Erythrodermia desquamativa

Hill, Lewis Webb

doi:10.1016/s0022-3476(34)80021-2

Cited by 6 publications

(14 citation statements)

References 3 publications

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“…2,4,6,23 LD may arise from a deficiency in opsonization of yeast and S. aureus through a deficit of complement factors. [6][7][8][9][10]13,20,27 However, studies differ in terms of the specific complement that is deficient and the degree of functional loss. In 1972, Miller 27 and Jacobs et al 9 separately established a link between C5 dysfunction and LD by showing complete restoration of yeast opsonic activity after the addition of purified human C5 into the sera of several LD patients.…”

Section: Pathogenesismentioning

confidence: 99%

“…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.…”

Section: Introductionmentioning

confidence: 99%

“…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. Widespread dermatologic scaling and exfoliation, in conjunction with these infections, leaves the infant at risk of sepsis and death if untreated.…”

Section: Introductionmentioning

confidence: 99%

“…Leiner's disease (LD), also known as erythroderma desquamativum (ED), is a life‐threatening condition that arises within the first few months of life and lasts several weeks to months 1‐8 . 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 6‐13 .…”

Section: Introductionmentioning

confidence: 99%

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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: Pathogenesismentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

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

Sanghvi

Schwartz

2020

Dermatologic Therapy

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“…In the American literature, case reports have been scarce. 6 So it is assumed that cases of typical "Leiner's disease" are uncommon or rarely observed in America.7 During the month of January, 1954, four cases of erythroderma desquamativum came under my observation at the Air Force Hospital in Wiesbaden, Germany. These cases were similar to the original ones described by Leiner ; however, certain characteristics were different.…”

mentioning

confidence: 99%