(Elephantiasis Arabum; Pachydermia; Elephantiasis Indica; Bucnemia tropica; Morbus elephus; Elephant leg; Barbados leg)
Definition. – A chronic hypertrophic disease of the skin and subcutaneous tissues, due to the local obstruction of blood and lymph vessels, resulting in enormous enlargement of the affected part.
Symptoms. – Two types of this disease are described, the endemic form, found in tropical countries and the sporadic, occurring in temperate climates. While these differ in their initial origin they are practically identical in their effect on the parts involved. This varies from a slight thickening of the whole or parts of the skin and subcutanous tissues to enormous enlargements of the same. The disease usually attacks the leg or foot, rarely bilaterally, however. The thighs, buttocks, scrotum, penis, labia, clitoris, upper extremities, individual parts of the face and, exceptionally, other regions may be involved. In either the endemic or sporadic cases it may start as a genuine erysipelas or may stimulate the latter in appearance and sensation, showing infiltration, swelling, vivid redness and tenseness of the skin. The endemic form often presents antecedent febrile disturbances (elephantoid fever) with excessive sweating, pronounced lumbar and joint pains, nausea, vomiting and chilliness. When the acute symptoms subside the parts are usually felt somewhat enlarged. Subsequent attacks of varying intensity and frequency add to the enlargement until the part involved may become two or more times its natural size. The genitals, especially the scrotum, show the most pronounced growth. This may be noted in the illustration of a case which accompanies the text. Other parts of the body seldom show such gigantic increase. Between the exacerbations of the disease the patient may be quite free from suffering beyond the inconvenience of abnormal weight and bulk.
The legs are most often involved and when elephantiasis is well developed, will be seen to be greatly swollen and edematous, hard and resistant to pressure, demonstrating subcutaneous involvement as well as skin hypertrophy. The natural lines of the skin are transformed into deep sulci, especially at the flexures and the joints, while prominent papillae or warty plaques covered with thick, softened or hard epidermis of a reddish-brown or deeper color will give to the skin an irregular uneven appearance.
Fig. 111 – Elephantiasis, sporadic type. The leg affected has been the seat of ten attacks of so-called “milk leg,” incident to pregnancies, scattered over a period of eighteen years. The conditions is now permanent.
Rarely the surface may be smooth or an eczematous condition may complicate the attack with severe itching. Varicose ulcers may develop and adenitis and keloidal growths may appear. In the linear depressions of the skin before mentioned the sweat and oil decompose and, mixed with the macerated epithelium, cover the opposing surfaces with a slimy, offensive fluid. Even scattered scars may be found mingled with other lesions. Rarely the bones are involved in the hypertrophic process, similar to acromegaly. In the endemic form both legs are often affected while in the sporadic type one member only is commonly involved.
Fig. 112 – Elephantiasis, endemic type, due to the filaria sanguinis hominis. Patient is a Chairman of middle age.
Fig. 113 – Side view of the lower extremities and scrotum which are the only parts involved. (courtesy of Dr. E.S. Coburn).
Etiology and Pathology. – While this disease is more frequent in early adult life it is occasionally observed in children and has even been noted as congenital. Males are afflicted in the proportion of three to one of females and the dark races are the more susceptible. Elephantiasis occurs in all parts of the world but the endemic type is limited to malarial regions of the tropical or subtropical countries. Manson has asserted that its geographical distribution is limited to that of the mosquito and at the present writing, it is generally admitted that this insect is an intermediary host for the essential agent, the filaria sanguinis hominis. Unhygienic living, poor food and other depleting influences are doubtless contributing factors but the disease is not contagious nor hereditary. Among the agents which may cause interference with or obstruction of the veins and lymphatics and hence lead to hypertrophic development, the following have been noted in sporadic elephantiasis: various neoplasms, ulcerations, chronic cutaneous diseases, syphilis, gonorrhea, recurrent erysipelas, phlegmasia dolens, diseases of the inguinal glands, local traumatisms or even pressure from too tight and long-continued bandages.
Pathological changes are chiefly the result of lymphatic obstruction which is due as has been intimated to various causes. No doubt the filaria is concerned in the blocking of the lymph vessels in the endemic form but many growths or inflammatory processes may obstruct the blood and lymph vessels in sporadic cases. The seat of the morbid changes is essentially the subcutaneous tissue where the connective tissue elements are mainly involved, but the whole integument may become thickened and often there is papillary, pigmentary, venous and lymphatic enlargement. When the disease has endured for some time the underlying muscles atrophy and show fatty degeneration while occasionally the bones may be enlarged.
Diagnosis – Sporadic elephantiasis may be recognized by the history of recurrent erysipelatous inflammation and the gradual enlargement of the parts. The endemic type may be known by febrile disturbances, its occurrence in tropical countries and by the isolation of the causative parasite which may be recognized by careful examination of the blood, drawn during the night.
Prognosis and Treatment. – Life is not so much endangered by the disease as that the existence of abnormal growth and weight may annoy the sufferer. Relief in the sporadic cases will often depend upon the removal or modification of the cause in the early stage or upon surgical treatment at a later period. It is said that removal from an endemic district will quickly benefit the filarial type, but advanced cases of this type are hard to cure.
Causal treatment must be adapted to each individual case. Patients may be considerably improved by specific hygienic measures, such as removal from the endemic district, better sanitation, good food and tonic treatment. Local conditions of obstruction call for even support with a rubber bandage by day and a wool or cotton roller at night. Surface conditions such as erysipelas, eczema, ulcers and warty growths should receive attention suited to their external and local need, but the routine use of an inunction, except for syphilitic gumma, or of a so called absorbent is not to be recommended. Ligation of the main artery of the leg and nerve stretching have been reported as successful in a number of cases. The galvanic and high-frequency currents and massage may be used generally or locally. Rest and elevation when the legs are involved is serviceable in all cases, and in acute exaggerations of the disease it is absolutely essential. Finally, surgery offers the best relief in cases of localized enlargements. This is especially true and safe in these days of perfected surgical technic. In the early stages of sporadic elephantiasis I have seen splendid results achieved by such remedies as Calcarea fluo., China, Hydrocot., and Silicea, plus attention to causal and local conditions.