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Review
. 2017 Apr;31(2):317-334.
doi: 10.1016/j.hoc.2016.11.006.

CD30+ Lymphoproliferative Disorders of the Skin

Maxwell B Sauder  1 , John T O'Malley  1 , Nicole R LeBoeuf  2
Affiliations
Review

CD30+ Lymphoproliferative Disorders of the Skin

Maxwell B Sauder et al. Hematol Oncol Clin North Am. 2017 Apr.

Abstract

Primary cutaneous CD30+ lymphoproliferative disorders encompass lymphomatoid papulosis (LyP), primary cutaneous anaplastic large cell lymphoma (pcALCL), and indeterminate cases. LyP is a benign disorder characterized by recurrent crops of red or violaceous papulonodules. Patients with LyP are at an increased risk of a secondary malignancy. pcALCL is characterized by a solitary red to violaceous nodule or tumor larger than 20 mm. LyP is benign, is limited to the skin, and self-resolves, with a 5-year survival rate of 100%; pcALCL is limited to the skin and responsive to directed therapies, with a 5-year survival rate of over 95%. Aggressive chemotherapeutic regimens should be avoided.

Keywords: CD30(+); Cutaneous lymphoproliferative disorders; Lymphomatoid papulosis; Primary cutaneous anaplastic large cell lymphoma; Secondary cutaneous anaplastic large cell lymphoma.

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Figures

Figure 1
Figure 1
Primary cutaneous CD30+ LPD clinical spectrum
Figure 2
Figure 2
Clinical presentations of lymphomatoid papulosis (LyP)

  1. 2A – Typical lesion of LyP: 6mm violaceous papule with necrotic center

  2. 2B, C – Crops of LyP in various stages of evolution

  3. 2D – Inflamed lesion of LyP, with a surrounding crop of more typical lesions

Figure 2
Figure 2
Clinical presentations of lymphomatoid papulosis (LyP)

  1. 2A – Typical lesion of LyP: 6mm violaceous papule with necrotic center

  2. 2B, C – Crops of LyP in various stages of evolution

  3. 2D – Inflamed lesion of LyP, with a surrounding crop of more typical lesions

Figure 2
Figure 2
Clinical presentations of lymphomatoid papulosis (LyP)

  1. 2A – Typical lesion of LyP: 6mm violaceous papule with necrotic center

  2. 2B, C – Crops of LyP in various stages of evolution

  3. 2D – Inflamed lesion of LyP, with a surrounding crop of more typical lesions

Figure 2
Figure 2
Clinical presentations of lymphomatoid papulosis (LyP)

  1. 2A – Typical lesion of LyP: 6mm violaceous papule with necrotic center

  2. 2B, C – Crops of LyP in various stages of evolution

  3. 2D – Inflamed lesion of LyP, with a surrounding crop of more typical lesions

Figure 3
Figure 3
Diagnosis algorithm for primary cutaneous CD30+ LPD
Figure 4
Figure 4
Clinical presentations of primary cutaneous ALCL

  1. 4A – Typical pcALCL tumour: Red, friable 2.5cm tumor, well-defined with central crusting

  2. 4B -- pcALCL tumor measuring 5.5 x 7.2cm with central clearing and hemorrhagic crust

  3. 4C – Early pink plaque of pcALCL

  4. 4D – Multifocal, localized and ulcerative pcALCL

Figure 4
Figure 4
Clinical presentations of primary cutaneous ALCL

  1. 4A – Typical pcALCL tumour: Red, friable 2.5cm tumor, well-defined with central crusting

  2. 4B -- pcALCL tumor measuring 5.5 x 7.2cm with central clearing and hemorrhagic crust

  3. 4C – Early pink plaque of pcALCL

  4. 4D – Multifocal, localized and ulcerative pcALCL

Figure 4
Figure 4
Clinical presentations of primary cutaneous ALCL

  1. 4A – Typical pcALCL tumour: Red, friable 2.5cm tumor, well-defined with central crusting

  2. 4B -- pcALCL tumor measuring 5.5 x 7.2cm with central clearing and hemorrhagic crust

  3. 4C – Early pink plaque of pcALCL

  4. 4D – Multifocal, localized and ulcerative pcALCL

Figure 4
Figure 4
Clinical presentations of primary cutaneous ALCL

  1. 4A – Typical pcALCL tumour: Red, friable 2.5cm tumor, well-defined with central crusting

  2. 4B -- pcALCL tumor measuring 5.5 x 7.2cm with central clearing and hemorrhagic crust

  3. 4C – Early pink plaque of pcALCL

  4. 4D – Multifocal, localized and ulcerative pcALCL

Figure 5
Figure 5
Cutaneous involvement of ALK- systemic ALCL, with annular plaques and tumors. Note evidence of scarring at prior sites on the arm and trunk.
See this image and copyright information in PMC

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