orders.
Using these increased frequencies in the calcula¬ ited the dermatology clinic of the Isesaki Municipal Hos-
tion of incidence of the triple association may not be ap¬
propriate, particularly considering the narrow range of ages
when JCML tends to appear. Even if one were to assume
a 3.6 per 1000 annual incidence rate of JCML in children
with NF1 and JXG, the cumulative incidence rate in these
children at the age of 14 years would be 1 per 20.
Finally, the higher reported frequencies of the vari¬
ous disorders were used purposely in our study. Using lower
frequencies would further increase the differences be¬
tween the observed and the expected values and would be
rightfully subject to criticism.
Since JCML usually appears during the few first y ears
of life, closely monitoring for signs of JCML in children
with NF1 and JXG is not a significant burden to a routine
follow-up in a child with NFL In view of this and the pre¬
viously mentioned arguments, the risk for JCML in chil¬
dren with NF1 and JXG should not be overlooked.
Until now, there were no available data that could
link NF1JXG, and JCML. In addition to an approach sug¬
gested by Gutmann et al, an assessment of neurofibromin
expression in bone marrow and in JXG lesions from pa¬
tients with leukemia, as well as neurofibromin expression
infXG lesions from patients with NF1 without JCML, may
also contribute to the delineation of possible links be¬
tween the 3 entities.
Alex Zvulunov, MD
Department of Dermatology
Soroka Medical Center
Beer-Sheva, 84101
Israel
1. Zvulunov A, Barak Y, Metzker A. Juvenile xanthogranuloma, neurofibroma-
tosis and juvenile myelogenous leukemia: world statistical analysis. Arch Der-
matol. 1995;131:904-908.
2. Huson SM, Harper PS, Compstone DAS. Von Recklinghausen neurofibroma-
tosis: a clinical and population study in South East Wales. Brain. 1988;111:
1355-1381.
3. Garty BZ, Laor A, Danon YL. Neurofibromatosis type 1 in Israel: survey of
young adults.J Med Genet. 1994;31:853-857.
4. Fuller LC, Cox B, Gardner RJ. Prevalence of von Recklinghausen neurofibro-
matosis in Dunedin, New Zealand. Neurofibromatosis. 1989;2:278-283.
5. Samuelsson B, Samuelsson S. Neurofibromatosis in Gothenburg, Sweden, I:
background, study design and epidemiology. Neurofibromatosis. 1989;2:6\x=req-\
22.
Hepatitis C Virus Infection
in Patients With Psoriasis
C virus (HCV) is known to induce vari-
Hepatitis ous skin manifestations, most likely by the for-
mation of circulating immune complexes.1 Pre-
viously, reported that HCV is closely associated with
we
prurigo, which can be treated with interferon.2 In an at-
tempt to see if HCV infection is associated with other der-
matologic manifestations, patients attending a derma-
tology clinic for the care of psoriasis were tested for
antibody to HCV and HCV RNA.
During January 1994 and March 1995, 27 consecu-
tive patients with psoriasis (16 men and 11 women) vis-
Downloaded From: http://archderm.jamanetwork.com/ by a University of Iowa User on 05/31/2015
pital, Gunma-Ken, Japan. None of the patients had been
referred from other hospitals. Antibody to HCV was de-
tected by the second-generation enzyme immunoassay
in 9 (33%) of the 27 patients with psoriasis and anti-
body to synthetic HCV core peptides3 in 12 (44%). The
frequency of antibody to HCV in the patients with pso-
riasis was higher than in 14 (2.8%) of 492 patients at¬
tending our dermatology clinics (P<.001) and in 11
(1.2%) of 923 age- and sex-matched blood donors
(P<.001).
Hepatitis C virus RNA was detected by reverse-
transcription polymerase chain reaction in 7 patients
(26%), all of whom were positive for antibody to HCV
core peptides, including 5 with antibody to HCV.
Genotypes of HCV determined by the second-
were
generation polymerase chain reaction method with
type-specific sense and antisense primers deduced
from the HCV core gene.4 They were Il/lb (2
patients), III/2a (2 patients), IV/2b (2 patients), and
mixed (Il/lb and III/2a) (1 patient). This distribution
of HCV genotypes was comparable to that found in
symptom-free HCV carriers and patients with hepatitis
C in Japan. None of the 27 patients was infected with
hepatitis B virus or human immunodeficiency virus
type 1.
The 7 patients with HCV RNA had higher levels of
alanine aminotransferase (79 ±42 vs 22 ±25 U/L, P<.001)
and aspartate aminotransferase (64 ±36 vs 21 ±7 U/L,
P<.05) and higher zinc turbidity test results (19.9±9.3
vs 8.3±2.3 Kunkel units [normal range, 4.0-13.0 Kunkel
units], P<.05) than the 20 patients without it. Of par¬
ticular note are the platelet counts, which were signifi¬
cantly lower in HCV-positive than HCV-negative pa¬
tients (148±64 vs 215±65X107L, P<.05). Immune
complexes decrease platelet counts.5 Therefore, it is ex¬
pected that skin lesions of psoriasis would involve vas¬
culitis associated with hepatic injury, some of which might
be induced by circulating immune complexes associ¬
ated with HCV infection.
Report of a Case. A 25-year-old man with HCV-
positive psoriasis had been using a steroid ointment
supplemented with crobetasol propionate for 5 years,
without remarkable effects. White petrolatum was
substituted for the ointment to avoid the effect of cor-
ticosteroids, and recombinant interferon alfa-2b
(Intron-A, Shering-Plough, Osaka, Japan) therapy was
initiated (10 million U/d for 1 week and then 3 times a
week for 23 weeks, to a total dose of 750 million U)
(Figure).
The patient's erythema intensified after he began the
interferon therapy. Hepatitis C virus RNA became un-
detectable in his serum samples at 6 weeks after he be¬
gan the therapy and stayed that way during the therapy.
His skin lesions subsided as his alanine aminotransfer¬
ase level normalized and then disappeared. He had no
skin lesions until 2 months after the completion of the
interferon therapy, when HCV RNA reappeared in his se¬
rum samples accompanied by elevated alanine amino¬
transferase levels. Psoriasis developed but was less se¬
vere than it had been before the therapy.
 ities. Thispathological condition is a therapeutic chal-
Interferon lenge as usual treatments of the common forms of LP are
most often ineffective (retinoids, photochemotherapy, lo-
cal and systemic corticosteroids). We report 2 cases of
severe acral erosive LP previously unresponsive to mul-
150
tiple therapeutic attempts and dramatically improved
2 loo
by thalidomide, a drug not classically used in this indi-
cation.
b' 50
Report of Cases. Case 1. A 60-year-old woman was re-
ferred in 1985 for a severe, hepatitis C\p=m-\negative,erosive
Mar Apr May Jun Jul Aug Sep Oct Nov Dec Jan Feb Mar LP that involved the lips, tongue, and extremities, lead-
1994 1995
ing to a nearly complete functional impairment of these
areas. High-potency local corticosteroids, systemic ster-
Clinical course and levels of alanine aminotransferase (ALT) and hepatitis
C virus (HCV) RNA in the serum sample from a 25-year-old man with oids, total-body photochemotherapy, etretinate, dap-
HCV-positive psoriasis who received interferon therapy. Relative dosage sone, and cyclosporin A resulted in a very limited or tran-
and duration of recombinant interferon alfa-2b therapy are indicated at the
top. The minus sign in parentheses indicates nondetectable levels.
sient improvement and/or unbearable side effects.
Eventually, treatment with oral thalidomide 150 mg daily
was started with dramatic improvement, leading to a com-
Conclusions. Our findings indicate that HCV infection
may cause psoriasis and that patients with psoriasis in¬
plete healing of distal erosions and to an impressive re-
duction of oral lesions after 4 months of treatment, al-
duced by HCV might benefit from interferon therapy.
lowing the patient to use her fingers and to walk without
assistance. No major side effects were noticed aside from
Kazuya Kanazawa, MD an asymptomatic mild lymphopenia ranging from 0.6 to
Gunma-Ken, Japan 0.7X 109/L, mainly involving the CD4 subset. Drug dos¬
Tatsuya Aikawa, MD age was progressively tapered down to 50 mg every other
Ibaraki-Ken, Japan day without recurrence after 3 years of follow-up; the dos¬
Fumio Tsuda, PhD age decrease was followed by the resolution of the lym¬
Tokyo, Japan phopenia.
Hiroaki Okamoto, MD Case 2. A 60-year-old woman was examined in 1994
Immunology Division for a widespread, hepatitis C-negative and disabling ac¬
Jichi Medical School ral erosive LP involving mainly the feet, impairing the
Minamikawachi-Machi patient's ability to walk. Topical and systemic steroids,
Tochigi-Ken 329-04, Japan etretinate, dapsone, and photochemotherapy were of no
benefit, and oral cyclosporin A could not be considered
This work was supported in part by the Ministry of Edu¬ because of hypertension. Thalidomide 150 mg daily re¬
cation, Science, and Culture of Japan and the Ministry of sulted in a nearly complete healing in 3 months. Pro¬
Health and Welfare of Japan, Tokyo. gressive decrease of the dosage down to 50 mg every other
We thank Sumitomo Metal Industries Ltd, Tokyo, for day was not followed by a relapse after 15 months of fol¬
the enzyme-linked immunosorbent assay kit (Smitest) used low-up, and no significant side effects developed.
in this study to detect antibody to HCV core peptides and
Comment. Thalidomide acts as an immunomodulatory
Japan Roche, Tokyo, for the HCV detection kit (Amplicor). and anti-inflammatory drug but through currently un¬
1. Pawlotsky JM, Dhumeaux D, Bagot M. Hepatitis C virus in dermatology: a known pathways. It is used in the treatment of several
review. Arch Dermatol. 1995;131:1185-1193.
2. Kanazawa K, Yaoita H, Tsuda F, Murata K, Okamoto H. Association of pru- inflammatory mucocutaneous diseases, particularly ery¬
rigo with hepatitis C virus infection. Arch Dermatol. 1995;131:852-853. thema nodosum leprosum, discoid lupus erythemato-
3. Aikawa T, Kimura I, Kojima M, et al. Cold activation of complement in sera
from patients with persistent hepatitis C virus infection on interferon therapy. sus, nodular prurigo, and recurrent giant aphthosis.l The
J Gastroenterol Hepatol. 1996;11:341-346. efficacy of thalidomide in LP has not been clearly as¬
4. Okamoto H, Kobata S, Tokita H, et al. A second-generation method of geno- sessed, but the drug is of potential interest in this in¬
typing hepatitis C virus by the polymerase chain reaction with sense and an-
tisense primers deduced from the core gene. J Virol Methods. 1996;57:31-45. flammatory condition of unknown origin as demon¬
5. de Noronha R, Taylor BA, Wild G, Triger DR, Greaves M. Inter-relationships strated by improvement of 2 cases of classic oral and
between platelet count, platelet IgG, serum IgG, immune complexes and
severity of liver disease. Clin Lab Haematol. 1991;13:127-135.
generalized LP.2,3 Our results suggest that oral thalido¬
mide may be an effective therapeutic alternative in dis¬
abling acral erosive LP without significant toxic reac¬
tions and allowing long-lasting remission. Indeed, the
Erosive Lichen Planus: clinical results were quickly obtained in the 2 cases, al¬
Dramatic Response to Thalidomide lowing a fundamental improvement of the quality of life
in the first patient, who had ideas of suicide because of
the disability caused by LP. No relapse, even moderate,

The
erosive variant of lichen planus (LP) is an

infrequent but disabling disease that can lead to was observed while tapering down the dosage of the drug.
major functional impairment of the affected
a The sole noticeable side effect observed in our 2 pa¬
areas, mainly the mucous membranes and the extrem- tients was a mild lymphopenia with no consequence, a

Downloaded From: http://archderm.jamanetwork.com/ by a University of Iowa User on 05/31/2015