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Cutaneous T-cell lymphoma in Child
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Cutaneous T-cell lymphoma in Child

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Contributors: Vivian Wong MD, PhD, Belinda Tan MD, PhD, Art Papier MD, Susan Burgin MD
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Synopsis

Primary cutaneous lymphomas may be either of T- or B-cell origin. Cutaneous T-cell lymphomas (CTCLs) account for 75%-80% of these lymphomas and are a heterogeneous group of neoplasms that vary considerably in their clinical presentation, histology, immunophenotype, genetics, and prognosis. CTCLs are seen most often in the elderly, but they can occur in patients of all ages. CTCL in children is rare, accounting for roughly 5% of all cases. It has been reported to occur as early as the second year of life. There is little literature evidence on the progression of CTCL in children and its management.

The definitive diagnosis of CTCL may require large or multiple biopsies as well as specialized testing of the biopsy specimens. Typing of the CTCL and staging are important to determine the extent of disease and treatment strategy.

Mycosis fungoides and its variants (folliculotropic mycosis fungoides, pagetoid reticulosis, and granulomatous slack skin), Sézary syndrome, lymphomatoid papulosis, and cutaneous anaplastic large cell lymphoma make up 90% of all CTCL cases.

This summary focuses on mycosis fungoides and variants and Sézary syndrome.

Other types of CTCL discussed separately include subcutaneous panniculitis-like T-cell lymphoma, adult T-cell leukemia / lymphoma, and nasal type extranodal NK/T-cell lymphoma.
 
Mycosis Fungoides
Mycosis fungoides (MF) is the most common type of CTCL, accounting for 50% of all primary CTCL cases. Erythematous patches and plaques with fine scale and tumors that anatomically favor the buttocks and sun-protected areas of the trunk and limbs characterize this subtype. The etiology remains unclear. Current hypotheses propose that persistent antigenic stimulation occurs and that CD8+ T-cells play a critical role. MF takes on an indolent course over years to decades. Associated features include pruritus, poikiloderma, and ulceration of tumors. Extracutaneous involvement correlates with generalized skin involvement and erythroderma. Neoplastic T-cells demonstrate a memory T-cell phenotype: CD3+, CD4+, CD45RO+. MF staging requires CTCL physician specialists. Some consider large plaque parapsoriasis to be early patch stage MF.

Hypopigmented MF – A hypopigmented variant of MF is seen primarily in individuals of African descent but has also been reported in those of Asian, Indian, and Latin American descent. The hypopigmented variant is the most common form seen in childhood.

Folliculotropic MF – This is characterized by lesions on the head and neck clinically and follicular involvement ("folliculotropism") microscopically. Most cases are seen in adult males, but children and adolescents may also be affected. It is a rare variant in childhood. Early stage (IA, IB) disease with patches and plaques with follicular accentuation and keratosis pilaris-like or acneiform lesions may exhibit better prognosis with an indolent course. These lesions have a predilection for the trunk and extremities. On the other hand, advanced stage (IIB) disease with indurated or infiltrative alopecic plaques and tumors may be more aggressive with poorer prognosis. These lesions favor the head and neck and are more pruritic. 5-year survival rate approaches 80%.

Pagetoid reticulosis – Pagetoid reticulosis, also known as Woringer-Kolopp disease, is rarely reported in childhood. It represents approximately 1% of all cases of CTCL. It is a localized variant of MF, characterized by a solitary, slow-growing patch or plaque on a distal extremity clinically and striking epidermotropism microscopically. Pagetoid reticulosis has a predilection for middle-aged males but it can be seen in any age group. Prognosis is excellent with a 100% 5-year survival rate.

Ketron-Goodman disease is a disseminated and aggressive variant of pagetoid reticulosis with a potential for systemic involvement.

Granulomatous slack skin – Granulomatous slack skin, also known as granulomatous dermohypodermitis, is a rare variant of CTCL (<1% of all cases) that is very rarely seen in childhood. It is characterized by asymptomatic pendulous loose skin masses in the body folds clinically, and granulomatous T-cell infiltration microscopically. The disease may be associated with a lymphoproliferative disorder, such as MF, Hodgkin lymphoma, and non-Hodgkin lymphoma. Males in the third and fourth decades are more commonly affected. Overall, prognosis is good with a 100% 5-year survival rate.

Sézary Syndrome
While erythrodermic MF has been reported in childhood, true Sézary syndrome has not.

Codes

ICD10CM:
C84.00 – Mycosis fungoides, unspecified site

SNOMEDCT:
118618005 – Mycosis fungoides

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Hypopigmented variant of MF (common in children):
Patch and plaque stage MF:
  • Atopic dermatitis – Common in childhood, and CTCL can present with thin, scaly plaques as does atopic dermatitis. Mild to moderate spongiosis is seen on histology. Look for lichenified plaques on the flexural surfaces and neck.
  • Superficial fungal infections (eg, tinea corporis) – Erythematous plaques with a raised, red scaling border. Central clearing is more common in tinea corporis. Check KOH.
  • Seborrheic dermatitis – Sebaceous distribution of erythematous scaling plaques. Chronic history of mesiolabial, glabellar, auricular pruritus, erythema, scale.
  • Chronic contact dermatitis (allergic, irritant) – Bright red erythematous or vesiculating plaques.
  • Psoriasis – Look for erythematous silver-scaled plaques, nail oil-drop changes, and nail pitting. A family history of psoriasis is often noted. Psoriasis and CTCL are histologically different, and a biopsy will aid in the diagnosis.
  • Childhood pityriasis rubra pilaris (PRP) – Look for orange-red, waxy-like keratoderma of the palms and soles. Islands of normal skin within larger plaques are characteristically seen in PRP. PRP and CTCL are histologically different, and a biopsy will aid in the diagnosis.
  • Pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides chronica – While both may display a clonal population of T cells, these are benign disorders.
  • Pityriasis rosea
  • Lichen planus
Tumor stage:
Folliculotropic MF:
Pagetoid reticulosis:
Granulomatous slack skin:

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Updated: 10/05/2016
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Cutaneous T-cell lymphoma in Child
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Cutaneous T-cell lymphoma (Mycosis Fungoides) : Hyperpigmented patch, Lymphadenopathy, Tumor, Pruritus
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