Definition. An endemic, chronic, constitutional disease, affecting the digestive, cerebrospinal and cutaneous systems, and usually terminating fatally.
In 1762 Gaspar Casal applied the name of “mal de la rosa” to a disease that had been known in Spain since 1735, and his description of the conditions now known as pellagra (so named by Frappoli in 17760 is still classical in many particulars. For many years this disease has been endemic in certain sections of southern Europe, especially in northern Italy and Romania, in Egypt and Mexico; and in addition isolated instances of its occurrence have been noted in many widely scattered sections of the globe. Although sporadic cases have been reported in our country since 1864, it is only since the report of Seavey in 1907, concerning cases in Alabama, and the investigations of Drs. Babcock and Waston of Columbia, s.C., which culminated in their trip to Italy in the summer of 1908 to establish the identity of the suspected American and of the Italian types of pellagra, that a widespread interest in the disease has arisen, not limited to dermatologists and alienists, but among general practitioners and health officials, especially of the affected section of the United States. This interest is due not doubt to the alarming increase in the number of case of an almost acute epidemic type which have been found in South and North Carolina and Georgia, as well as in numerous well-scattered sections of twenty-two States. It is estimated that there are at least 10,000 cases now present in the United States and they are here to stay and increase unless prompt and efficient steps are taken to discover the causal factors.
Symptoms. Pellagra can present a multiplicity of symptoms, some of which are fairly characteristic, varying with the individual, and the country in which he lives, but the whole picture of the disease course, extending over some months or years, can alone establish a sure diagnosis in the absence of many typical acute cases and the lack of confirmatory diagnostic factors, such as the microscope or serum diagnosis. Usually the gastrointestinal and cutaneous symptoms precede the cerebral and cerebrospinal complications. Despite the fact that sporadic cases are being reported constantly, the endemic character of the disease should never be forgotten. Essentially chronic in its course, it shows periods of remission and aggravation in recurrent attacks. There is often an indefinitely described prodromal period covering several winters preceding the spring during which the only apparent onset, includes a peculiar psychic depression, headache, dizziness, lassitude and general weakness, articular and rheumatic pains of trunk, legs, and especially of hands and feet. A coated tongue, gaseous eructations, tense abdomen and diarrhea and eye symptoms occur in a large percentage of case. The skin eruption, considered by itself, is rather, harmless and quite unimportant as compared with the graver gastrointestinal and nervous phenomena, but as a diagnostic feature it is all- important.
The skin involvement may be primary or secondary and presents characteristic symmetry in location, more or less typical color and a well-defined and circumscribed border, especially of the lesions on the neck and hands. Usually the areas exposed to the sun, as the face, neck, upper chest and back, dorsal surface of hands and feet, lower part of the forearms, or even the elbows if exposed, or, as in the case of native Egyptians, the entire body, and rarely unexposed parts show a dermatitis starting not unlike a ptomaine eruption, toxic erythema or sunburn. The term “pellagrous collar” or neck band corresponds to the area exposed to light and is limited by the upper border of the shirt. The lesions appear as diffuse, bright, livid red or dull brownish-red macules which may become greatly swollen and itch when exposed to the sun. Later they become chocolate-colored and gradually disappear or may continue, as in the evolution of a typical dermatitis, developing vesicles, bullae, pustules and crusts. Within from one or three weeks the subactue symptoms disappear and olive-brown pigmentation, desquamation, atrophy and superficial scarring ensue. After a number of these attacks, the affected skin loses its elasticity, is shining, tender, bronze-brown in colour and markedly atrophic. The epidermic is thin and looks like silk. Weakness and emaciation follow, with a dry, red, denuded tongue, marked stomatitis, painful swallowing, an aggravation of cerebral symptoms, delirium, uncontrollable diarrhoea, and death. In some cases that I have seen the dermal symptoms were the principal ones, but in all well-developed types they became minor in importance, but enough has been said to emphasize the gastrointestinal and cutaneous features and their serious import, so lastly, a word concerning the mental symptoms which give rise to the great and necessary interest taken in this disease by the authorities of public insane asylums here and abroad. Melancholia is the common type, showing in mild cases disinclination to thought or action; later, refusal of food and suicidal tendencies. Maniacal symptoms rarely develop but may do so during apparent stupor. The sensorimotor disturbances are of all degrees, even to paralysis.
An acute form of pellagra, typhoid in character, has been described showing a rapid and violent febrile stage with delirium, trismus and opisthotonous, but in many instances this appears to be an acute exaggeration of the chronic course.
Etiology and Pathology. While much original research has lately been devoted to this subject, it may still be said to be sub judice. I believe with Sambon and other investigators that pellagra is a protozoan disease transmitted by the sand fly (simulium) or other insects. The main facts in favor of this theory are its occurrence in rural districts as compared to towns or cities, its presence in the same endemic centers in Italy for over a hundred years, the coincident appearance of the sand fly in these districts, that in infected regions whole families are attacked at the same time, that the arrival of an infected person in a non-infected region does not spread the disease, that children born in an infected region develop the disease while those born after removal to a non- pellagrous vicinity do not, and lastly pellagra is not hereditary or contagious.
For many years the belief that the ingestion of diseased or damaged maize or corn produced pellagra, was accepted as gospel largely due to the thorough investigations of Lombroso, of Turin, who claimed that certain fungi found in maize when exposed to moisture produced in the corn a toxin which taken into the system caused pellagra. On the other hand, many authenticated cases have developed who never ate maize, and in some regions where it is largely grown and eaten there are no pellagrins. Various organisms have been fond by a number of investigators, and Guertin, Nicolas and Jambon are inclined to believe that pellagra is a condition rather than a disease and may be symptomatic of very different diseases, due to impaired nutrition. There is doubt even now that all the cases so widely scattered are identical disease processes, so it would seem that more than one of the etiological factors mentioned, or modifications of them, might prove the true causative agents. Crocker’s alliteration, “peasant life, poverty and polenta plus exposure as an exciting cause,” is true to a large extent of other countries as well as Europe. There is no doubt that the most marked exciting cause is exposure to the sun’s rays, but poverty, poor food and faulty personal and general hygiene are contributory. Women are more commonly attacked and while any age or sex may be involved, the commonest period is between 20 and 50 years of life, but I have seen over twenty case in children younger than 12 years. Blacks are attacked as well as whites and in our country the disease does not limit itself to the poor.
Postmortem examinations have revealed sclerosis of the brain and cord, pachymeningitis, fatty, anemic and atrophic conditions in the internal organs and pigmentary changes. It would seem that the initial changes were in the central nervous system, although the point of entry may be the stomach or intestines. As regards the blood, there is usually found a mild secondary anemia, leukocytosis only rarely, and the differential count negative.
Diagnosis is not difficult if the gastrointestinal, skin and mental symptoms are present in sufficient clearness with the endemic feature added. The three d’s- dermatitis, diarrhoea and dementia-are strikingly suggestive. It may be necessary to consider sunburn, erythema multiforme, vitiligo and eczema, and rarely lepra, syphilis, actinomycosis, dermatitis herpetiformis, acrodynia or any profound toxemia.
Prognosis and Treatment. The former is grave in all but the mildest cases and even these may terminate fatally after a period of five or more years, if not properly cared for and promptly diagnosed.
No treatment of an epidemic or endemic disease can result successfully that does not attempt to isolate a causative factor and then proceed to remove it. Hence, it is that some of our Southern STates and our National Government are now actively engaged in research work along these lines. Large sums have been spent by Italy, Austria, Spain, Portugal and Romania to stamp out this disease. A more careful supervision of the collection, storage and handling of maize products, enforced improvement in the hygienic surroundings of those affected, different climatic conditions when possible, and even segregation are advised. As regards individual cases, hospital treatment should be insisted on when possible, while tonics, a generous diet, massage, galvanism, the high-frequency currents, alcoholic and salt baths, together with remedies variously demanded, are to be thought of. Saline solutions, atoxyl, mercury, arsenic, thyroid extract and the various intestinal ferments have all had their trials but the specific remedy, it may be a serum, is yet undiscovered. Most writers who claim virtue for the indicated remedy, have had absolutely no actual experience in its use, hence I am not inclined to mention every remedy that might be collected by repertory methods but only those which I have had an opportunity to observe. Argentum nit., Arnica, Arsen., Bovista, Phosphorus and Ustilago may be studied.