Erythroderma desquamativa of the newborn infant

Leiner's disease (erythroderma desquamativum): A review and approach to therapy

“…The common causes of death from LD are meningitis, pneumonia, nephritis, and S. aureus sepsis. Overwhelming infection proved fatal in at least 10% of cases 14,25 . Without adequate symptomatic treatment, the mortality exceeds 50% 4,9,10,18,25,38 …”

“…5,27 At this stage, there is no fever, chills, pruritus, Nikolsky sign, nor changes in the oral mucosa, although there may be generalized soft nontender lymphadenopathy. 5,12,14 Within 2 to 6 days of scaling, the extensive exfoliation in the infant can lead to critical complications, such as infections, impaired thermoregulation, and severe fluid loss. 19 Gastrointestinal symptoms of LD typically manifest subsequent to the cutaneous changes.…”

“…[1][2][3][4][5][6][7][8] It is a syndrome characterized by a clinical tetrad of severe, widespread, seborrheic-like erythroderma beginning on the scalp or diaper area, followed by a persistent gastrointestinal disturbance, marked wasting and weight loss, and recurrent staphylococcal and candidal infections. [6][7][8][9][10][11][12][13] Although originally reported in 1908 as a rare, autosomal dominant (AD) trait associated with a defect in yeast opsonization from dysfunctional complement 5 (C5), the "LD" phenotype may occur in infants with a variety of immunodeficiency disorders, such as complement 3 (C3) or complement 4 (C4) dysfunction, severe combined immunodeficiency (SCID), hypogammaglobulinemia, and hyperimmunoglobulinemia E. 2,3,6,14,15 Austrian pediatrician Carl Leiner 7 in his original series during 1908 concluded that LD is a potentially fatal systemic condition that has a genetic link, a correlation with breastfed infants, and characteristic severe gastrointestinal disturbances. Later works confirmed the existence of both a familial and nonfamilial form of LD, but did not substantiate breastfeeding as a consistent finding.…”

“…It is a syndrome characterized by a clinical tetrad of severe, widespread, seborrheic‐like erythroderma beginning on the scalp or diaper area, followed by a persistent gastrointestinal disturbance, marked wasting and weight loss, and recurrent staphylococcal and candidal infections 6‐13 . Although originally reported in 1908 as a rare, autosomal dominant (AD) trait associated with a defect in yeast opsonization from dysfunctional complement 5 (C5), the “LD” phenotype may occur in infants with a variety of immunodeficiency disorders, such as complement 3 (C3) or complement 4 (C4) dysfunction, severe combined immunodeficiency (SCID), hypogammaglobulinemia, and hyperimmunoglobulinemia E 2,3,6,14,15 …”

“…Later works confirmed the existence of both a familial and nonfamilial form of LD, but did not substantiate breastfeeding as a consistent finding. 8,9,12,14,[16][17][18] The hereditary form has been frequently associated with complement C3 and C5 dysfunction, yielding defective bacterial and yeast opsonization for phagocytosis. [8][9][10]15,16,19 During infancy, when the innate immune system is the primary defense, this inability to opsonize renders the infant highly susceptible to recurrent Staphylococcal aureus (S. aureus), gram-negative bacteria, and Candida infections.…”