Volume 16

Number 2, Part 1 Correspondence 395

February 1987

The patient's condition continued to improve although REFERENCES

small amounts of blood were intermittently passed through 1. Gellis S, Glass FA. Pemphigus: a survey of 170 patients
the rectum. In an attempt to localize the source of blood loss, admitted to Bellevue Hospital from 1911 to 1941. Arch
various tests were performed; barium enema, gastroscopy, Dermatel Syphilol 1941;44:321-36.
and technetium scan of the abdomen revealed no abnormality. 2. Ahmed AR, Moy R. Death in pemphigus. J AM ACAD
Coagulation screen and platelet count had remained normal DERMA'rOL1982;7:221-8.
throughout. During this period the skin had been steadily 3. Savin JA. Events leading to death of patients with pem-
improving, and although a further significant hemorrhage was phigus and bullous pemphigoid. Br J Dermatol 1978;
clearly a possibility, Iaparotomy was not considered advisable 101:521-34.
in view of the patient's general condition.
However, the lower abdominal pain and perineal pain re-
curred suddenly, followed by a further severe episode of rectal Acquired ichthyosis in a patient with
bleeding. On this occasion the hemorrhage persisted, and acquired i m m u n o d e f i c i e n c y s y n d r o m e and
despite the replacement of large quantities of blood the patient Kaposi's sarcoma
became severly hypotensive. An emergency laparotomy was
then arranged. To the Editor: We have observed a case of acquired
At operation the source of hemorrhage was identified as a ichthyosis in a patient with the acquired immunodefi-
single spurting vessel located just within the anorectal j unction ciency syndrome (AIDS) and Kaposi's sarcoma. To our
and arising from otherwise normal-looking mucosa. Ligation knowledge, the association of acquired ichthyosis with
of this vessel resulted, in arrest of the hemorrhage. At this AIDS-related Kaposi's sarcoma has not been previously
stage, blood began to well up from the pelvic cavity, and reported. Acquired ichthyosis has been reported in as-
despite all efforts the hemorrhage could not be an'ested. Clot- sociation with the classic form of Kaposi's sarcoma
ting was not seen to occur, and it was considered that this
(multiple idiopathic hemorrhagic sarcoma). 1-3
terminal, uncontrollable hemorrhage was caused by a failure
of coagulation resulting from massive transfusion. Case report. A 32-year-old bisexual man was admitted
The postmortem examination failed to reveal a specific to the San Diego Veterans Administration Medical Center in
cause for the hemorrhage. In particular, there was no evi- October 1985 with a diganosis of AIDS. His symptoms had
dence, either on routine investigation or on direct immune- begun approximately 18 months prior to admission, with the
fluorescence examination, of pemphigus vulgaris involving onset of fatigue, diarrhea, a 60-pound weight loss, and ab-
the bowel. normal neurologic behavior. Six months prior to admission,
the patient noticed reddish brown papules on his arms and
Discussion. Pemphigus vulgaris is a disorder that, the onset of generalized dry skin. There was no prior personal
prior to the advent of systemic corticosteroid therapy, or family history of ichthyosis. The patient's past medical
history included treatment of syphilis and gonorrhea, as well
was largely untreatable and frequently fatal.~ Despite
as hepatitis B. He was taking no medications known to induce
modern treatment it remains a serious disease with a
ichthyosiform skin changes.
significant mortality rate. The most common cause of Examination of the patient's skin revealed multiple 0.5-
death is infection, with septicemia and pneumonia being cm, reddish brown papules scattered on his left arm and the
responsible for the majority of fatalities. 2'3 upper part of his back. In addition, there was generalized
Significant hemorrhage from the large bowel is ex- ichthyosis with large and adherent scales on the trunk and
tremely unusual in patients with pemphigus, and when extremities, consistent with a diagnosis of acquired ichthyosis
it does occur, there is usually some other associated
abnormality to account for it?
This case is the first, to our knowledge, in which a
patient suffering from pemphigus vulgaris has died as
a direct consequence of uncontrollable hemorrhage
from a rectal vesseI in this fashion. We suggest that the
possibility of this association be borne in mind in the
management of patients with pemphigus and large-
bowel hemorrhage o f uncertain origin.
J. Ashworth, M.R.C.P., N. H. Cox, M.R.C.P.,
W. R. Pickard, F,R.C.S., C. Mackay, F.R.C.S., and
D. T. Roberts, F.R.C.P.
Western Infirmary, Glasgow G. 11.6. NT.
United Kingdom Fig. 1. Ichthyotic skin on the patient's right leg.
 Journal of the
American Academy of
396 Correspondence
Dermatology

2. Krakowski A, Brenner S, Covo J. In Kaposi's sarcoma.

Arch Dermatol 1975; l 11:1213-4.
3. Kohn SR. A variant of ichthyosis. Arch Dermatol
1976;112:1616.
4. Haynes HA, Curth HO. Cutaneous manifestations asso-
ciated with malignant internal disease. In: Fitzpatrick T,
Eisen AZ, Wolff K, et al, eds. Dermatology and general
medicine. 2rid ed. New York: McGraw-Hill Book Co,
1979:1349-50.
5. Bories C, Blanchet-Bardon C[, Marie JP, et al. lchtyose
acquise au cours d'un syndrome d'immunodepression
aquise. Presse Med 1984;13:1573.

Norwegian scabies in acquired

Fig. 2. Low-power view of skin biopsy specimen, show- immunodeficiency syndrome: Report of a case
ing compact hyperkeratosis, an intact granular layer, resulting in death from associated sepsis
slight acanthosis, and a normal-appearing dermis. (He-
matoxylin-eosin stain; x 100.) To the Editor: The crusted (Norwegian) form is an
uncommon and distinctive variant of scabies. It is usu-
(Fig. 1). The ichthyosis spared the flexures. The patient was ally associated with immunosuppression or neurologic
also noted to have oral candidiasis, and Cryptococcus organ- impairment, although it has r a r e l y been reported in nor-
isms were cultured from his stool. Results of thyroid function
mal persons. Its incidence is increasing, perhaps as a
tests were normal.
result of the increasing use o f immunosuppressive
Histopathologic examination of representative skin papules
confirmed the diagnosis of Kaposi's sarcoma. A biopsy spec- agents. In contrast to the c o m m o n form of scabies, in
imen of representative ichthyotic skin (Fig. 2) showed hy- which less than a score of adult f e m a l e mites are pres-
perkeratosis, an intact granular layer, slight acanthosis, and ent, l hundreds to thousands of Sarcoptes mites infest
an unremarkable underlying dermis, consistent with a diag- patients with this form. 2 It is h i g h l y contagious, even
nosis of ichthyosis. through fomites. Patients have hyperkeratotic plaques
on erythematous bases. Lesions m a y be generalized but
Discussion. Acquired ichthyosis is most commonly
are especially prominent on the n e c k , scalp, and trunk.
reported in association with Hodgkin's disease and has
Subungual spaces may be i n v o l v e d . 3 Itching is usually
also been reported in association with reticulolympho-
minimal. Associated findings i n c l u d e lymphadenopa-
sarcoma, aplastic anemia, mycosis fungoides, spindle-
thy, eosinophilia, elevated levels o f IgE, 4 and decreased
cell sarcoma, multiple myeloma, breast carcinoma, and
levels of IgA. s We describe a c a s e of crusted scabies
metastatic lung carcinoma. 4 Acquired ichthyosis has
in a patient with acquired immunodeficiency syndrome
also been reported in association with several cases of
(AIDS) who died with bacterrnia, pericarditis, and
the classic form of Kaposi's sarcoma, i-3 Although there
pneumonia.
has been one previous report of acquired ichthyosis in
association with AIDS, 5 to our knowledge there have Case report. A 35-year-old homosexual man presented to
been no previous reports of acquired ichthyosis asso- the University of California, San Diego, Medical Center be-
ciated with AIDS-related Kaposi's sarcoma. The tem- cause of increasing weakness, shortness of breath, and a gen-
poral relationship between the onset of this patient's eralized eruption with painful fissuring. He had had multiple
male sexual partners, and he had a l-year history of fever
Kaposi's sarcoma and the onset of acquired ichthyosis
and chills. Three months prior to his presentation, skin lesions
leads us to believe that the two conditions were related.
appeared as linear, red, intensely pruritic "welts." Pruritus
Lorraine Young, M.D., and gradually lessened as the lesions thickened and coalesced to
Howard K. Steinman, M.D. cover most of his body. Several days prior to the patient's
Veterans Administration Medical Center, admission to the medical center, painful fissures developed
in the flexures.
University of California, San Diego,
The patient was acutely ill with shaking chills and dyspnea.
School of Medicine, San Diego, CA 92161 His oral tempeature was 96 ° F, blood pressure was 116/60
mm Hg, and pulse was 100 beats/min. There was jugular
REFERENCES vein distention, and $3 heart sound, hepatosplenomegaly, pit-
l. Krakowski A, Brenner S, Covo J, et al. Acquired ichthy- ting edema to the mid portion of the thigh, and cervical and
osis in Kaposi's sarcoma. Dermatologica 1973;147:348- axillary lymph node enlargement.
551. There were striking gray-brown hyperkeratotic plaques