MULTIPLE BENIGN SARCOID¹

Synonyms.—Sarcoid Tumor; Sarcoid; Miliary lupoid; Benign miliary lupoid.

The name multiple benign sarcoid is one usually employed to desig­
nate a group of neoplasmata characterized by a limited growth and
comparative benignancy, and a favorable response in a proportion of the
cases to arsenical treatment. It was Boeck’s notable paper in 1897 that
first attracted attention to this relatively mild form of tumor, and later
Darier who added much further knowledge; followed by Pawloff, Spieg-
ler, Fendt, Gottheil, Hallopeau, Pollitzer, G. H. Fox and Wile, Paul
Unna, Jr., and others. Darier’s study of his own cases and the literature
led to his division of the reported cases into four distinct types or groups:
(1) the multiple benign sarcoid of Boeck; (2) the subcutaneous sarcoid
of Darier-Roussy; (3) the nodular erythema-induratum-like sarcoid
of the extremities, and (4) the Spiegler-Fendt sarcoid, with some appar­
ent kinship to the neoplastic lymphoderma. In addition to these four
types, Darier calls attention to the fact that a mixed type is occasionally
observed.

The Boeck type of sarcoid may present as a single lesion or in
moderate or even large numbers, and upon face, back, shoulders, and
extensor surfaces of the arms. The lesions may be papular, nodu­
lar, or infiltrated plaques, and make their appearance slowly or rap­
idly, some being superficial, others deep in the cutis. The growths
vary in size from a millet-seed to a large bean, and are at first faintly
pinkish or rose colored, later reddish, livid, and finally brownish; they
are smooth, firm and elastic, intimately associated with the skin, and
move with it; telangiectases are sometimes to be seen peripherally,
and under glass pressure minute grayish-yellow foci are often to be
detected in the nodule, hence the later term, “miliary lupoid,^,, given by
Boeck to the malady. A plaque, if present, usually consists of a limited
or somewhat diffused, more or less even infiltration, or of closely crowded
nodules with associated infiltration. Boeck recognized three types of

¹ Important Literature: Boeck, Jour. Cutan. Dis., 1899, p. 543; Kaposi, Festschrift
zu von Moritz, 1900, p. 153; and Archiv, 1905, lxxiii, p. 71; Darier et Roussy, Annales,
1904, v, pp. 144 and 347; Darier, Monatshefte, 1910, L, p. 419 (with review and full
bibliography); Colcott Fox, Brit. Jour. Derm., 1893, pp. 225, 293, and 338; Spiegler,
Archiv, 1894, xxvii, p. 163; Joseph, Archiv, 1898, xlvi, p. 177; Philippson, Giorn. ital.,
1898, xxxiii, p. 61; Thibiérge et Revaut, Annales, 1899, p. 513; Fendt, Archiv, 1900,
liii, p. 213; Carle, Lyon Med., 1901, xcvi, p. 358; Gottheil, Jour. Cutan. Dis., 1902, p,
400; Pawloff, Monatshefte, 1904, xxxviii, p. 469; Winkler, Archiv, 1905, lxxvii, p. 3;
Pelagatti, Giorn. Hal., 1907, xlviii, p. 425; Thibiérge and Bord, Annales, 1907, p. 113;
Opificius, Archiv, 1907, lxxxv, p. 239; Pollitzer, Jour. Cutan. Dis., 1908, p. 15; Kreibich
und Kraus, Archiv, 1908, xcii, p. 173; Kren und Weidenfeld, Archiv, 1909, xcix, p. 79;
Urban, Archiv, 1910, ci, p. 175; G. H. Fox and Wile, Jour. Cutan. Dis., 1911, p. 375
(case report with case and histologic illustrations; with brief review and bibliography of
main papers; I am indebted to this paper); Pöhlmann, Archiv, 1910, cii, p. 108 (case
report with review); Polland, “Sarcomatosis Cutis” (Spiegler), Archiv, 1912,cxi, No. 1,
p. 3—abs. in Jour. Cutan. Dis., 1912, p. 362; and Unna, Jr., Monatshefte, 1912, lv,
p. 1203, (case illustrations and review).

MULTIPLE BENIGN SARCOID                          903

his group: (1) the large nodular, with pea- to pigeon’s-egg-sized lesions;
(2) the small nodular-papular, millet-seed­ to pea-sized tumors, some­
times close together, and (3) the diffuse infiltrating form, probably a
combination of the other two. Occasionally the surface may show slight
scaliness. A peculiarity of many of the tumors is that they are much
smaller looking to the sight than to palpation. After some duration,
months or years, a nodule frequently tends to involution, usually first
shrinking centrally, and gradually disappearing, leaving a brownish stain
or slight atrophic scar. There seems to be no tendency to caseation or
ulceration. There are usually no subjective symptoms. Enlargement
of the lymphatic glands is observed in some cases. The malady pre­
sents in some instances a rough resemblance to lupus vulgaris and
sarcoma.

The second or Darier-Roussy type consists of painless, hazel-nut-
to walnut-sized round or oval subcutaneous nodules, occurring on the
trunk and especially the upper part; it is rare, but few cases having been
recorded. The third, or erythema-induratum-like group comprise the
cases reported by Pelegatti, Thibiérge and Bord, Colcott Fox, Darier,
and others; the lesions bear close resemblance to the erythema induratum
of Bazin, occur chiefly in women, and on the legs and arms; are nut- to
pigeon’s-egg-sized, reddish, purplish or livid tumors, chronic in their
course, and occasionally terminating in ulceration. The fourth, or
Spiegler-Fendt, type, resembles clinically the other groups, more espe­
cially the first two. While the cases comprised under these four groupings
by Darier are not common—rather rare, in fact—those of the third group
are met with most frequently and following this, in the order named,
those of the first, fourth, and second. The malady is seen predominantly
in women. The first, second, and third groups seem to show some re­
lationship to tuberculosis, the fourth group showing a suggestion of
lymphogranulomata. Darier and Roussy advanced the theory that
sarcoid is a tuberculide, and possibly due to a low-grade infection with
the tubercle bacilli, basing it mainly on the histopathology; tuberculosis
has been found, however, in a proportion of the patients, and in almost
one-third of the cases there has been a reaction to tuberculin.

Histologic examination, upon which diagnosis must be mainly based,
discloses (Boeck) “through the whole depth of the corium from the papil­
lary layer to the limits of subcutaneous tissue, sharply circumscribed
foci of a new growth separated from each other; higher power shows that
the cells of the new growth are of the type of epithelioid connective­ tissue
cells, and that the tumor, as a rule, has its origin in the perivascular
lymph spaces. The proliferated cells soon enclose the greatly dilated ves­
sels with a compact cylindric mass; as proliferation increases and the
foci take different shapes, though still sharply circumscribed, the resem­
blance to epithelioid cells becomes more marked. The nuclei are large
and vesicular, less deeply stained and show distinct nucleoli; the nuclei
are sometimes multiple. The cell protoplasm is increased in amount
and sends out prolongations in different directions. In a few instances
giant-cells of the sarcomatous type are found. Mitosis is scarcely any­
where to be detected.” At the periphery are seen lymphocytes in varying

904

NEW GROWTHS

numbers, few plasm cells, and scattered here and there giant-cells having
many nuclei, and rarely giant-cells of the true Langhans type (Fox and
Wile). There is no evidence of caseation necrosis. The histology of the
lesions of the second group is in the main very similar, but presents an
even closer resemblance to tuberculosis; numerous giant-cells are seen.
The Spiegler-Fendt tumor show “aggregations of round cells more or
less circumscribed, at times enclosed in a capsule of connective tissue.
Giant-cells and epithelioid cells occur in small numbers.’’

Prognosis and Treatment.—All these various cases are slow in
progress, and with the exception of the erythema-induration type, which
sometimes breaks down, give rise to no pain or actual discomfort, except
the disfigurement and stiffness and unwieldiness of the affected parts
when much infiltrated. The malady is benign, but few instances of
fatal termination occurring. The duration is indefinite, but in some cases
gradual disappearance ensues, and in others there is favorable response
to arsenical medication. Darier reports also favorable influence from
the x-ray, calomel, and tuberculin.

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