Leiner's Disease

Lynch, Francis W.

doi:10.1001/archderm.1957.01550170097016

Cited by 4 publications

(22 citation statements)

References 5 publications

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“…It typically becomes apparent within the first month, starting with severe erythroderma and followed by overwhelming diarrhea 7 . A higher incidence in females compared to males has been reported, as well as in infants who are breastfed 5,7,18,25 . Although no geographical association has been described with LD, a majority of cases seem to originate from the Central Europe 12,16,23 …”

Section: Epidemiologymentioning

confidence: 99%

“…The pathogenesis of LD remains unclear, but it has been attributed to several etiologies (Table 1). LD has been correlated with a malfunction in opsonization of yeast and S. aureus and is possibly associated with Omenn syndrome, other forms of SCID, complement C3 and C5 deficiency, hypogammaglobulinemia and hypergammaglobulinemia E. 3,8,12,14,18,26,27 Other studies have also suggested an association with familial atopy, 14,18 celiac disease, 28 Langerhans cell histiocytosis, 29 metabolic acidosis, 11 and several nutritional factors such as biotin deficiency. 2,4,6,23 LD may arise from a deficiency in opsonization of yeast and S. aureus through a deficit of complement factors.…”

Section: Pathogenesismentioning

confidence: 99%

“…4,18,23,25,27 While the classification of the diarrhea is found to be malabsorptive and resistant to treatment, the relationship between the erythroderma and the diarrhea is unknown. 18,27 Some studies suggest that the deficiencies of biotin, pyridoxine, and riboflavin increase the scaly, desquamative seborrheic-type eruptions manifested in this condition. 4,23 The severity of the gastrointestinal symptoms tends to match that of the dermatologic manifestations in LD.…”

Section: Clinical Manifestationsmentioning

confidence: 99%

“…While most studies of LD were from Central Europe, the American pediatrician Hill 1 observed 21 examples of erythroderma among 800 infantile eczema patients in the United States in 1934 13,18 . Hill, however, regarded LD as “a symptom complex occurring in certain seborrheic and eczematous infants”, rather than as an erythroderma alone 18 .…”

Section: Historymentioning

confidence: 99%

“…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 8,9,12,14,16‐18 . The hereditary form has been frequently associated with complement C3 and C5 dysfunction, yielding defective bacterial and yeast opsonization for phagocytosis 8‐10,15,16,19 .…”

Section: Introductionmentioning

confidence: 96%

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

Sanghvi

Schwartz

2020

Dermatologic Therapy

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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.

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Section: Epidemiologymentioning

confidence: 99%