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Lymphoproliferative Disorders

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Severe Skin Diseases in Children

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Abstract

The diagnosis of a lymphoproliferative disorder in a pediatric patient is often difficult: These rare conditions are frequently unrecognized by primary care providers, and access to subspecialty dermatology care is limited for many patients. Upon the establishment of a correct diagnosis, patient and parental concerns escalate while the dermatologist ponders the severity of the disease and the risks and benefits of each possible therapeutic option. Treatment modalities vary from observation with judicious follow-up to antibiotics, immunosuppressants, and phototherapy. Many variables are balanced when the treatment regimen is enacted, including the following: the general health of the patient, patient and family ability for compliance, insurance coverage of off-label therapeutic medication usages, and geographic ease of in-office therapies and follow-up exams. Regardless of the treatment method chosen, a strong educational foundation for the patient, a patient-provider relationship of trust, and physician comfort with the treatment method used are imperative for optimal patient outcomes. In this chapter, treatment modalities, from simple to complex, for lymphoproliferative disorders such as pityriasis lichenoides, lymphomatoid papulosis, plasmacytoma, and cutaneous lymphoma will be reviewed.

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Abbreviations

ALCL:

Anaplastic large cell lymphoma

ALK:

Anaplastic lymphoma receptor kinase

bbUVB:

Broadband Ultraviolet B

CLH:

Cutaneous lymphoid hyperplasia

CTCL:

Cutaneous T-cell lymphoma

FUMHD:

Febrile ulceronecrotic Mucha-Habermann disease

LyP:

Lymphomatoid papulosis

MED:

Minimal erythema dose

MF:

Mycosis fungoides

MTX:

Methotrexate

MZL:

Marginal zone lymphoma

nbUVB:

Narrowband Ultraviolet B

PCBCL:

Primary cutaneous B-cell lymphoma

PL:

Pityriasis lichenoides

PLC:

Pityriasis lichenoides chronica

PLEVA:

Pityriasis lichenoides et varioliformis acuta

PUVA:

Psoralen-ultraviolet A

UV:

Ultraviolet

UVA:

Ultraviolet A

UVA-1:

Ultraviolet A-1

UVB:

Ultraviolet B

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Authors and Affiliations

  1. Department of Dermatology, Mayo Clinic, 200 First Street SW, Mayo Building East 5, Rochester, MN, 55905, USA

    Christian L. Baum MS, MD & Dawn M. Davis MD

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  1. Christian L. Baum MS, MD
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  2. Dawn M. Davis MD
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Correspondence to Dawn M. Davis MD .

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  1. Pediatric & Adolescent Dermatology, Rady Children's Hospital, San Diego, California, USA

    Wynnis L. Tom

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Baum, C.L., Davis, D.M. (2014). Lymphoproliferative Disorders. In: Tom, W. (eds) Severe Skin Diseases in Children. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-39532-1_13

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  • DOI: https://doi.org/10.1007/978-3-642-39532-1_13

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