Journal of the Philippine Medical Association 2021;100(1):58-65
Subcutaneous panniculitis-like T-cell lymphoma with hemophagocytic syndrome: A case report.
Ranillo V. Gamutan 1
Affiliations
Keywords
Subcutaneous panniculitis-like T-cell lymphoma; lupus erythematosus panniculitis
Country
Philippines
Language
English
Abstract
INTRODUCTION:Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare disease, accounting for less than 1 % of non-Hodgkin's lymphomas that is
characterized by infiltration of T-cells in the
subcutaneous adipose tissue.
CASE PRESENTATION:A 21-year-old Filipino female
presented with intermittent fever which eventually
was associated with multiple eruption of cutaneous
lesions. The serum antinuclear antibody (ANA) titer
and pattern revealed negative results. Further
clinical investigation prompted a skin lesion punch
biopsy revealing atypical lymphoid infiltrates with
lobular panniculitis consistent with SPTCL.
lmmunohistochemical studies stained strongly
posItIve for CD3, CDS, granzyme B and negative
for CD20, CD4, and CD56. The case was also
compounded with the existence of hemophagocytic
syndrome having fulfilled five of the eight criteria .
She was given prednisone (1 mg/kg/day) with
gradual resolution of cutaneous lesions leaving
marked hyperpigmentation and lipoatrophy.
DISCUSSION:SPTCL presents with a myriad of
systemic symptoms but will always present with
skin lesions. The biopsy for histopathological
interpretation is commonly performed as a first
diagnostic step followed by immunohistochemical
staining. Clinical presentation, histo-pathological
findings, and 1mmunohistochemical results together
clinched the diagnosis of SPTCL.
CONCLUSION:Patients with history of intermittent fever associated with cutaneous lesions and systemic clinical findings, SPTCL should be a differential diagnosis. However, lupus erythematosus panniculitis (LEP) must be ruled out first as its closest mimicker. This case report along with other large case series provides further evidence that monotherapy with oral prednisone can be an initial choice of therapy.
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