ARTICLE
Auteur(s) : Susanne
Topf1, Matthias Lüftl1, Ulrich
Neisius1, Thomas Brabletz2, Miklos Simon
Jr.1, Gerold Schuler1, Erwin S
Schultz1
1Department of Dermatology, University Hospital
Erlangen, Hartmannstr. 14, D-91052 Erlangen, Germany
2Department of Pathology, Friedrich-Alexander University
Erlangen-Nuremberg, Krankenhausstr. 8-10, 91054 Erlangen,
Germany
accepté le 4 Mai 2005
Mycosis fungoides (MF) represents a low-risk, cutaneous,
non-Hodgkin, T-cell lymphoma with a wide spectrum of
clinicopathological manifestations and therefore it may mimic a
number of other dermatoses [1].Mycosis fungoides palmaris et
plantaris is a rare variant of mycosis fungoides-type cutaneous T
cell lymphoma (CTCL) [1-9] predominantly affecting the palms and
/or soles, sometimes extending to fingers, arms and feet [2, 3].
Clinical variations range from hyperkeratotic [4, 6, 10, 11] or
dyshidrotic lesions, pustules [12], hyperpigmented patches and
plaques, psoriasiform plaques [9], verrucous changes, ulcerations
or nail dystrophy.We report on an 18-year-old patient with a 5-year
history of non-classified “chronic palmoplantar eczema” poorly
responding to standard therapies. Histopathological,
immunohistochemical and molecular findings revealed an atypical
case of MF. This differential diagnosis should be kept in mind when
dealing with palmoplantar eczema which is refractory to common
treatment modalities [4-6, 8, 13].
Case report
An 18-year-old patient presented with a 5-year history of
non-classified “chronic palmoplantar eczema”, which had been
refractory to different therapies including topical
glucocorticoids. Beginning on the right hand 5 years previously,
the number of cutaneous lesions increased and similar lesions
appeared on the other hand and feet. In the course of the disease,
hands, feet, and partially the elbows and knees became involved.
The patient presented with discrete erythematous, hyperkeratotic
papules on the palms and soles, partially showing dark comedo-like
plugs (( figure
1 )). A more prominent finding was erythematous plaques,
again with formation of comedo-like plugs, predominantly affecting
the metacarpophalangeal joints (( figure 2 )A and 2B). In
addition, psoriasis-like erythematosquamous plaques were found on
the elbows and knees. No other cutaneous involvement was
observed.
Histopathology of a skin biopsy showed a dense, band-like
lymphocytic infiltrate in the upper dermis. Abnormal lymphocytes
with slightly enlarged nuclei were found throughout the entire
epidermis in association with scant spongiosis (( figure 3 )). Focally, the
cornification was distinctly altered with areas of comedo-like
parakeratotic plugs. There was no deep lymphocytic infiltrate
surrounding and infiltrating the hair follicles or eccrine glands
as is found in follicular or syringotropic MF. Immunohistochemical
staining documented that the infiltrating cells were mainly CD4+
(80%) with only 20% of the lymphocytes expressing CD8 (( figure 4 )). There was
no expression of CD30 detectable. Analysis of the common γ-chain of
the T-cell-receptor by polymerase chain reaction revealed a
monoclonal rearrangement (( figure 5 )). Physical
examination, abdominal/lymph node ultrasound, chest X-ray and
laboratory blood tests – including chemical and hematological
analysis as well as peripheral blood examination for circulating
Sezary cells – showed no evidence of systemic involvement.
Taken together these results allowed the diagnosis of MF
palmaris et plantaris T1N0M0 according to the EORTC classification.
The patient received topical treatment with class IV
glucocorticoids (clobetasol propionate) and topical
photochemotherapy with psoralen and ultraviolet A radiation (PUVA)
four times weekly. After 2 weeks partial remission of the lesions
was observed, but unfortunately, the patient was lost for further
follow-up.
Discussion
MF may have various clinical expressions [1, 7, 14-18]. Distinct
variants may show skin involvement restricted to defined areas of
the body only. MF palmaris et plantaris was recently introduced as
a distinct variant that groups patients with skin lesions confined
to the palms and soles based on clinical and histopathologic
MF-like characteristics [3, 7, 13].
The prevalence of MFPP among MF patients is said to be 0.6% [1,
3]. The lesions are either strictly confined to the palms and/or
soles or may extend to the feet, arms and fingers [1]. In most
cases the disease remains restricted to the initial area and shows
a slow benign course, lacking dissemination and extracutaneous
spread. If typical MF lesions somewhere else on the body do not
accompany these changes, the clinical diagnosis can be
challenging.
A wide spectrum of clinical differential diagnoses, including
dyshidrotic eczema, contact dermatitis, lichen planus, verrucae,
palmoplantar psoriasis, mycotic infections, and granuloma anulare
have to be considered [3, 14, 15, 19]. The benign course of the
disease and partial remission to conventional therapies may lead to
a delay in establishing the right diagnosis and adequate therapy.
In our patient the skin lesions reminded us in particular of a
circumscribed juvenile pityriasis rubra pilaris which can present
with keratotic papules surrounded by an orange-red erythema
confined to the extremities [20] but immunohistochemical and
molecular biological findings revealed a cutaneous T cell lymphoma.
Comedo-like plugs can also be found in follicular (pilotropic) MF
but histopathology revealed that there was no deep lymphocytic
infiltrate surrounding and infiltrating the hair follicles, as is
the case in this special form of MF.
Topical and systemic glucocorticoids usually lead to temporary
or partial remission without complete clearing of the lesions.
Longer lasting remissions can be achieved by topical
photochemotherapy. As further treatment modalities, topical
carmustine, electron-beam therapy, and CO2 laser treatment can be
listed [8, 13]. From our point of view, a higher awareness of this
disease entity as a relevant clinical differential diagnosis of
non-classified chronic eczematous lesions of the hands and soles
that respond poorly to standard therapies is important, since
therapeutic approaches to this disease variant differ from standard
symptomatic treatment regimes [7, 8].
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