Definition. A rare inflammatory disease of the skin, occurring usually during pregnancy, characterized by the appearance of grouped miliary pustules.
Symptoms. It is principally to Kaposi that we are indebted for the description of this disease. There are less than forty recorded cases of this rare condition but they follow a course so typical that it is necessary to group them as a distinct entity. The prodromata consist of chills, high fever, dry tongue, vomiting and delirium and these may appear before the onset of fresh outbreaks of the eruption. The temperature, however, may remain continuous or become remittent. The lesions consist of pin-head-sized pustules, appearing in patches the size of a lentil to that of a penny, the pustules being opaque at first, later greenish-yellow, usually situated on a red and swollen base. Within a day or two they dry into dirty brown crusts, and new pustules are formed around them in one or more closely placed circles; these in turn dry and add to the crust. Thus the patches, usually beginning first on the inner aspect of the thighs, groin, axillae or around the navel, may spread over large areas and by coalescence with adjacent patches the entire surface may be involved in three or four months’ time. The skin as a whole is hot, swollen, covered with scales or denuded and oozing but it never ulcerates. Excoriations and fissures will frequently be found bordered by circles of pustules. The mucous lining of the mouth and throat may present circumscribed, gray patches with a depressed center.
Etiology and Pathology. The disease is looked upon as pyemic or septicemic in character. While the etiological factors are obscure, the vast majority of case have been observed in pregnant women. Hence, the pathology is probably akin to that of herpes gestationis. The fact that there was an absence of septic, uterine or other disease in some cases supports the supposition that it is reflex in origin and trophic in its result on the skin. Postumortem examinations have shown evidence of nephritis and phthisis. Also there heave been noted pustules and ulcers in the folds of the esophagus, most numerous near the cardiac portion of the stomach.
Diagnosis should not present any difficulty, especially if the patient were a pregnant woman. Dermatitis herpetiformis presents multiform lesions and usually does not run such a persistent course. Pemphigus vegetans in its advanced stage might bear a resemblance to impetigo herpetiformis but the former will give a history of a bullous onset while the latter starts with grouped pustules.
Prognosis and Treatment. The outlook is very grave, only a few cases having recovered. Treatment is practically along the same lines as suggested for dermatitis herpetiformis, with the accent upon the selection of a drug to meet the constitutional symptoms. General antisepticemic treatment would seem to be indicated, and the uterus should be emptied of its contents, Iris ver., Clematis, Croton tig., Kali brom., Lachesis, and Nat.mur. may be studied.