(Fibroma fungoides; Inflammatory fungoid neoplasm; Granuloma fungoides; Lymphodermia perniciosa; Sarcomatosis generalis)
Definition – A rare, chronic disease, often characterized by pre-cursory symptoms of an eczematoid, urticarial, erysipeloid or lichenoid type and later by ulcerating fungoid tumors.
Symptoms – Varied lesions, such as have been described in the definition comprise the early premycosic stage. This period may last for two or more years and ordinarily presents one or more red, sharply defined, circinate, non- elevated or slightly raised patches. Itching is usually pronounced, scaling may be present and there is great variance in the size, shape and distribution of the lesions, showing a tendency toward peripheral extension and central clearing. The spread and coalescence of the individual lesions causes large or gyrate patches to form which at the onset are usually seen on the trunk. At the end of this period the infiltration is pronounced and pea-sized or larger nodules and plaques develop, assuming an irregular, circinate or gyrate shape, distinctly elevated and of a pink or reddish-blue color. While these nodules are characteristic of the disease, it is the fungoid stage which is the more typical. These later growths vary in size from a pea to an orange, develop slowly or rapidly into sessile or pedunculated tumors, are of a reddish, purplish color, or of a normal skin tint, usually hemispheric and firm, with a tendency to grouping and to increase and decrease in size in a remarkably short time. At this stage the extremities and face are affected as well as the trunk. There may be at times intense itching of the skin over and around the tumors. But on the development of the ulcerating lesions or mushroom-like growths, the health of the patient which was not affected in the early stages is seriously compromised, and adenitis, diarrhea and general exhaustion cause a fatal ending.
Fig. 193 – Mycosis fungoides of five years’ duration. Eczematous and urticarial lesions were present during the first three years of the process. No effect from careful, persistent treatment, including the X-rays and high- frequency currents (courtesy of Dr. Harlow Brooks).
Fig. 194 – Rear view of Fig. 193, showing the nodular growths very plainly.
Etiology and Pathology – This condition usually affects adults, especially men, beyond the age of forty. It is believed to be an infective granuloma probably due to an unknown microparasite. However, it is not known to be contagious or hereditary. Microscopically the tumors consist of small round cells imbedded in a delicate fibrous matrix, which resembles round-celled sarcoma, lymphosarcoma and the infective granulomata.
Diagnosis – The prodromal eruptions of this affection may resemble the more common diseases, such as eczema, psoriasis or lupus erythematosus. Generally the absence of some diagnostic feature of these diseases will afford ground for doubt: thus, the patches of eczema are not sharply defined or so persistent and usually give rise to more discharge; psoriasis seeks the extensor surfaces and lupus erythematosus the face; the latter is usually unattended with much itching but often with atrophic scarring.
After the tumor-like growths develop and their variable manner of coming and going is noted, little difficulty will be found in differentiating them from other neoplasms of the skin. Multiple pigmented sarcoma is thought by some to be closely related if not the same disease, but it is not preceded by eczematoid eruptions; pruritus is exceptional and the tumors disappear by ulceration and not by resolution, as may be the case in mycosis. Syphilitic gumma are not attended with itching, do not project from the surface so prominently or become pedunculated, and other characteristic signs of syphilis can usually be found. The tubercles of leprosy do not fungate, itch or pass through the rapid changes of mycosis fungoides. Anesthesia is apt to be present and is sometimes extensive in leprosy and its ulcers may be deep and disfiguring; moreover its tubercles always contain the bacillus leprae which is easily found on efficient microscopic examination.
Prognosis and Treatment – The result is generally fatal, although the duration may vary from a few months to fifteen years. The X-rays present the only important therapeutic agent, for their application in a judicious manner will completely control the disease, relieve the itching, disperse the tumors for a while and, if applied as quickly as new-growths appear, may prevent their ulceration. The early stage, including the distressing subjective symptoms, should be treated with soothing lotions and antiseptic ointments as outlined under the treatment of eczema. The fungating tumors may be washed with hot borax or potassium permanganate solutions and dusted over with a non-irritating anti-septic powder such as aristol, iodoform, bismuth or boric acid. Arsenic has long been used hypodermically for this condition and, only recently, salvarsan has been successfully employed. Tonics, a generous diet and careful bathing are always necessary. A well indicated drug would seem to be most essential, even if it accomplished nothing but the relief of transient symptoms. I have used the following to good advantage: Calcarea carb., Kali brom., Lachesis and Phosphorus.