DIFFUSE IDIOPATHIC ATROPHY OF SKIN²

Synonyms.—Atrophia maculosa cutis; Acrodermatitis chronica atrophicans;
anetodermia.

A more or less diffuse atrophy of the skin (also called general idio-
pathic atrophy, progressive idiopathic atrophy, atrophia cutis uni-

that it should be considered as an unusual type of morphea or localized scleroderma
(“morphœa guttata”); Juliusberg‘s case, Dermatolog. Zeitschr., 1909, vol. xv, p. 12
(with clinical and histologic illustrations), seems to strengthen this view; Riecke,
Archiv, 1909, vol. xcix, p. 181 (with colored case and histologic illustration). It is
possible that an occasional case might be explained upon the basis of a previous lichen
planus of the atrophic type (lichen planus atrophicus).

¹ Langer, Med. Jahrbücher, 1880, p. 49 (with 6 histologic cuts).

² Literature: E. Wilson, Lectures on Dermatology, London, 1878, p. 393; Schwimmer,
Die Neuropatischen Dermatonosen, p. 189; Atkinson, Richmond and Louisville Med.
Jour., 1877, vol. xxiv, p. 564; Glax, Allg. Wiener med. Zeitung, No. 35, 1874; full abs.
in Archiv, 1875, P. 144; Buchwald, Archiv, 1883, p. 553 (with illustration); Behrend,
abs. in Archiv, 1885, p. 346 (original in Berlin, klin. Wochenschr., No. 6, 1885);
Touton, Deutsch. med. Wochenschr., 1886, p. 6; Pospelow (2 cases), Annales, 1886,
P. 505 (with illustration and references); Groen, Norsk Magazine, abs.-ref. in Lancet,
1891, vol. ii, p. 1238; Beer, Archiv, 1892, p. 835; Williams, Brit. Jour. Derm., 1894,
p. 342 (case demonstration); Bronson, Jour. Cutan. Dis., 1895,p. 1 (with colored plates
and other illustrations and references); Elliot, ibid., 1895, p. 152; Fordyce, ibid., 1897,
p. 230 (case demonstration); Kaposi, Archiv, 1897, vol. xxxix, p. 413 (case demonstra-

DIFFUSE IDIOPATHIC ATROPHY OF THE SKIN         623

versalis) has been reported from time to time. The chief features are:
atrophic thinning, dryness, and a variable degree of branniness, or ill-
defined, thin, flaky scaliness, with usually loss of the hairs and absence,
relative or complete, of the sweat secretion; and sometimes a white,
streaky, or patchy appearance, frequently with interspersing of brownish
discoloration; with, in typical examples, a variable amount of looseness
and wrinkling of the skin, and in some cases a conspicuousness of the
surface veins. In some instances there is an underlying dusky-red hue,
with sometimes intermingling of purplish or brownish. In some places,
varying considerably in different cases, there is also noted a waxy,
glistening, parchment-like surface, as in macular atrophy. Indeed, not
infrequently the atrophic changes are more marked here and there,
producing spot-like or small, white, thin, scar-like areas, somewhat
depressed. On parts covering prominent bony projections, as at the

Fig. 147.—Atrophy of the skin; old woman; chiefly involving the legs, especially the
knee and lower thigh regions.

ankles, ulcers are apt to form. In Fordyce‘s patient a number of out­
breaks of bullæ about the ankles occurred. As a rule, there are no dis­
tinctive subjective symptoms, although occasionally shooting pains,

tion); Neumann, ibid., 1898, vol. xliv, p. 3 (with references and 3 colored histologic
cuts); Colombini, Monatshefte, 1898, vol. xxviii, p. 65; Holder, Jour. Cutan. Dis., 1899,
p. 37 (case demonstration); Kingsbury, “A Case of Acrodermatitis Chronica Atro-
phicans with Co­existing Scleroderma,” ibid., 1907, p. 414; Malinowski, “Atrophie
idiopathique de la peau,” Annales, 1908, p. 562, reports 5 cases, reviews subject, with
bibliography; Kanoky and Sutton, “A Comparative Study of Acrodermatitis Chronica
Atrophicans and Diffuse Scleroderma, with Associated Morphœa Atrophica,” Jour.
Cutan. Dis., Dec 1909 (illustrated, with bibliography); Beck, “Beitrag zur Lehre
von der idiopathischen Hautatrophie, Archiv, February, 1910, C, p. 117 (reviews entire
subject described under idiopathic atrophy, acrodermatitis chronica atrophicans, and
erythema paralyticum; with clinical and histologic study of 9 cases); the writer thinks
name should be dermatitis chronica atrophicans, although in those cases limited to ex­
tremities he thinks Herxheimer's name—Acrodermatitis Chronica Atrophicans ap­
propriate; Finger, “Die Hautatrophien (atrophia diffusa, anetodermia, atrophia
maculosa) und deren Verhältniss Zur Skleroderma, Wien. Med. Wochenschr, 1910,
Nos. 2 and 3.

624

ATROPHIES

as in Bronson's case, involving both extremities, and rarely a sensitive­
ness to touch or pressure. The amount of surface involved varies from
a part of one or two limbs, as in Elliot's patient, to that of practically
the entire surface, as in Neumann‘s and Colombini‘s cases, and in the
congenital cases of Behrend and Williams. The lower extremities are
most commonly involved, and the region of the knees is a not infrequent
starting-point; in some both upper and lower extremities are affected
(Touton, Pospelow, Groen, Bronson, Fordyce, Holder). In several
instances the first evidence was upon the dorsal surface of the hand.

Some of the cases, more especially those first reported, could, I
believe, be better placed as examples, probably anomalous, of other
maladies. There is, as Crocker suggests, a strong suggestion of sclero-
derma, with marked atrophic tendency in several,—especially in those
of Wilson, Schwimmer, Atkinson, and Glax,—although Atkinson's
case, which was unilateral, lacked the ordinary features of scleroderma,
and had much in common with cutaneous atrophy. Some cases were
of more or less limited character and chiefly patchy, as in Beer‘s patient;
in this there was preceding edema, and it was somewhat suggestive of a
circumscribed scleroderma. Both Schwimmer and Glax, at the time,
thought their cases probably, but incorrectly, belonged under xeroderma
pigmentosum. Those described by Buchwald, Pospelow, Bronson, and
Colombini have much in common, especially as to laxity of the skin;
this, in one of Pospelow‘s cases, was, however, somewhat extreme,
resembling dermatolysis. In Neumann‘s patient, practically universal,
there was diffuse redness, with thinning, wrinkling, and furfuraceous
and lamellar desquamation. The wrinkling observed in most of these
patients, as in Bronson‘s patient, follows the cleavage lines of the skin;
in most instances it is not especially noticeable, resembling, at a dis­
tance, minute striæ, giving the thinned skin a cigarette-paper appear­
ance, but in others it is of the nature of distinct folds. In Bronson‘s
case, as in some others, there is remarkable symmetry in the distribution.
In some patients the neighboring lymphatic glands were enlarged.

The causes of the disease are not known; in rare instances it is con­
genital, in others, and probably in almost all, appearing at mature or
advancing adult age. In 10 cases it began in 1 (Pospelow—female, aged
fifty) at the age of sixteen, 1 (Buchwald—male, aged thirty-six) at
twenty, 3 (Bronson, Elliot, Neumann—males, aged forty-five, forty-five,
and thirty-two) at about thirty, 1 (Touton—male, aged fifty-seven) at
thirty-five, 1 (Fordyce—female, aged forty) at thirty-eight, 1 (Holder—
female, aged fifty-four) at the age of forty-six, 1 (Pospelow—female,
aged fifty) at forty-eight, and 1 (Colombini—female, aged fifty-five)
at fifty-four and a half. In 12 cases the sexes were evenly divided.
The health of most patients seemed fair, although in several instances the
malady followed “taking cold” or a chill; in the cases of Fordyce and
Holder there were associated symptoms of headache, dizziness, and ex­
treme nervousness. The primary pathologic condition in some in­
stances—in all, according to Finger—appears to be a scarcely per­
ceptible inflammatory process, which seems borne out by the histologic
examination (Colombini). In Elliot‘s case a purplish-red zone bounded

KRAUROSIS VULVÆ

625

the advancing area of atrophy, and this Elliot believed was the primary
step in the process, and the atrophy only a consequence. The process
in this case and in some others bears in this respect, in my judgment, a
close analogy to that of morphea. The changes found are those of well-
marked atrophy, involving the entire integument and glandular struc­
tures, similar, in fact, to those observed in striæ et maculæ atrophicæ.
Fordyce found marked changes in the vessels, the lumen of some being
completely obliterated.

The prognosis for the malady is not favorable for recovery,
although beyond variable discomfort, however, the general health did
not seriously suffer, except in the generalized cases. There seems prac­
tically no tendency to extensive involutionary changes, the malady
usually progressing up to a certain point, and then remaining, relatively
at least, stationary. In most instances several years elapse before reach­
ing its greatest extension, although in Kaposi's and Colombini's cases
in five or six months almost the entire surface was involved. The gen­
eral treatment indicated would be arsenic, cod-liver oil, and tonics,
with mild oily applications to reduce the dryness and harshness.

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