Lymphomatoid Papulosis in Children

Lymphomatoid papulosis (LyP) is a clinically benign skin disorder characterized by chronic and recurrent self-healing papulonodular lesions. This disorder occurs rarely in children. The sites of lesions include the limbs and/or trunk. The eruption recurs episodically, often ulcerates, and heals spontaneously in 3-8 weeks, leaving an atrophic scar occasionally. LyP results from a clonal T-cell proliferation, which may explain its evolution or coexistence with Hodgkin disease, mycosis fungoides, or anaplastic large cell lymphoma (ALCL).

Histology

LyP is characterized by superficial and deep infiltrates consisting of atypical lymphocytes with a hyperchromatic, pleomorphic nucleus that can obscure the der-moepidermal junction. Similar cellular infiltrates can also be found in the epidermis along with spongiosis, parakeratosis, and neutrophils. Two types of activated T-cell infiltrates are found:

1. LyP type A lesions: LyP lesions of this cell type predominantly contain Ki-1+ (CD30+), Reed-Sternberg-type cells with a pale staining, convoluted nucleus, prominent nucleoli, and moderately basophilic cytoplasm.

2. LyP type B lesions: LyP lesions of this cell type contain the Sezary-type cell with a cerebriform, hyperchromatic nucleus and scant cytoplasm. Only some of these cells are CD30+.

The atypical cells of LyP have been shown to be of T-cell origin, in most cases expressing CD2, CD3, and CD4. However, these cells lack the expression of some of the usual pan-T-cell antigens, such as CD5, CD7, or both. Clonal rearrangement of the T-cell antigen receptor has been demonstrated in most of the cases studied. Thus, LyP serves as an example of a benign lymphoproliferative disorder in spite of the evidence of clonal T-cell receptor gene rearrangement.

A lymphoma evolving from lymphomatoid papulosis can be recognized by enlarging or persistent skin lesions, peripheral lymphadenopathy, or circulating atypical lymphocytes. A biopsy of suspicious skin lesions or enlarged lymph nodes for histologic, immunologic, cytogenetic, or gene rearrangement studies is indicated. It has also been suggested that a thorough physical examination every 6 months in children with lymphomatoid papulosis with attention to growth and development (as a tool in assessing occult malignancies in children), skin lesions, and lymph nodes should be carried out. Additional studies such as bone marrow examination and radioimaging studies should be performed as needed.

Table 13-8 shows the clinical and histologic differences among cutaneous CD30+ non-Hodgkin lymphoma (NHL) (including ALCL), borderline cases, and LyP.

Treatment

Treatment involves oral antibiotics, systemic corticosteroids, low-dose methotrexate, psoralen with ultraviolet radiation (PUVA), topical steroids, or ultraviolet beam.

Prognosis

The duration of LyP ranges from 1 to 40 years. Approximately 23 children have been described with Lyp and evolution to lymphomas has occurred in 2 of the 23 cases.

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