Leiner Syndrome

At a glance

It is a severe skin disorder characterized by erythroderma with an onset during infancy. The presence of seborrheic dermatitis, diarrhea, and recurrent systemic infections defines this medical condition. Other features include large patches of red skin, and delayed central nervous system development due to complement C5 deficiency (acquired deficiency in most patients).

Synonyms

Leiner’s Disease, Leiner Phenotype, Complement C5 Deficiency; Dermatitis Exfoliativa Generalisata; Desquamative Erythroderma in Infants; Eczema Universale Seborrhoeicum; Erythrodermia Desquamativa of Leiner; Erythrodermia Desquamativa in Infants; Erythroderma Desquamativum; Erythrodermic Seborrheic Dermatitis in the Trianon of Life; Exfoliative Dermatitis; Seborrheic Diathesis in Infants; Leiner-Moussous Disease.

History

First described in 1908 by Karl Leiner, an Austrian pediatrician, who reported the association of acquired exfoliative erythroderma, dermatitis, diarrhea, and failure to thrive in 43 infants. The term Leiner Phenotype is often used as it refers to the triad of medical symptoms that constitutes a phenotype.

Incidence

The exact incidence remains unknown. Prevalent during the first 2 months of life. It is reported that male and female infants are affected equally. Breast-fed infants are most frequently affected.

Genetic inheritance

Not a genetic disorder in most patients. Gene map locus of hereditary complement C5 deficiency is 9q34.1.

Pathophysiology

Leiner disease includes a heterogeneous group of disorders. A common feature of these disorders is temporary or permanent (hereditary forms) plasma deficiency in complement C5 activity resulting in decrease of opsonic properties of plasma (deficiency in phagocytosis).

Diagnosis

The clinical presentation establishes the diagnosis. It is caused by a deficiency in complement C5 activity in plasma.

Clinical aspects

Syndrome characterized by desquamation (severe generalized seborrheic dermatitis), erythroderma, recurrent local and systemic infection, severe diarrhea, marked wasting, central nervous system deficiency, and failure to thrive. It begins as seborrheic eczematoid lesions of the scalp and face or the gluteal region, eventually spreading to other areas. Keratitis and corneal ulcers may occur. Caused by complement C5 deficiency in infancy; rapid onset in second to fourth month of life.

Precautions before anesthesia

Elective anesthesia and surgery should be postponed until skin covering is restored in relevant areas and the infection is controlled.

Anesthetic considerations

Patients with Leiner disease are prone to severe bacterial contamination as a consequence of their extensive skin lesions and deficiency in phagocytosis. Large spectrum antibioprophylaxis should be considered at the time of anesthesia. Because of extended skin lesions, there is a danger of systemic toxicity with topical agents (especially disinfectants and skin preparation solutions).

Pharmacological implications

Use topical agents with extreme care because ...