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Assessment of Histological Criteria in The Diagnosis of Mycosis Fungoides
Assessment of Histological Criteria in The Diagnosis of Mycosis Fungoides
Report
Oxford, UK
International
IJD
Blackwell
?0011-9059
Science,
Journal
Ltdof Dermatology
2002
Histologic
Naraghi
Supplement
et criteria
al.
in mycosis fungoides
Zahra Safee Naraghi, MD, Hassan Seirafi, MD, Mahin Valikhani, MD, Forshad Farnaghi, MD,
Susan Kavusi, MD, and Yahya Dowlati
Abstract
Background The histologic diagnosis of early mycosis fungoides (MF) can be difficult to
establish in many instances because the subtle changes observed in patches of MF are also
present in many inflammatory dermatoses.
Methods To assess the frequency and significance of many of these histologic parameters, we
retrospectively reviewed 50 slides from patients with documented MF in patch, plaque, and
tumor stages. The diagnosis of MF was unequivocally established either by the progression of
patients to advanced stages of the disease or by indubitable histologic findings. In the second
phase of the study, we compared the histologic parameters observed in 24 patch stage MF
patients with those in 24 non-MF patients. The non-MF group were patients whose pathologic
pattern was suspicious for MF, but who definitely did not have MF on clinical grounds. The two
groups were matched by histologic pattern. Two different observers evaluated the slides and the
intensities of 32 histologic parameters were graded on a four-point scale to minimize the
subjective variability in the histologic reports.
Results On univariate analysis, the following parameters achieved significance in
distinguishing MF from non-MF: Pautriers microabscesses, haloed lymphocytes, disproportionate
epidermotropism, epidermal lymphocytes larger than dermal lymphocytes, hyperconvoluted
lymphocytes in the epidermis and dermis, absence of dyskeratosis, and papillary dermal fibrosis.
None of these features proved to have additional discriminating power on multivariate analysis.
Conclusions The efficacy of single histologic features in the diagnosis of early MF is generally
poor and, to discriminate MF from its inflammatory simulators, a combination of cytologic and
architectural features must be used.
Introduction
The histologic diagnosis of early stages of mycosis fungoides
(MF) may be difficult in many instances: early patches of
MF may show only subtle histologic changes that can easily
be confused with those of inflammatory dermatoses.1 In
approximately half of patients, biopsies are diagnostic of
MF from the outset, but, in many patients, histologic findings
are only suggestive of MF and repeat biopsies over time with
clinicopathologic correlation will clarify the correct diagnosis.2,3
Another problem is the low agreement rate in reporting
biopsies suggestive of MF among dermatopathologists due to
a lack of specifically defined histologic criteria.4
Immunophenotyping and T-cell receptor gene rearrangement
studies by polymerase chain reaction have been utilized as
adjunctive techniques to provide additional useful information
in difficult cases; however, the immunophenotypic aberrations
2003 The International Society of Dermatology
45
46
Naraghi et al.
Results
The results of the histologic evaluation of 50 slides from 30
patients in patch, plaque, and tumor stages of MF are summarized in Table 2.
We reviewed 24 slides from patch stage MF, 18 from
plaque stage MF, and eight from tumor stage MF; 60% of the
patients were males.11 The patients ages in the different
groups are given in Table 3. There were five cases below
30 years of age in patch stage MF, confirming that MF is not
a disease exclusively of older individuals, but also affects
young patients.12
The results of the second phase of our study are summarized
in Table 4. The P value for each parameter is recorded and the
sensitivity and specificity of all criteria are noted in Table 4.
In univariate analysis, we found that the degree of
epidermotropism, Pautriers microabscesses, haloed
lymphocytes, disproportionate epidermotropism, larger
epidermal lymphocytes, hyperconvoluted lymphocytes in the
2003 The International Society of Dermatology
Naraghi et al.
1+
2+
3+
4+
Atrophy
Acanthosis
Parakeratosis
Spongiosis
Intensity of epidermotropism
Pautriers microabscesses
Haloed lymphocytes
Disproportionate
epidermotropism
Epidermal > dermal
lymphocytes
Hyperconvoluted
epidermal lymphocytes
Dyskeratosis
Intensity of
dermal infiltrate
Eosinophils
Plasma cells
Histiocytes
Hyperconvoluted
dermal lymphocytes
Papillary fibrosis
Dermal edema
Telangiectasia
Perieccrine lymphocytes
Follicular mucinosis
Follicular infiltration
Vasculopathy
Mitosis
Pattern of
epidermotropism
Basal layer
None
None
None
None
None
None
None
None
Mild
Mild
< 10%
< 10%
1 5/40
Present/40
1 5/40
Mild
Moderate
Moderate
10 50%
10 50%
6 10/40
6 10/40
Moderate
Severe
Severe
> 50%
> 50%
> 10/40
> 10/40
Severe
None
10% larger/100
10 50% larger/100
None
1 5/100
5 10/100
> 10/100
None
Sparse
1 5/40
Mild perivascular
or lichenoid
1 5/40
1 5/40
1 5/40
1 5/100
6 10/40
Dense lichenoid
> 10/40
Very dense or deep
6 10/40
6 10/40
6 10/40
6 10/100
> 10/40
> 10/40
> 10/40
> 10/100
< 10%
< 10%
< 1 vessel
1 5/40
< 10%
1 5/40
Present
Present (> 3/500)
Pagetoid
10 50%
10 50%
1 3 vessels
6 10/40
10 50%
6 10/40
Mixed
> 50%
> 50%
> 3 vessels
> 10/40
> 50%
> 10/40
Perivascular
Diffuse vacuolar
change
Pattern of
dermal infiltrate
Location of
dermal infiltrate
Focal vacuolar
change
Lichenoid
Papillary dermis
Papillary and
reticular dermis
Monomorph
Small
Spongiotic
Lichenoid
Psoriasiform
Spongioticlichenoid
Spongiotic
psoriasiform
Lichenoid
psoriasiform
Spongioticlichenoid
psoriasiform
Polymorph
Small, medium
Granulomatous
Small, medium, large
Dermal infiltrate
Dermal lymphocytes
Pattern
None
None
None
None
None
None
None
None
None
None
None
None
Single lymphs
in basal layer
Intact
Papillary and
reticular dermis
and subcutis
47
48
Naraghi et al.
Patch % > 2+
Plaque % > 2+
Tumor % > 2+
Total % > 2+
Atrophy
Acanthosis
Parakeratosis
Spongiosis
Pautriers microabcesses
Haloed lymphocytes
Disproportionate epidermotropism
Epidermal > dermal lymphocytes
Hyperconvoluted epidermal lymphocytes
Dyskeratosis
Intensity dermal infiltrate
Eosinophils
Plasma cells
Histiocytes
Hyperconvoluted dermal lymphocytes
Papillary dermal fibrosis
Dermal edema
Telangiectasia
Perieccrine lymphocytes
Follicular mucinosis
Follicular infiltration
Vasculopathy
Mitosis
Pattern of epidermotropism
Single basal
Pagetoid
Mixed
Basal layer
Intact
Focal vacuolar
Diffuse vacuolar
Pattern of dermal infiltrate
Perivascular
Lichenoid
Location of dermal infiltrate
Papillary dermis
Papillary and upper reticular dermis
Papillary and reticular dermis
Papillary and reticular dermis
and subcutaneous fat
Deep reticular dermis
and subcutaneous fat
Dermal infiltrate
Monomorphous
Polymorphous
Granulomatous
Dermal lymphocytes
Small
Small, medium
Small, medium, large
Pattern
Spongiotic
Lichenoid
Psoriasiform
Spongioticlichenoid
Spongioticpsoriasiform
Lichenoidpsoriasiform
Spongioticlichenoidpsoriasiform
41.7
66.7
54.2
54.2
37.5
87.5
75
41.7
62.5
29.8
87.5
29.2
25
75
83.3
95.8
66.7
100
13
0
45.8
25
0
22.2
77.8
77.8
72.2
66
94.4
83
61.1
83.3
44.4
94.4
72.2
44.4
100
77.8
88.9
61.1
83.3
17.6
22.2
55.6
50
33.3
25
75
87.5
75
12.5
10
50
25
75
0
100
87.5
62.5
87.5
100
62.5
37.5
87.5
83.3
37.5
87.5
50
62.5
32
72
68
64
44
92
74
46
72
26
92
54
38
86
84
88
60
92
22
14
56
38
22
79
8
12.5
33
28
39
62.5
25
125
60
18
22
12.5
62.5
25
22
44
33
62.5
0
37.5
24
46
30
54
46
39
61
54
21
25
0
22
33
33
11
0
0
25
62.5
34
22
28
14
12.5
71
29
0
55.5
28
16.5
25
75
0
58
36
6
83
17
0
67
33
0
25
62.5
12.5
68
30
2
4
58
12.5
8
4
12.5
0
0
28
17
28
5
11
11
12.5
25
12.5
12.5
25
0
12.5
4
42
14
16
8
10
6
0
100
40
60
Naraghi et al.
Patch
Plaque
Tumor
Total
Range (years)
Mean (years)
17 77
47.2
30 78
57.2
42 75
58.7
17 78
52.7
49
50
Naraghi et al.
Parameter
Atrophy
Acanthosis
Parakeratosis
Spongiosis
Pautriers microabscesses
Haloed lymphocytes
Disproportionate epidermotropism
Epidermal > dermal lymphocytes
Hyperconvoluted epidermal lymphocytes
Dyskeratosis
Intensity dermal infiltrate
Eosinophils
Plasma cells
Histiocytes
Hyperconvoluted dermal lymphocytes
Papillary fibrosis
Dermal edema
Telangiectasia
Perieccrine lymphocytes
Follicular mucinosis
Follicular infiltration
Mitosis
Pattern of epidermotropism
Single basal
Pagetoid
Mixed
Basal layer
Intact
Focal vacuolar
Diffuse vacuolar
Pattern of dermal infiltrate
Perivascular
Lichenoid
Location of dermal infiltrate
Papillary dermis
Papillary and upper reticular dermis
Papillary and reticular dermis
Papillary and reticular dermis and subcutis
Dermal infiltrate
Monomorphous
Polymorphous
Granulomatous
Dermal lymphocytes
Small
Small, medium
Medium
Pattern
Spongiotic
Lichenoid
Psoriasiform
Spongioticlichenoid
Spongioticpsoriasiform
Lichenoidpsoriasiform
Spongioticlichenoidpsoriasiform
Patch MF > 2+
(%)
Non-MF > 2+
(%)
Sensitivity
(%)
Specificity
(%)
P
value
41.7
66.7
54.2
54.2
37.5
87.5
75
41
62.5
20.8
87.5
29.2
25
75
83.3
95.8
66.7
100
13
0
45.8
0
50
50
33.3
75
0
33.3
8.3
0
25
70.8
75
29.2
12.5
91.7
12.5
50
87.5
91.7
15
0
63.6
0
42
67
54
54
37.5
87.5
75
41
62.5
21
87.5
29
25
75
83
96
67
100
13
0
46
0
50
50
67
25
100
67
92
100
75
29
25
71
87.5
8
87.5
50
12.5
8
85
36
0.77
0.38
0.24
0.22
0.002
0
0
0.001
0.019
0.001
0.46
1
0.46
0.24
0
0.001
0.168
0.489
0.712
0.14
79
8
12.5
87.5
0
12.5
79
8
12.5
12.5
100
87.5
0.35
12.5
62.5
25
20.8
29.1
50
12.5
62.5
25
79
71
50
0.067
54
46
58
42
54
46
42
58
54
21
25
0
37.5
25
33
4
54
21
25
0
62.5
75
67
96
0.49
71
29
0
62.5
37.5
0
71
29
0
37.5
62.5
0.76
83
12.5
4
0
100
100
0.113
83
12.5
4
4
58
12.5
8
4
12.5
0
100
0
0
4
58
12.5
8
4
12.5
0
Naraghi et al.
51
52
Naraghi et al.