ERYTHRODERMA DESQUAMATIVA & DERMATITIS EXFOLIATIVA NEONATORUM

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

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

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.