1 - Front Cover [Seite 1]
2 - Cutaneous Lymphomas [Seite Cutaneous Lymphomas]
- 2 [Seite 2]
3 - copyright [Seite copyright]
- 3 [Seite 3]
4 - Contributors [Seite 4]
5 - Contents [Seite 6]
6 - Surgical Pathology Clinics [Seite Surgical Pathology Clinics]
- 8 [Seite 8]
7 - Preface [Seite Preface]
- 10 [Seite 10]
8 - A General Approach to the Diagnosis of Cutaneous Lymphomas and Pseudolymphomas [Seite 12]
8.1 - Abstract [Seite 12]
8.2 - Key points [Seite 12]
8.3 - Overview [Seite 12]
8.4 - References [Seite 19]
9 - Mycosis Fungoides [Seite 20]
9.1 - Abstract [Seite 20]
9.2 - Key points [Seite 20]
9.3 - Overview [Seite 20]
9.4 - Clinical features [Seite 20]
9.5 - Diagnosis: microscopic features [Seite 24]
9.6 - Diagnosis: ancillary studies [Seite 30]
9.7 - Differential diagnosis [Seite 34]
9.8 - Prognosis [Seite 42]
9.9 - References [Seite 43]
10 - Mycosis Fungoides Variants [Seite 46]
10.1 - Abstract [Seite 46]
10.2 - Key points [Seite 46]
10.3 - Overview [Seite 46]
10.4 - Folliculotropic mycosis fungoides [Seite 47]
10.4.1 - Overview [Seite 47]
10.4.2 - Clinical Features [Seite 47]
10.4.3 - Diagnosis: Microscopic Features [Seite 47]
10.4.4 - Diagnosis: Ancillary Studies [Seite 51]
10.4.5 - Differential Diagnosis [Seite 51]
10.4.6 - Prognosis [Seite 51]
10.5 - Pagetoid reticulosis (Woringer-Kolopp disease) [Seite 52]
10.5.1 - Overview [Seite 52]
10.5.2 - Clinical Features [Seite 52]
10.5.3 - Diagnosis: Microscopic Features [Seite 53]
10.5.4 - Diagnosis: Ancillary Studies [Seite 53]
10.5.5 - Differential Diagnosis [Seite 53]
10.5.6 - Prognosis [Seite 55]
10.6 - Granulomatous slack skin syndrome [Seite 55]
10.6.1 - Overview [Seite 55]
10.6.2 - Clinical Features [Seite 56]
10.6.3 - Diagnosis: Microscopic Features [Seite 56]
10.6.4 - Diagnosis: Ancillary Studies [Seite 56]
10.6.5 - Differential Diagnosis [Seite 56]
10.6.6 - Prognosis [Seite 58]
10.7 - Hypopigmented mycosis fungoides [Seite 58]
10.7.1 - Overview [Seite 58]
10.7.2 - Clinical Features [Seite 59]
10.7.3 - Diagnosis: Microscopic Features [Seite 59]
10.7.4 - Diagnosis: Ancillary Studies [Seite 59]
10.7.5 - Differential Diagnosis [Seite 60]
10.7.6 - Prognosis [Seite 60]
10.8 - Other rare variants of MF [Seite 60]
10.9 - References [Seite 64]
11 - Sézary Syndrome [Seite 68]
11.1 - Abstract [Seite 68]
11.2 - Key points [Seite 68]
11.3 - Overview [Seite 68]
11.4 - Clinical features [Seite 69]
11.5 - Diagnosis: microscopic features [Seite 70]
11.6 - Diagnosis: ancillary studies [Seite 73]
11.7 - Differential diagnosis [Seite 76]
11.8 - Prognosis [Seite 77]
11.9 - References [Seite 78]
12 - Cutaneous CD30-Positive Lymphoproliferative Disorders [Seite 80]
12.1 - Abstract [Seite 80]
12.2 - Overview [Seite 80]
12.3 - LyP [Seite 81]
12.3.1 - Overview [Seite 81]
12.3.2 - Diagnosis: Clinical Features [Seite 81]
12.3.3 - Diagnosis: Microscopic Features [Seite 82]
12.3.4 - Diagnosis: Ancillary Studies [Seite 85]
12.3.5 - Differential Diagnosis [Seite 89]
12.3.6 - Prognosis [Seite 91]
12.4 - Primary cutaneous anaplastic large-cell lymphoma [Seite 93]
12.4.1 - Overview [Seite 93]
12.4.2 - Clinical Features [Seite 94]
12.4.3 - Diagnosis: Microscopic Features [Seite 94]
12.4.4 - Diagnosis: Ancillary Studies [Seite 97]
12.4.5 - Differential Diagnosis [Seite 97]
12.4.6 - Prognosis [Seite 98]
12.5 - Borderline lesions [Seite 99]
12.6 - CD30 as prognostic marker and expression in other lymphomas [Seite 100]
12.7 - CD30 as therapeutic marker [Seite 100]
12.8 - References [Seite 101]
13 - CD30-Negative Cutaneous T-Cell Lymphomas Other than Mycosis Fungoides [Seite 106]
13.1 - Abstract [Seite 106]
13.2 - Key points [Seite 106]
13.3 - Overview [Seite 106]
13.4 - Subcutaneous panniculitis-like T-cell lymphoma [Seite 107]
13.4.1 - Overview [Seite 107]
13.4.2 - Clinical Features [Seite 107]
13.4.3 - Diagnosis: Microscopic Features [Seite 108]
13.4.4 - Diagnosis: Ancillary Studies [Seite 108]
13.4.5 - Differential Diagnosis [Seite 110]
13.4.6 - Prognosis [Seite 110]
13.5 - Extranodal NK/T-cell lymphoma, nasal type [Seite 110]
13.5.1 - Overview [Seite 110]
13.5.2 - Clinical Features [Seite 110]
13.5.3 - Diagnosis: Microscopic Features [Seite 110]
13.5.4 - Diagnosis: Ancillary Studies [Seite 110]
13.5.5 - Differential Diagnosis [Seite 111]
13.5.6 - Prognosis [Seite 111]
13.6 - Hydroa vacciniforme-like lymphoma [Seite 111]
13.6.1 - Overview [Seite 111]
13.6.2 - Clinical Features [Seite 111]
13.6.3 - Diagnosis: Microscopic Features [Seite 113]
13.6.4 - Diagnosis: Ancillary Studies [Seite 114]
13.6.5 - Differential Diagnosis [Seite 114]
13.6.6 - Prognosis [Seite 114]
13.7 - Primary cutaneous gamma-delta T-cell lymphoma [Seite 114]
13.7.1 - Overview [Seite 114]
13.7.2 - Clinical Features [Seite 114]
13.7.3 - Diagnosis: Microscopic Features [Seite 115]
13.7.4 - Diagnosis: Ancillary Studies [Seite 115]
13.7.5 - Differential Diagnosis [Seite 115]
13.7.6 - Prognosis [Seite 115]
13.8 - Primary cutaneous aggressive epidermotropic CD8-positive cytotoxic T-cell lymphoma (provisional entity) [Seite 116]
13.8.1 - Overview [Seite 116]
13.8.2 - Clinical Features [Seite 116]
13.8.3 - Diagnosis: Microscopic Features [Seite 116]
13.8.4 - Diagnosis: Ancillary Studies [Seite 116]
13.8.5 - Differential Diagnosis [Seite 116]
13.8.6 - Prognosis [Seite 118]
13.9 - Primary cutaneous CD4-positive small/medium-sized pleomorphic T-cell lymphoma (provisional entity) [Seite 118]
13.9.1 - Overview [Seite 118]
13.9.2 - Clinical Features [Seite 118]
13.9.3 - Diagnosis: Microscopic Features [Seite 118]
13.9.4 - Diagnosis: Ancillary Studies [Seite 121]
13.9.5 - Differential Diagnosis [Seite 121]
13.9.6 - Prognosis [Seite 123]
13.10 - Primary cutaneous peripheral T-cell lymphoma, not otherwise specified [Seite 125]
13.10.1 - Overview [Seite 125]
13.10.2 - Clinical Features [Seite 125]
13.10.3 - Diagnosis: Microscopic Features [Seite 125]
13.10.4 - Diagnosis: Ancillary Studies [Seite 125]
13.10.5 - Differential Diagnosis [Seite 126]
13.10.6 - Prognosis [Seite 127]
13.11 - References [Seite 127]
14 - Primary Cutaneous B-Cell Lymphomas [Seite 130]
14.1 - Abstract [Seite 130]
14.2 - Overview [Seite 130]
14.3 - PCMZL [Seite 131]
14.3.1 - Overview/Definition [Seite 131]
14.3.2 - Clinical Features [Seite 131]
14.3.3 - Diagnosis: Microscopic Features [Seite 131]
14.3.4 - Diagnosis: Ancillary Studies [Seite 132]
14.3.5 - Differential Diagnosis [Seite 137]
14.3.6 - Prognosis [Seite 142]
14.4 - PCFCL [Seite 144]
14.4.1 - Overview/Definition [Seite 144]
14.4.2 - Clinical Features [Seite 144]
14.4.3 - Diagnosis: Microscopic Features [Seite 145]
14.4.4 - Diagnosis: Ancillary Studies [Seite 147]
14.4.5 - Differential Diagnosis [Seite 148]
14.4.6 - Prognosis [Seite 151]
14.5 - PCDLBCL-LT [Seite 151]
14.5.1 - Overview/Definition [Seite 151]
14.5.2 - Clinical Features [Seite 151]
14.5.3 - Diagnosis: Microscopic Features [Seite 151]
14.5.4 - Diagnosis: Ancillary Studies [Seite 151]
14.5.5 - Differential Diagnosis [Seite 153]
14.5.6 - Prognosis [Seite 157]
14.6 - Acknowledgments [Seite 158]
14.7 - References [Seite 158]
15 - Index [Seite 162]
A General Approach to the Diagnosis of Cutaneous Lymphomas and Pseudolymphomas
Antonio Subtil, MD, MBAantonio.subtil@yale.edu, Yale Dermatopathology Laboratory, Yale School of Medicine, 15 York Street, LMP 5031, New Haven, CT 06520-8059, USA
Abstract
Both pathology and dermatology rely heavily on a visual approach to learning, generating differential diagnoses, and making diagnoses. Pattern recognition and the development of diagnostic frameworks are essential elements in this process. In addition to images, several algorithms and summaries are presented in this article to provide a practical approach to the diagnosis of cutaneous lymphoproliferative disorders. The critical importance of clinical pathologic correlation is also emphasized.
Keywords
Cutaneous lymphomas
Pseudolymphomas
Skin
T-cell
B-cell
Lymphoma
Diagnosis
Histopathology
Dermis
Epidermis
Key points
• Both pathology and dermatology rely heavily on a visual approach to learning, generating differential diagnoses (DDx), and making diagnoses.
• Pattern recognition and the development of diagnostic frameworks are essential elements in this process.
• In addition to images, several tables and algorithms are presented in this article to provide a practical approach to the diagnosis of cutaneous lymphoproliferative disorders.
• The critical importance of clinical pathologic correlation is also emphasized.
Overview
This issue on “Cutaneous Lymphomas” in Surgical Pathology Clinics provides an organized and updated review of this challenging topic by an international team of experts and bridges critical knowledge gaps in the diagnosis of cutaneous lymphomas. In addition to multiple clinical and microscopic images, several tables are presented to aid in diagnosis and staging. Both common and rare entities are reviewed—mycosis fungoides (MF), unique clinicopathologic variants of MF, cutaneous CD30-positive lymphoproliferative disorders, T-cell lymphomas without clinical features of MF or CD30 expression, and cutaneous B-cell lymphomas. For all entities, careful correlation of clinical and histopathologic findings is essential to arrive at the correct diagnosis (Fig. 1).
Fig. 1 Clinical pathologic correlation is essential to the diagnosis of skin lymphoma (HE, original magnification ×600).
Considering the heterogeneity and complexity of cutaneous lymphoproliferative disorders,1–3 this introductory article includes several tables and algorithms to provide a practical and logical approach to DDx based on different clinical findings (Fig. 2, Table 1) and histopathologic patterns (Boxes 1–6, Figs. 3–7). Within the differential list, each diagnostic possibility includes a link to a corresponding article for further information. In addition, it is important to recognize that several diseases (benign and malignant) may mimic cutaneous lymphomas. A comprehensive list of pseudolymphomas is provided in Box 7.
Box 1 Differential diagnosis of large cell lymphoid infiltrate (>25%–30% large cells)
• Large cell transformation of MF
See the article by Pincus elsewhere in this issue.
• LyP type C
The histologic features of LyP type C and pcALCL as well as sALCL are identical. To separate these entities, integration of clinical presentation and staging results as well as phenotypic and genetic findings are mandatory. See the article by Kempf elsewhere in this issue.
• Cutaneous anaplastic large cell lymphoma
See the article by Kempf elsewhere in this issue.
• Some cases of aggressive cytotoxic cutaneous lymphomas
See the article by Willemze elsewhere in this issue.
• Primary cutaneous peripheral T-cell lymphoma, unspecified
See the article by Willemze elsewhere in this issue.
• Primary cutaneous diffuse large B-cell lymphoma, leg type
See the article by Sundram elsewhere in this issue.
Abbreviations: LyP, lymphomatoid papulosis; pcALCL, primary cutaneous anaplastic large cell lymphoma; sALCL, systemic anaplastic large cell lymphoma.
Box 2 Differential diagnosis of perifollicular accentuation (lymphoid infiltrate around hair follicles)
• Folliculotropic MF
See the article by Martinez-Escala and colleagues elsewhere in this issue.
• Primary cutaneous marginal zone lymphoma
See the article by Sundram elsewhere in this issue.
• Some cases of lymphomatoid papulosis
See the article by Kempf elsewhere in this issue.
• Cutaneous pseudolymphoma: herpes folliculitis, pseudolymphomatous folliculitis, persistent arthropod bite reactions (see Box 7)
Box 3 Differential diagnosis of cutaneous lymphoid infiltrate with frequent eosinophils
• Folliculotropic MF
See the article by Martinez-Escala and colleagues elsewhere in this issue.
• Lymphomatoid papulosis type A
See the article by Kempf elsewhere in this issue.
• Cutaneous pseudolymphoma: lymphomatoid drug eruption, persistent arthropod bite reactions, scabies, exaggerated bitelike reactions in the setting of systemic hematologic disorders (see Box 7)
Box 4 Differential diagnosis of CD8 expression
• Lymphomatoid papulosis type D
See the article by Kempf elsewhere in this issue.
• Some cases of otherwise classical MF
See the article by Pincus elsewhere in this issue.
• Many cases of pagetoid reticulosis
See the article by Martinez-Escala and colleagues elsewhere in this issue.
• Some cases of cutaneous anaplastic large cell lymphoma
See the article by Kempf elsewhere in this issue.
• Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma
See the article by Willemze elsewhere in this issue.
• Subcutaneous panniculitis-like T-cell lymphoma
See the article by Willemze elsewhere in this issue.
• Many cases of cutaneous gamma/delta T-cell lymphoma
See the article by Willemze elsewhere in this issue.
• Indolent CD8+ lymphoid proliferation of the ear
See the article by Willemze elsewhere in this issue.
• Cutaneous pseudolymphoma: CD8+ infiltrates in the setting of advanced AIDS (see Box 7)
Box 5 Differential diagnosis of CD30 expression
• Lymphomatoid papulosis
See the article by Kempf elsewhere in this issue.
• Cutaneous anaplastic large cell lymphoma
See the article by Kempf elsewhere in this issue.
• Many (but not all) cases of large cell transformation of MF
See the article by Pincus elsewhere in this issue.
• Some cases of folliculotropic MF and pagetoid reticulosis
See the article by Martinez-Escala and colleagues elsewhere in this...
