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

Abstract

Leiner's disease (LD) is a rare and serious syndrome of infantile erythroderma of severe and progressive generalized seborrheic‐like dermatitis, recalcitrant diarrhea, malabsorption and wasting, and recurrent local and systemic infections. The purpose of this study is to provide an updated review on management with a summarized review of available peer‐reviewed articles on LD. The mechanisms underlying this disease process remain unclear. The diagnosis includes demonstration of deficient opsonic activity along with the clinical tetrad of erythroderma, persistent gastrointestinal disturbance, superimposed bacterial or candidal infection, and marked wasting. An important correlation between LD and defective yeast and Staphylococcus aureus opsonization has been established. For the familial form of LD, an association of either complement three deficiency or complement five dysfunction has been made. LD should be distinguished from other types of infantile erythroderma, including Omenn syndrome. Treatment includes fluid and nutrition replacement, antibiotics to control infection, and fresh‐frozen plasma therapy. The prognosis is unclear; it depends on treatment. LD is a life‐threatening condition that requires prompt identification and hospitalization. Affected infants who receive vigorous treatment not only have the prospect of surviving, but also generally lead a normal life after infancy.

Leiner’s Disease (Erythroderma Desquamativum) in a Baby Boy

“…possible etiologies include C3, C4, and biotin deficiency. 28 Other immune system diseases are also common in infants with Leiner's disease. 1,2,3,4,13 Causes of deficiency in complement components can be primary or secondary ones.…”

“…6 The clinical tetrads of Leiner's disease include generalized erythroderma resembling seborrheic dermatitis, recurrent secondary infection with S. aureus, Candida, or Gramnegative bacteria, persistent malabsorption diarrhea, and failure to thrive. 28…”

“…Common ones included clinical tetrad and found opsonization defects of Baker's yeast and S. aureus, while less common ones included biotin deficiency as evidenced by a significant response to biotin replacement therapy. 28 Diagnosis is made based on clinical symptoms. Typical clinical symptoms are reddish seborrheic lesions in the scalp, the area around the mouth, and the gluteal region.…”

“…1 The histological changes of the skin in Leiner's disease remain unclear but resemble erythroderma and most closely resemble seborrheic dermatitis, which are acanthosis, parakeratosis, hyperkeratosis, papillary edema, and mild lymphocytic infiltration in the epidermis, papillary, and deeper perivascular layers. 28 Laboratory tests can be ordered to confirm the diagnosis, which includes measurement of complement levels, immunoglobulin levels, and bacterial cultures. 9 Anemia, dysgammaglobulinemia with low IgG and high IgM, and leukocytosis with normal humoral and cellular immunity can be found.…”

Pediatric Dermatitis Seborrhoica - A Clinical and Therapeutic Review

“…This is a pediatric emergency, and patients need to be hospitalized. [ 8 ] A rare complication of DS is eczema herpeticum. [ 9 ]…”