Ann Dermatol.  2016 Oct;28(5):540-547. 10.5021/ad.2016.28.5.540.

Pityriasis Lichenoides-like Mycosis Fungoides: Clinical and Histologic Features and Response to Phototherapy

• Jang MS
• Kang DY
• Park JB
• Kim JH
• Park KA
• Rim H
• Suh KS

• KMID: 2382876
• DOI: http://doi.org/10.5021/ad.2016.28.5.540

Abstract

BACKGROUND
Pityriasis lichenoides (PL)-like skin lesions rarely appear as a specific manifestation of mycosis fungoides (MF).
OBJECTIVE
We investigated the clinicopathological features, immunophenotypes, and treatments of PL-like MF.
METHODS
This study included 15 patients with PL-like lesions selected from a population of 316 patients diagnosed with MF at one institution.
RESULTS
The patients were between 4 and 59 years of age. Four patients were older than 20 years of age. All of the patients had early-stage MF. In all patients, the atypical lymphocytic infiltrate had a perivascular distribution with epidermotropism. The CD4/CD8 ratio was <1 in 12 patients. Thirteen patients were treated with either narrowband ultraviolet B (NBUVB) or psoralen+ultraviolet A (PUVA), and all of them had complete responses.
CONCLUSION
PL-like MF appears to have a favorable prognosis and occurrence of this variant in adults is uncommon. MF should be suspected in the case of a PL-like skin eruption. Therefore, biopsy is required to confirm the diagnosis of PL-like MF, and NBUVB is a clinically effective treatment.

Keyword

Mycosis fungoides; Phototherapy; Pityriasis lichenoides

MeSH Terms

Adult
Biopsy
Diagnosis
Humans
Mycosis Fungoides*
Phototherapy*
Pityriasis Lichenoides
Pityriasis*
Prognosis
Skin

Figure

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  Fig. 1 (A) Generalized erythematous scaly discrete papules on the whole body (patient 1). (B) Brownish to erythematous crustered scaly papules (patient 6).

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  Fig. 2 (A) Biopsy specimens showing psoriasiform lichenoid infiltration (H&E, ×40). (B) Upper epidermal necrosis (arrow) and dermal papillae stuffed with lymphocytes (H&E, ×100). (C) Epidermotropism, "haloed" lymphocytes, and basal vacuolar change (H&E, ×200). (D) Intraepidermal lymphocytes that are larger than those in the dermis, hyperchromatic atypical cells (arrows), and Pautrier's microabscess (arrowhead) (H&E, ×400).

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  Fig. 3 Some atypical lymphocytes with positive immunohistochemical staining of (A) CD3, (B) CD4, (C) CD8, and negative staining of (D) CD30. CD8-positive T cells are predominant than CD4-positive T cells in the dermis, and CD4-positive T cells are predominant than CD8-positive T cells in the epidermis (×100) (patient 7).

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