder. Initial clinical signs of desquamative erythroderma resemble seborrheic dermatitis, since the observations include erythematous lesions of the scalp with significant scaling and yellowish layers, which subsequently spread to the skin of the forehead and cheeks. The skin is dry, swollen, peels off in large pieces, causing thinning out of hair, or sometimes bald spots. In addition, the disease can initially manifest as swollen, erythematous, infiltrated areas resembling diaper rash, with numerous layers of serous and purulent crusts. These sites first appear in the large folds of skin (usually the area of groin and buttocks), and then quickly spread across the skin, sometimes leaving small patches of unaffected skin. As a result of proliferation of pathological process in the skin folds, oozing erosion surfaces can form, sometimes covering rather large patches. Medical condition of the child with this disorder is severe, initially against the background of normal temperature or low-grade fever and is accompanied by symptoms of dyspepsia (diarrhea, regurgitation, and vomiting). Proliferation of skin symptoms is associated with developing anemia and hypoproteinemia, while general analysis of the blood shows decrease in the number of eosinophils or their absence. If the body temperature increases to fever, this points to emergence of complications, such as otitis media, pneumonia, phlegmon, pyelonephritis, and sepsis. All these manifestations of desquamative erythroderma can lead to the growth of pathogen microflora and deplete immune reserves, resulting in death. However, timely diagnosis of this disorder and appropriate treatment help to ensure a favorable prognosis. Therefore, awareness of pediatricians about desquamative erythroderma (Leiner’s disease), its causes and clinical course is important for early diagnosis and prescribing an effective treatment.
first_indexed 2024-12-20T15:39:02Z
format Article
id doaj.art-f12279c5a9f3437899fe4e6c7fa44111
institution Directory Open Access Journal
issn 2224-0551
2307-1168
language English
last_indexed 2024-12-20T15:39:02Z
publishDate 2017-03-01
publisher Zaslavsky O.Yu.
record_format Article
series Zdorovʹe Rebenka
spelling doaj.art-f12279c5a9f3437899fe4e6c7fa441112022-12-21T19:35:17ZengZaslavsky O.Yu.Zdorovʹe Rebenka2224-05512307-11682017-03-01122.129730010.22141/2224-0551.12.2.1.2017.100998100998Clinical case of desquamative erythroderma (Leiner’s disease) in a young childО.М. Shulhai0І.Y. Shostak1А.B. Kabakova2О.І. Khara3State Institution of Higher Education “I. Horbachevskyy Ternopil State Medical University”, Ternopil, UkraineRegional Children’s Clinical Hospital, Ternopil, UkraineRegional Children’s Clinical Hospital, Ternopil, UkraineState Institution of Higher Education “I. Horbachevskyy Ternopil State Medical University”, Ternopil, UkraineWe present a clinical case based on our observation of an infant with rare disease, desquamative erythroderma (Leiner’s disease). This disease is characterized by dermatitis in infants, usually found during the period of one week to four months of an infant’s life. The causes of desquamative erythroderma are: action of allergens in breast milk; sensitizing effect of pathogenic microflora (cocci and fungi); metabolic disorders; vitamin deficiencies, especially a significant deficiency of biotin (vitamin B7), vitamin A and carotenoids in breast milk; enzymopathies. It was also suggested that compromised immune system, such as a deficit of the system complement proteins C4 and C5, play a role in development of this disorder. Initial clinical signs of desquamative erythroderma resemble seborrheic dermatitis, since the observations include erythematous lesions of the scalp with significant scaling and yellowish layers, which subsequently spread to the skin of the forehead and cheeks. The skin is dry, swollen, peels off in large pieces, causing thinning out of hair, or sometimes bald spots. In addition, the disease can initially manifest as swollen, erythematous, infiltrated areas resembling diaper rash, with numerous layers of serous and purulent crusts. These sites first appear in the large folds of skin (usually the area of groin and buttocks), and then quickly spread across the skin, sometimes leaving small patches of unaffected skin. As a result of proliferation of pathological process in the skin folds, oozing erosion surfaces can form, sometimes covering rather large patches. Medical condition of the child with this disorder is severe, initially against the background of normal temperature or low-grade fever and is accompanied by symptoms of dyspepsia (diarrhea, regurgitation, and vomiting). Proliferation of skin symptoms is associated with developing anemia and hypoproteinemia, while general analysis of the blood shows decrease in the number of eosinophils or their absence. If the body temperature increases to fever, this points to emergence of complications, such as otitis media, pneumonia, phlegmon, pyelonephritis, and sepsis. All these manifestations of desquamative erythroderma can lead to the growth of pathogen microflora and deplete immune reserves, resulting in death. However, timely diagnosis of this disorder and appropriate treatment help to ensure a favorable prognosis. Therefore, awareness of pediatricians about desquamative erythroderma (Leiner’s disease), its causes and clinical course is important for early diagnosis and prescribing an effective treatment.http://childshealth.zaslavsky.com.ua/article/view/100998desquamative erythroderma (Leiner’s disease)young child
spellingShingle О.М. Shulhai
І.Y. Shostak
А.B. Kabakova
О.І. Khara
Clinical case of desquamative erythroderma (Leiner’s disease) in a young child
Zdorovʹe Rebenka
desquamative erythroderma (Leiner’s disease)
young child
title Clinical case of desquamative erythroderma (Leiner’s disease) in a young child
title_full Clinical case of desquamative erythroderma (Leiner’s disease) in a young child
title_fullStr Clinical case of desquamative erythroderma (Leiner’s disease) in a young child
title_full_unstemmed Clinical case of desquamative erythroderma (Leiner’s disease) in a young child
title_short Clinical case of desquamative erythroderma (Leiner’s disease) in a young child
title_sort clinical case of desquamative erythroderma leiner s disease in a young child
topic desquamative erythroderma (Leiner’s disease)
young child
url http://childshealth.zaslavsky.com.ua/article/view/100998
work_keys_str_mv AT omshulhai clinicalcaseofdesquamativeerythrodermaleinersdiseaseinayoungchild
AT íyshostak clinicalcaseofdesquamativeerythrodermaleinersdiseaseinayoungchild
AT abkabakova clinicalcaseofdesquamativeerythrodermaleinersdiseaseinayoungchild
AT oíkhara clinicalcaseofdesquamativeerythrodermaleinersdiseaseinayoungchild