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

Abstract

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.

Keywords

• desquamative erythroderma (Leiner’s disease)
• young child