ERYTHRODERMA DESQUAMATIVA
This
disease was first described by Leiner in 1908 and since then has been mentioned
frequently as occurring abroad but rarely has been observed in this country.
According to Leiner, the disease occurs chiefly in nursing infants as a general
exfoliative dermatitis with marked redness of the
skin and scaling, usually suggesting a severe type of seborrheic dermatitis.
The lesions involve particularly the anogenital region, scalp and face but are
also diffuse on the trunk and extremities. At first there is a diffuse
inflammatory redness that may cover the whole body, which later becomes covered with grayish-white scales that may
be fine and branny, or very large so that sheets are exfoliated. The
supraorbital areas are red and swollen and may be crusted. In fact, as the
process develops there may be general exfoliation, cracking, and thickening of
the skin. These infants usually are in a
poor general condition. They may be in a state of athrcpsia, and
diarrhea is commonly present. There is general glandular enlargement. The nails
are destroyed. About one third of the cases die.
At autopsy Leiner found degeneration of the heart muscle, fatty degeneration and infiltration of the liver, and
catarrhal changes in the intestinal mucosa. In the skin there were a dilatation
of vessels in the papillary region and an
increase of leukocytes, with slight inflammation, edema, and parakeratosis
of the epidermis.
The
disease is probably a severe type of erythroderma due to pyogenic cocci and
superimposed upon seborrheic dermatitis.
DERMATITIS EXFOLIATIVA NEONATORUM
DERMATITIS EXFOLIATIVA NEONATORUM
This condition, which is also
known as keratolysis neonatorum or Bitters disease, is a rare septic
exfoliative condition of the newborn which runs a severe course and has a high
mortality. The onset is usually during the
first few weeks of extrauterine life, being marked by localized redness about
the mouth which becomes universal and is accompanied by exfoliation and eczematization. Vesicles, bullae, and
impetiginous lesions may be present, and cultures from these have shown
hemolytic streptococci. The disease is
possibly related to impetigo of the newborn. Wiener reported two cases
in fraternal twins that started simultaneously at 5 weeks of age and suggested
that an attack of severe poison ivy dermatitis near the end of the mother's pregnancy produced antibodies that, passing through
the placenta into the bodies of the twins, could account for the occurrence of
the exfoliative dermatitis in them. The skin of newborn and, particularly,
premature infants is refractory to antigen antibody stimuli which might account
for the delayed onset.
Dermatitis exfoliativa neonatorum
is commonly seen in vigorous and breast-fed infants. The first lesions usually
occur in the course of the second week after birth. Although the earliest
lesions generally appear as red spots on the lace, particularly about the mouth and on the chin, the initial patches may rarely occur on other
areas such as the neck, shoulders and chest. Generally the erythema spreads
eccentrically over the lower parts of the face and then in two or three days
over the rest of the body. Fissures radiate about the mouth. The skin becomes
scarlet, and vesicles and bullae
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Dermatitis exfoliativa neonatorum |
may be superimposed upon it. Denudcment of the superficial
layers ol the epidermis in large pieces may begin before the erythema has
become generalized. Yellow crusts are .sometimes present. Small vesicles may
occur on the lips. Stomatitis, rhinitis and corneal ulcers have been reported.
In uncomplicated cases there is usually no fever but secondary infections such
as-subcutaneous abscesses, pyoderma, gangrenous lesions, and bronchopneumonia
are frequently observed.
This disease must be differentiated
from erythroderma exfoliativa (Lciner), pityriasis rubra, impetigo neonatorum,
erythroderma ichthy osiforme congenitum, epidermolysis bullosa congenitalis and
congenital syphilis. Leiner's disease is not contagious and begins in the
anogenita) region in the skin folds as an erythematous complication of
dermatitis seborrheica. It usually disappears after a duration of three or four
weeks. No bullae are observed. However, Cole believes after careful study of
eleven cases that both Ritter's and Leiner's disease are variants of the same
disease entity. In erythroderma ichthyosiforme congenitum, the first areas
affected arc. the face and the articular folds and there may be plantar and
palmar keratoderma.
Treatment is by antibiotics, and other
remedies suggested for impetigo neonatorum.