An Elderly Male With Anemia and Thrombocytopenia

Cutaneous Lymphoma: A Young Woman With Scaly Patches and Disseminated Erythema

Amaya Llorente-Chávez, MD,¹ Fabiola Cassiano-Quezada, MD,²Daniel Montante-Montes de Oca, MD,³ and Gladys Patricia Agreda-Vásquez, MD¹

1. Hematology and Oncology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, México City, México

2. Internal Medicine Service, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirá, México City, México

3. Pathology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, México City, México

Published on: July 1, 2025

A 26-year-old woman developed progressive pruritic erythema and patches that became persistent and worsened over the years. Recurrent Staphylococcus aureus infections appeared due to disruption of the skin barrier, and antibiotics were administered. Physical examination revealed indurated erythema and scaly patches with diffuse lichenification, plaques, and tumors affecting 40% of her body surface (Figure 1). Some skin lesions had an infiltrating appearance, with irregular borders and atrophy in a “bathing trunk” distribution with variation in size and shape. An extensive workup was performed, with active infection ruled out. The blood immunophenotype showed 632 total lymphocytes with 25% CD4+/CD26-. Skin biopsy (Figure 2) immunohistochemistry showed CD3+, CD4+, CD5+, CD2-, CD7-, CD8-, and CD30-. A positron emission tomography/computed tomography scan found hypermetabolism of regional axillary and inguinal lymph nodes without extra nodal involvement. The axillary node biopsy found a mature T-cell phenotype (CD3+, CD4+, CD5+, CD7-, CD8-, and CD30-).

Figure 1. Physical skin examination

Lateral and frontal view of disseminated erythema and scaly plaques with an infiltrating appearance, atrophy, and variation in size and shape (A, B). Scaly plaque with irregular borders (C).

Figure 2. Skin biopsy

Photomicrograph of skin excision showing mild spongiotic and psoriasiform pattern with hematoxylin and eosin stain, 2x (A). Epidermotropism by atypical lymphocytes of cerebriform nuclei and Pautrier-type microabscesses with hematoxylin and eosin stain, 40x (B).

The authors indicated no relevant conflicts of interest.

References

Hristov AC, Tejasvi T, Wilcox RA. Mycosis fungoides and Sézary syndrome: 2019 update on diagnosis, risk-stratification, and management. Am J Hematol. 2019;94(9):1027-1041.
Larocca C, Kupper T. Mycosis fungoides and Sézary syndrome: an update. Hematol Oncol Clin North Am. 2019;33(1):103-120.
Scarisbrick JJ, Prince HM, Vermeer MH, et al. Cutaneous Lymphoma International Consortium study of outcome in advanced stages of mycosis fungoides and Sézary syndrome: effect of specific prognostic markers on survival and development of a prognostic model. J Clin Oncol. 2015;33(32):3766-3773.
Magro CM, Momtahen S, Kiuru M. Primary cutaneous small cell variant of anaplastic large cell lymphoma: a case series and review of the literature. Am J Dermatopathol. 2017;39(12):877-889.
Broccoli A, Zinzani PL. Peripheral T-cell lymphoma, not otherwise specified. Blood. 2017;129(9):1103-1112.

What is the diagnosis?

Correct!

Mycosis fungoides is a mature T-cell, non-Hodgkin lymphoma that predominantly affects the skin, lymph nodes, blood, and viscera. Intraepidermal lymphocytes with spongiosis or epidermotropism and Pautrier microabscesses along with cerebriform cytomorphology are characteristic. Positive stains for mature T-cell phenotype include CD45RO, CD2, CD3, CD4, CD5, and CD7. Clonality is expressed by the T-cell receptor (TCR) beta chain gene. TNMB (tumor, node, metastasis, blood) classification for this patient was stage IVA2: tumor 2b (skin involvement of patches and plaques); node 3 (clinical abnormal lymph nodes and Dutch grade 3); metastasis 0 (no visceral organ involvement); and blood 0 (<250 mcL of CD4+/CD26- or CD4+/CD7- cells). At this stage, the five-year survival is 32%, with disease-specific survival of 44%.^1-3

PC-ALCL is characterized by skin infiltration of anaplastic and pleomorphic T cells positive for CD30. Epidermotropism can be found, but cerebriform lymphocytes are not identified. The immunohistochemistry shows positive stains for CD30, CD4, CD45, granzyme B, TIA1, and perforin. It is negative for CD2, CD3, CD5, CD7, ALK, and CD56. Most cases present as a localized lesion such as a nodule, and 20% of cases have a diffuse distribution. The minority of cases have nodal dissemination.⁴

Sézary syndrome presents with erythroderma (involvement of more than 80% of the body surface), lymphadenopathy, and a Sézary cell count of more than 1,000 cells/mL or loss of one or more T-cell antigens on flow cytometry and T-cell clone (>40% of CD4+/CD7- and >30% of CD4+/CD26-). Skin biopsy can show limited to perivascular lymphocytic and eosinophilic dermatitis with spongiosis. Epidermotropism and Pautrier microabscesses are not common. Sézary cells are atypical lymphocytes with a cerebriform nuclei that can be found infiltrating lymph nodes or in peripheral blood. Immunohistochemistry highlights the absence of conventional T-cell markers, but loss of CD7 or CD26 is highly sensitive and specific for diagnosis.^1-2

PTCL-NOS commonly presents with lymphadenopathy and extranodal disease in most cases. Lymph node biopsy reveals a paracortical or diffuse infiltrate by Reed-Sternberg-like cells. Skin biopsy shows nodules from a lymphocyte infiltration of the dermis and subcutis with central necrosis and epidermotropism. There is a variable expression of T-cell markers CD2, CD3, CD5, and CD7, as well as variable expression of CD30. Positive TIA1, CD56, granzyme B, and perforin can also be found. Clonal rearrangement for the TCR gene is necessary.⁵

Choice A (Primary cutaneous anaplastic large cell lymphoma (PC-ALCL)) is incorrect.

Choice B (Sézary syndrome) is incorrect.

Choice D (Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS)) is incorrect.

Cutaneous Lymphoma: A Young Woman With Scaly Patches and Disseminated Erythema

What is the diagnosis?

Correct!

ASH Publications

American Society of Hematology

Cutaneous Lymphoma: A Young Woman With Scaly Patches and Disseminated Erythema

What is the diagnosis?

Correct!

ASH Publications

American Society of Hematology

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