TRENDSin Immunology Vol.22 No.3 March 2001
http://immunology.trends.com 1471-4906/01/$ – see front matter © 2001 Elsevier Science Ltd. All rights reserved. PII: S1471-4906(00)01844-5
130 Opinion Opinion
Human epidermal melanocytes (MCs) function as a
pivotal protective barrier against ultraviolet (UV)
radiation and oxidative stress by generating the
radical-scavenging pigment melanin
1
. In addition,
they function in local immune reactivity as antigen-
processing and antigen-presenting cells (APCs)
2
. In
the disfiguring and psychologically debilitating
disease vitiligo, MCs are eliminated from the
lesional skin
3
in association with T-cell infiltrates
4,5
(Fig. 1). Recent data suggest that the destruction of
MCs in vitiligo is mediated by skin-homing
autoreactive T cells
6,7
and by additional specific and
nonspecific immune components, including
antibodies, complement factors and nitric oxide
(NO), aided by melanogenic metabolites such as
toxic ortho-quinones
1,8,9
.
Studies on host melanoma immunity show that
similar factors to those in vitiligo operate in the
destruction of malignantly transformed MCs. In
particular, the prevalence of antibodies and reactive
T cells against the differentiation antigens of MCs in
both vitiligo and melanoma patients demonstrates a
clear parallel between autoimmunity and tumour
immunity
1,10
. In vitiligo, natural immune tolerance is
over-ridden such that the host immune surveillance
system can orchestrate MC destruction, whereas in
melanoma, an immune effector function of potential
benefit to the host does not occur.
Although much information is available on the
mechanism of melanoma immunity, the
pathomechanism of vitiligo, in the context of
immunobiology of MCs, has received little attention
to date. This article presents the viewpoint that
autoimmunity and tumour immunity are two sides of
the same coin; by elucidating the molecular
pathogenesis of vitiligo, new therapeutic strategies
for either disease might be revealed.
MCs in normal human skin
Melanogenesis and pigmentation
MCs contribute to the prevention of skin cancer by
generating melanin within compartments called
melanosomes. Melanin-containing melanosomes are
a specialized lysosome of MCs, and are distributed
among surrounding keratinocytes (KCs) by a process
that involves transfer of complete melanosomal
organelles via dendrite-like processes that extend
from a melanocyte to neighbouring KCs. Compounds
specifically expressed in the melanosomal
compartment of the cell and involved in the
melanogenic pathway
1
are shown in Fig. 2. Briefly,
melanogenesis involves multiple catalytic
conversions starting from the amino acid tyrosine,
leading to the formation of either pheomelanin
(yellow/red melanin) or eumelanin (brown/black
melanin), depending on the composition of the
melanosome. Most of these melanosome-specific
compounds, except the P-protein, have been described
in recent years as target antigens in tumour
immunity and autoimmunity
11,12
.
MCs and the skin immune response
Apart from responding to environmental growth
factors and cytokines, MCs (like KCs) generate
cytokines themselves. These cytokines assist in the
maturation and migration of professional APCs
such as Langerhans cells (LCs), and in the
recruitment of immune infiltrates into the skin
13
.
Indeed, such cytokine production, combined with
the strategic position and dendritic morphology of
melanocytes, has led to the suggestion that MCs, in
conjunction with KCs and LCs, are active
participants within the skin immune response
14
(Box 1). In this regard, it was demonstrated that
melanocytes are capable of phagocytosis, and can
process and present antigens to MHC class II-
restricted T cells
2
. The processing of external
antigens appears to involve fusion of phagosomes
with melanosomes (Ref. 14), rather than with
lysosomes, because melanosomes take over all
lysosomal functions within the MC (Ref. 15). The
presentation of antigens to CD4
+
T cells is
supported by the expression of several
costimulatory molecules
2
, such as intercellular
adhesion molecule 1 (ICAM-1) and leukocyte
A symbiotic concept
of autoimmunity and
tumour immunity:
lessons from vitiligo
Pranab K. Das, René M.J.G.J. van den Wijngaard,
Anna Wankowicz-Kalinska and I. Caroline Le Poole
Vitiligo is a skin disease in which melanocytes (MCs) are eradicated from
lesional epidermis, resulting in disfiguring loss of pigment. MCs are destroyed
by M C-reactive T cells, as well as other non-immune and immune components.
Similarities exist between the autoimmunity observed in vitiligo and the
tumour immunity observed in melanoma immuno-surveillance. An analysis of
these mechanisms might lead to the development of new therapies for both
vitiligo and melanoma.
Pranab K. Das*
Dept of Pathology
René M.J.G.J. van den
Wijngaard
Dept of Gastroenterology
Anna Wankowicz-
Kalinska
Dept of Pathology,
Academic Medical Centre,
Amsterdam University,
Meibergdreef 9, 1105 AZ,
Amsterdam, The
Netherlands.
* e-mail: p.k.das@
amc.uva.nl
I. Caroline Le Poole
Skin Disease Research
Laboratories, Cardinal
Bernardin Cancer Center,
Loyola University,
Maywood, IL 60153, USA.
‘Studies on host melanoma
immunity show that similar factors
to those in vitiligo operate in the
destruction of malignantly
transformed MCs.’