New Orleans — For a variety of reasons, cutaneous T-cell lymphoma (CTCL) — a low-grade lymphoma that typically presents in or on the skin
— can be difficult to diagnose.
CTCL should not be confused with peripheral T-cell lymphomas (PTL) or adult T-cell lymphoma/leukemia (ATLL), which are more
aggressive forms of lymphoma that may also present initially in or on the skin but require different types of treatment. Thus,
accurate diagnosis is crucial.
According to Jacqueline M. Junkins-Hopkins, M.D., CTCL features overlap with more inflammatory dermatoses, making them difficult
to differentiate from the pseudolymphoma and, as a result, often making accurate diagnosis problematic. In addition, she says,
histopathology of the early disease is not always diagnosable, so that CTCL and its variants may escape diagnosis entirely.
Dr. Junkins-Hopkins, assistant professor, University of Pennsylvania Health System's department of dermatology, provided insight
on the topic at the 63rd Annual Meeting of the American Academy of Dermatology here.
Types and traits Dr. Junkins-Hopkins notes that CTCL presents itself as two main types: classic mycosis fungoides (MF) and non-mycosis fungoides
or non-epidermotropic CTCL. She also says there are a multitude of MF variants, among which are:
- pagetoid reticulosis
- folliculotropic MF, with or without mucinosis
- granulomatous slack skin
- hypopigmented
- syringotropic/syringolymphoid hyperplasia
- palmar plantar
- bullous
Further, she identifies four variants of erythrodermic T-cell lymphoma — Sezary syndrome (an advanced form of MF), erythrodermic
MF, ATLL, and HIV-related HTLV II — and notes what she refers to as "clonal disorders":
- large and small plaque parapsoriasis
- lymphomatoid papulosis
- acute and chronic pityriasis lichenoides
- clonal cutaneous lymphoid hyperplasia
- clonal dermatitis
As to the clinical approach to CTCL infiltrates, Dr. Junkins-Hopkins says it should focus on history (duration, site, morphology
and drugs, as well as course/treatment response); possible previous biopsies; and new biopsies.
"Often, more than one biopsy is necessary. They should be repeated if the histology is non-specific, and the deep-sampling
variety is helpful," she says. "Adjunctive tests also are often needed."
Dr. Junkins-Hopkins adds that in the case of classic patch/plaque CTCL, or MF, if the clinical presentation points to MF,
non-diagnostic histology is acceptable. In any case, she stresses, "Communication between clinician and pathologist is critical."
Typical histology of CTCL-MF, Dr. Junkins-Hopkins notes, includes the following features:
- irregular proliferation of rete or atrophy
- epidermotropism ("beading" of lymphocytes along the basal keratinocytes and/or "stuffing" of dermal papilla by collections
of atypical lymphocytes)
- Pautrier's microabcess
- absence of epidermal alteration (spongiosis, lichenoid tissue reaction)
- cerebriform lymphocytes with perinuclear halos pepper epidermis with minimal spongiosis
- papillary dermal fibroplasias
- lymphocytes patchy to band-like, initially sparing vascular plexus below papillary dermis
Patch/plaque MF histologic simulators include:
- spongiatic dermatosis (allergic contact dermatitis, eczema)
- pigmented purpora/lichen striatus
- lichenoid keratosis
- lichenoid dermatosis
- drug-induced cutaneous lymphoid hyperplasia
- lichen sclerosis
- normal skin/no pathology/non-specific chronic inflammation (especially if partially treated)
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