3rd BSDDermatopathology Self Assessment

Case BSD 2015 12

M.Y.Walsh

CLINICAL HISTORY

• 72 year old male

• Recent history of colitis

• Bilateral lower limb weakness

• Chest infection

• Rash over back macular

• Rash on scalp raised and indurated

• Positive serology luetic disease

Histological features biopsy on scalp

• Epidermaltrophism of atypical lymphocytes into the epidermis, hair follicle epithelium and skin appendage epithelium.

• Atypical lymphocytes extend into dermis

•

Histology features biopsy back

• Epidermaltrophism by atypical lymphocytes

• Pautrier’s microabscess

• Lymphocytes large ,atypical convoluted nuclei

• Similar infiltrate in dermis

• Follicular epithelium shows epithelial trophism

• None of the biopsies had large numbers of plasma cells

• Warthin –Starry stains negative

• Immunocytochemistry negative for Treponema pallidum

• Diagnosis at this stage

• Cutaneous T cell lymphoma(mycoses fungoides)

• Immunocytochemistry on all biopsies gave a similar pattern of staining

IMMUNOCYTOCHEMISTRY

• CD20 –ve

• CD79a-ve

• CD3 +ve

• CD4+ve

• CD8++ve

• CD15-ve

• Cd30-ve

Diagnosis on basis of immunocytochemistry

• CD8 predominate T cell lymphoma(CD 8 predominant mycoses fungoides)

Mycoses fungoides

• Usually CD4 > CD8 but 10% CD8>CD4.

• Both types usually have an indolent clinical course

IMMUNOCYTOCHEMISTRY

• CD56 -ve Beta F 1 +ve

• CD43 +ve Mib-1 +++ve

• CD117 -ve TIA 1 -ve

• CD138 -ve

• CD2 +/-ve

• CD5 +ve

• CD7 +ve

Imminocytochemistry

• There is a clinical and histological overlap with gamma delta CTCL.

• Beta F-1 postivity helps to distinguish epidermotropic CD8+ve cytotoxic CTCL which is positive with beta F-1.

• HIV negative

PCR

• PCR showed positive Tcell receptor gene rearrangement

BSD 2015 12

• A. Syphilis

• B. Anaplastic large cell CD30 lymphoma.

• C. Primary cutaneous aggressive epidermotrophic CD8postive cytotoxic Tcelllymphoma

• D. Diffuse large B-cell lymphoma.

Diagnosis

• Primary cutaneous aggressive epidermotropicCD8 +ve cytotoxic cutaneous lymphoma

Prognosis

• Aggressive lymphoma

Clinical course

• Patient developed lymphomatous deposits in the lung,brain and other organs and died a few months after his presentation

TYPICAL CLINICAL FEATURES

• Generalized skin lesions including scaling patches and plaques,papules ,nodules and tumour.

• Oral involvement common

• Dissemination to unusual sites,lung ,brain, testis.

• Lymph nodes may not be involved

Primary cutaneous aggressive epidermotropic CD8+ve cytotoxic

cutaneous Tcell lymphoma

• Is a distinct subgroup

• Prominently epidermotropic infiltrate

• Aggressive clinical behaviour

• Distinct clinical presentation

IMMUNOCYTOCHEMISTRY

• CD20 -ve

• CD79a -ve

• CD3 -ve

• CD4 +ve

• CD8 ++ve

• CD15 -ve

• CD30 -ve