« PreviousContinue »
are a mixture of both acuminate and flat angular papules. Patches form by coalescence. Secondary lesions: vesicles, pustules, eczema are usually present. It never occurs on the genitals. Under the microscope the structure of a tubercle is readily made out.
Lichen nitidus: This rare disease can hardly be confused with lichen acuminatus. It rather resembles lichen scrofulosorum. The lesions are similar in shape and color to the flat variety of papules in the latter disease; there is a complete absence of itching and constitutional symptoms; it is chronic and stationary. Under the microscope the structure is that of a tubercle.
Lichen Hindus (Wilan) is found on the legs of old people and during the course of lichen scrofulosorum. The lesion consists of a hemorrhagic papule.
Lichen syphiliticus at a glance, presents a picture very similar to lichen acuminatus. There is the same papule, only a little paler or yellowish, with its horny plug which does not pull out as readily as in lichen acuminatus. The papules, however, are very numerous and closely packed, and are symmetrically distributed. Their presence on the face, to the hair line, is very striking. They may be preceded by macules. Finally, the history of the case and the presence of associated lesions, as pustules, etc., ought to make the diagnosis certain.
Tertiary papular syphilid resembles lichen acuminatus by its grouping in lines, circles, etc., but its localization, the presence of associated lesions and the history of the case will help the differential diagnosis.
Psoriasis can be mistaken only for universal lichen acuminatus, but the adherent scales which leave bleeding points when removed, the absolutely smooth surface under the scales and the absence of discrete acuminate papules at the border, make the distinction easy.
Psoriasis palmaris ct plantaris occurs late in psoriasis, and only in very severe cases; in lichen acuminatus palmar and plantar lesions may be the first symptoms. There is also less swelling in psoriasis, and the edges are less sharply outlined.
General exfoliating erythrodermia: It may be impossible to distinguish this condition from universal lichen acuminatus. A careful search must be made for discrete papules and the history must be taken into consideration.
Prurigo: The papule is relatively soft without horny plugs. It occurs mainly on the extensor surfaces of the extremities and is of comparatively short duration.
Papular eczema should not be confused with this disease. The papule is soft and there are associated lesions present.
Tar, chrysarobin and pyrogallol rashes: A comedo-like body capping a papule bears some resemblance to the disease in question, but the history of the case and the inflammatory symptoms will make a mistake impossible.
ETIOLOGY OF LICHEN ACUMINATUS
The cause of this disease is unknown. Intestinal putrefaction may cause a weakened resistance against the real causative factor or may generate the latter. Deterioration of the general health is provocative. In the same way nervous strain, worry and fear may be contributing causes. Exacerbations of the disease occur during periods of body depression ( Cunningham).
There is no difference in opinion as to the hyperkeratosis; but writers differ as to whether the infiltration of the cutis is primary or secondary.
Stratum Contemn: There is a general hyerkeratosis, the horny layer is increased from two to ten times. It is composed of wavy, homogeneous, lamellar layers. There is but rarely any parakeratosis, only occasionally a nucleated horny cell can be found, and that at the base only. Conical, globular or cylindrical masses project into the hair follicle, forming the plug. The latter is composed of concentrically arranged lamellar layers, like an onion. The lanugo hair may be retained in the mass, or it may have fallen out and an oblong space is found corresponding to it. The plug may become so large as to pull down the surface skin with it; in that manner two follicles may unite to form a twin follicle. There are two other locations where a plug can be formed, namely, in the sweat gland duct and in the rete pegs. A marked hyperkeratosis and plug formation has been observed in the sweat gland duct, which occasionally leads to sweat gland cysts by occlusion of the outlet. Cornification and horny plug formation also may take place in some of the rete pegs.
The rete malpighii is hypertrophied. The cells of the rete may show slight changes, the nuclei are large, vesicular and separated from the protoplasm. The basal layer may be increased in thickness or it may be missing, and between the cells of the rete malpighii and the corneum there is a space filled with round cells, leukocytes and detritus. The whole process is due to edema.
Papillary Layer: In the early stage, there is vascular dilatation and perivascular infiltration in the papillae leading downward even to the subcutis. In the second stage the infiltration becomes diffuse. In severe cases the infiltration may extend into the epithelial layer. In the third stage the diffuse character is lost and the infiltration is localized about the hair follicle, also about the sweat and sebaceous glands to a lesser extent.
Character of the Cells: (a) Mononuclear leukocytes, (b) Large spindle-shaped fibroblasts, (c) Few plasma cells, (d) A fairly large number of mast cells.'
Arrectores pilorum are hypertrophied. Fibrous, elastic and nerve tissues are normal.
REPORT OF CASE
History.—S. R., aged 40, a moving picture operator, came to the Cornell Dispensary, Jan. 16, 1916.
He stated that in June, 1915, large red blotches appeared in his groins and spread to the back, the abdomen and the chest. Simultaneously with the eruption there came an intense itching. The latter appeared rather suddenly. The itching was most intense in places where parts come in contact with each other and where perspiration is more profuse, as in the groins, axillae and about the scrotum.
He does not remember having had any fever. As to headaches, nothing definite can be stated, as at the time of the onset of the disease the patient was a chronic sufferer from headaches.
The blotches remained stationary for about a month, then they gradually subsided. The itching at that time was still as intense as at the start. Gradually little black "pimples" like blackheads began to appear. Among them were red "pimples" like prickly heat.
The rash first appeared in the groins and about the hips and from there it continually spread upward and only very little downward. About December, 1915, the patient states, the skin began to feel rough and look dirty. The itching was still as intense as before.
Status Praesens (Jan. 16, 1916). — There is a discrete papular eruption covering the entire abdomen, the chest to just above the nipple line, the back, both buttocks and a belt of very thickly crowded papules about the waist line. The eruption extends downward on the posterior surfaces of both thighs to about the junction of the middle and lower third. The papules are less numerous on the anterior surfaces of the thighs and become still fewer as the eruption extends downward. Below the middle of the thigh there is not a single papule to be seen. There are several discrete papules on the glans penis and on the prepuce. Posteriorly, the eruption extends upward to the seventh cervical vertebra, over both scapular regions, both deltoids and down on the extensor surfaces of the arms, forearms, wrists, hands and fingers. The papules are especially prominent on the first and second phalanges where they are arranged in parallel rows. On the flexor surfaces of the arm the lesions are fewer and none is seen on the flexor surfaces of the forearms, hands and fingers. On the inner surfaces of both cheeks, about half a dozen papules can be found. They are discrete, gray in color and are indistinguishable from those of lichen planus.
The distribution is strikingly symmetrical. If there is a patch of very closely crowded papules in any region on one side of the body, there is a correspondng one on the opposite side. Similarly, when the papules are only sparingly present or are entirely absent from a place on one side of the body, the same will be true of the corresponding place on the" opposite side. Most of the papules are diffusely scattered without arrangement, but a number appear in larger and smaller groups or form straight and curved lines, circles and segments of circles. On the backs of the first and second phalanges the papules are arranged in straight parallel rows.
The size of the papule is variable, averaging about the size of a large pinhead, and most of them are well raised above the surface.
The papules vary from red to purple in color. A small number are yellowish-brown. They are tough to the touch and a rough, gray, horny plug projects from the apex of nearly every papule. The plugs are up to 2 mm. in length; they can be pulled out quite readily and on removal leave a red, shiny depression in the center of the lesion. Some plugs are shorter and stubbier and brown to almost black in color. On a number of lesions the plug is so tiny that it can hardly be seen, but it can be felt quite readily with the finger. A smaller number lack the plugs entirely and only a slight fine scaliness can be seen to cap the top of each papule. Finally several papules have no plugs and no scales, the color being that of normal skin. The lesion as a whole resembles goose-skin.
General Symptoms— Outside of the itching there is only a slight pallor. The pulse is 76, normal, full and compressible.
Blood Examination, Jan. 18, 1916: hemoglobin, 84 per cent.; white blood cells and differential count, normal.
Treatment.—The patient was given Vw grain Asiatic pills, was instructed to take one pill three times a day and to increase the dose gradually until ten pills per day were reached. Local Treatment: The only drug used was ammoniated mercury, 5 per cent, in ointment form, applied once daily.
Biopsy.—This was made on the above date. Specimens were stained with hematoxylin-eosin; polychrome-orcein; von Pappenheim; safranin-wasserblau; dahlia; picro-lithio-carmine; picro-nigrocin; and hematoxylin-Delafield.
Pathologic Findings.—The corneal layer is much thickened and appears in homogeneous, wavy, superimposed lamellae. No parakeratotic cells are to be found. The stratum lucidum cannot be made out. The stratum granulosum is apparently normal. Stratum malpighii is thickened. The rete pegs are not elongated but are thickened. The basal layer is normal.
The hair follicle is widely dilated, more so toward its mouth. A horny mass fills out the cavity and it contains in its center an unaltered hair. The horny mass is arranged in longitudinal concentric lamellae, except for the deepest portion where the arrangement is more or less horizontal and the outline of the horny cells is distinctly retained.
The stratum granulosum of the follicle appears to be coarser and in some places the granules run together in large fat-like droplets.
The rete appears thinner in places and thicker in others.
The basal layer is thickened in places and in others is thinned or missing, being obscured by the round cell infiltration into the epithelial layer. In one place there is a large space between what appears to be the rete and the infiltrate surrounding the follicle, the basal layer being completely absent.
Throughout the whole corium and more so in the upper layer there are to be seen long rows and circles of round cell infiltrations, indicating the presence of a perivascular localization. The latter is more marked about the dilated follicle and also the sweat glands.
The fibrous sheath of the follicle is thickly infiltrated; the infiltration extends quite a distance out into the corium.
Cells composing the infiltrate: mononuclear leukocytes, large spindle-shaped fibroblasts, plasma cells, few in number, and comparatively many mast cells. Mitotic figures were only rarely observed.
Progress of the Condition and Results of Treatment.—March 8, 1916: The itching is much improved. Some papules can be seen to be involuting. They are smaller and paler. There are fewer papules on the backs of the fingers than there were on the previous visit.
March 22, 1916: No further improvement is noticed. Treatment is continued.
April 13, 1916: There is some slight improvement in both the itching and the appearance of the papules. The arsenic treatment was continued but the local treatment was changed to 5 per cent, oleum rusci in Lassar's paste.
A second biopsy was made. The section was taken from the back and a spot selected where the papules had the color of the normal skin, to determine the presence of infiltration. The section passed through two papules, one cut transversely and the other longitudinally. The latter was a double follicle filled with a bifurcated plug. Although the papules selected were indistinguishable from the normal skin, the infiltration was as marked as in the specimen from the first biopsy. Neither of these papules contained a hair.
May 24, 1916: There is marked improvement. There is still a number of deep red papules, raised above the surface and capped with long horny plugs. Most of the lesions, however, are smaller; the color is dirty gray, pink or yellowish-brown. The papule is capped with an almost fiat, black, horny plug, not unlike a comedo. A large number of papules are normal in color and possesses no plug.
Scattered among the other papules and especially numerous in the groins and axillae, there are deep red papules of the size of a sago seed. They present at the apex a grayish white mass, resembling a pustule. The whole lesion resembles a pustular acne. Embedded in the pustule-like apex of the papule is a horny plug, which comes out with the slightest scratching with the finger nail and leaves behind a deep crater-like, gaping, moist and shiny depression. No pus, however, can be detected. The origin of the above described lesion may be that some involuting papules are influenced by heat and moisture and also by irritation from scratching. When a plug is pulled out of a small colorless papule, the latter turns pink in color, becomes almost flat and has in its center a small punched-out cavity. If the same operation is performed on a number of closely packed papules, the surface looks honeycombed.
A third biopsy was taken. Now lesions were selected that had no visible horny plugs and as a whole looked like goose skin. There were two papules in the field, both cut transversely. From one the plug had fallen out and left a cavity in its place, the other contained a small plug without a lanugo hair. Infiltration was present, though in less marked degree than in the previous sections.
May 27, 1916: Improvement is rapid. Most of the lesions are entirely flat. The keratotic plugs are black and only slightly project from a nearly normal surface. They come out with the slightest scratching over the surface with the finger nails and leave a very minute normally colored dimple behind them. Other papules are in various stages of involution. Arsenic treatment is discontinued; the local treatment is still kept up.
June 23, 1916: The lesions have almost entirely disappeared and only scattered here and there are individual papules and groups of papules in various stages of involution. There are many large, brown pigmented patches over the shoulders, arms and the back; also in the form of a belt around the waist, in other words, in all locations where the lesions were most numerous.
Scattered all over the body, but comparatively few in number, are horny plugs on an almost flat, slightly brownish surface. The plugs are indistinguishable from comedones at first glance; only when scratched out from their site does the difference become evident. They are hard and short horny masses. The lesions in the mouth are about half their former size, but are still present and distinct.
Jan. 22, 1918: The skin is entirely soft and smooth. It .is entirely free of any eruption. Patches of pigmentation, however, are still present, especally about the waist line.
March 19, 1918: Pigmentation is still present.
July 7, 1918: Pigmentation has not entirely disappeared.