HYDROA VACCINIFORME

Synonyms.—Recurrent summer eruption (Hutchinson); Hydroa æstivale.

Definition.—A recurrent, usually vesicular, scarring summer
eruption, beginning in early life, almost always in males, and, as a rule,
disappearing toward adult age.

This rare disease¹ was first clearly described by Bazin, later by
Hutchinson, Handford, Jamieson, Brooke, Crocker, Bowen, White,
and others, and while in some of the reported cases there are minor
divergences, they all present vesicles, usually pronounced, and with
central depression, but sometimes papules with slight vesicular cap­
ping, and are followed by slight scarring.

Symptoms.—The eruption is entirely or for the most part on un­
covered regions, especially the nose, cheeks, and ears, although excep­
tionally it may be sparsely scattered over the general surface. It is in
almost all cases of vesicular nature, and an outbreak may be preceded
by arthritic or other systemic symptoms of slight character. The
lesions often begin with a preceding feeling of burning of the part, as
discrete or grouped red spots or elevations on which a vesicle or small
bulla develops; many show a surrounding red areola. As a rule, subject­
ive symptoms, are, however, slight or entirely wanting; rarely there may
be some itching. The lesions are variable as to size, from that of a pin-
head to a pea; at first with clear contents; later milky and sometimes sero-
purulent. In a number of the vesicles, and in some cases in most or all of
them, there occurs a slight sinking in or umbilication in the central portion,
drying here to a thin reddish or blackish crust, while the periphery
consists of a surrounding wall of fluid, which may extend slightly, re­
sembling somewhat a small vaccine vesicle, finally crusting. Others

¹ Literature: Bowen, Jour. Cutan. Dis., 1894, p. 89 (a good review of the subject,
histologic examination, with cut; and with literature references to cases of Bazin, Hutch-
inson, Handford, Jamieson, Berliner, Buri, Broes van Dort, Brooke, Boeck, Crocker;
and also a comparison of some of the reported cases of acne necrotica, acne varioli-
formis); Jarisch, Verhandl. des V. Cong. Deutsch. dermat. Gesell., 1895; Colcott Fox,
Brit. Jour. Derm., 1894, p. 236; 1897, p. 476; 1898, p. 409; 1899, p. 464; Graham,
Jour. Cutan. Dis., 1896, p. 41 (good review of subject); Mibelli, Giorn. ital., 1896,
fasc. vi, p. 690 (histologic examination)—abstract in Annales, 1897, p. 672; J. C.
White, Jour. Cutan. Dis., 1898, p. 514; McCall Anderson (two brothers), Brit. Jour.
Derm., 1898, p. 1; Crocker, ibid., 1900, p. 39; Adamson, “On Cases of Hydroa Æstivale
of Mild Type: Their Relationship with Hutcbinson's ‘Summer Prurigo’ and with
‘Hydroa Vacciniforme' of Bazin,” Brit. Jour. Derm., 1906, p. 125 (5 cases, histologic
cut, review, and full bibliography); Kanoky, Jour. Amer. Med. Assoc, 1907, vol. xlix,
p. 1774, reports a case in female child of eight, beginning at age of four, in which the
face was free, the lesions being found on legs, forearms, and dorsal surfaces of both
hands. Tapken, “Ueber ein Fall von Hydroa Vacciniforme (Bazin), Inaug. Disser-
tation," München, 1911 (review and discussion, with the report of a case).

HYDROA VACCINIFORME                               359

dry up evenly and become crusted, and others again may rupture ac­
cidentally or spontaneously and slowly crust over. Frequently two
or three closely grouped lesions coalesce and form a flattened, irregu­
larly outlined, somewhat large bleb. The crusts, which have usually
formed in three or four days from the first appearance of the lesions,
may be yellowish to a red color; they drop off after a variable period,
usually several days or a week or more, disclosing a red, pit-like per­
manent scar, which in the course of time becomes white.

The process in some spots may halt at the erythematous stage
and disappear without trace. The fresh outbreaks may take place
almost continuously, or the attack last two to four weeks, to recur
again upon moderate or prolonged exposure to sun or wind; or the
disease go on indefinitely, at least up to youth or manhood, when the
tendency subsides. Numerous scars and, in some cases, a good deal of
cicatricial disfigurement of the nose and ears remain as permanent
factors, as in cases reported by McCall Anderson and J. C. White.

In some cases (summer prurigo), similar or somewhat allied, the
eruption may consist of conic papules of a pale-red color, and with,
in some, a disposition to minute central vesiculation; in disappearing
they, in most instances, leave insignificant scars. Itching is usually a
feature, although not always present. It is in many respects similar
to the vacciniforme eruption just described, except that the lesions
are more distinctly papular, with less tendency to group; and it is apt
to be more extensively distributed.

The hydroa puerorum¹ of Unna, while classed by most writers as
synonymous with these cases described, differs in important particu­
lars: There is no predilection for exposed parts; recurrence of attacks
does not seem to be dependent upon external influences; there is a
distinct tendency to a coalescence of the vesicles to form blebs; and
the lesions are superficial, with no disposition to pitting or scarring.²
In some of its clinical aspects it bears more resemblance to a mild der­
matitis herpetiformis.

Etiology and Pathology.—The disease is rare, and begins, with
few exceptions, in the first several years of life, and is occasionally
seen in two members of the same family. It is an eruption in which
exposure to the sun and the wind is an important, if not essential, etio-
logic factor. It is, therefore, as a rule, a disease of the summer, the
outbreaks usually disappearing toward cool weather; in some instances,
however, attacks occur during the cold season as well. It recurs the fol­
lowing year, and so continues, becoming less active as puberty is ap­
proached, and disappearing when adult age is reached. Exceptionally
it has been observed to begin later in life, and to continue to a later stage.
In a few cases (McCall Anderson's) the urine during the outbreak of
the efflorescences was noted to be dark wine-colored, and to contain
hematoporphyrin. It is seen almost exclusively in boys. It has some

¹ Unna, “Hydroa Puerorum,” Monatshefte, 1889, vol. ix, p. 108.

²Haase and Hirschler, “Hydroa Puerorum” (Unna), Jour. Cutan. Dis., 1908, p.
199, go over the ground carefully, maintaining the distinct character of the disease (with
review of the subject and references).

360

INFLAMMATIONS

features in common with erythema bullosum and dermatitis herpeti-
formis, and, in some cases, to a slight extent with acne varioliformis.

The pathologic anatomy, studied by Bowen, Mibelli, Adamson, and
others, shows that the process is an inflammatory one, beginning in the
rete and upper corium, with sometimes distinct edema and cellular infil­
tration of the papillary layer, resulting in vesicle formation in the rete
and subsequent circumscribed necrosis extending deeply in the derma and
sometimes into the subcutaneous tissue.

Diagnosis.—Its occurrence solely or most severely in summer,
usually in boys and in early life, the distribution, characters, and course
of the lesions, with scars usually following, and its repeated recurrence
are diagnostic features; scars do not form in erythema bullosum or in
dermatitis herpetiformis, besides differing in some of the other characters
named.

Prognosis and Treatment.—The patient can usually be made
more comfortable by proper measures, and the attacks less active and
frequent by avoidance of the sun, heat, and wind, but so far the ap­
proach to adult age seems the only factor which stays the disease.
As the chemical rays of the sun may be of some causative influence,
the wearing of orange or red or dark-colored veils, as has been sug­
gested, can be tried. Treatment is to be mild in character, and is
essentially the same as used in other vesicular and bullous diseases.

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