Subcutaneous panniculitis-like T-cell lymphoma with hemophagocytic syndrome: A case report

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.

A Case of Lupus Erythematosus Panniculitis of the Scalp Presenting as Doughnut-shaped Alopecia along Blaschko's lines / 대한피부과학회지

A 9-year-old girl presented with a 2-year history of doughnut-shaped hair loss on the frontal scalp, resembling the symmetric distribution of Blaschko's lines. Physical examination showed an alopecic patch with mild scalp induration. Histopathology revealed lymphoplasmacytic infiltration of the perifollicular dermis and subcutaneous lobules with abundant mucin deposition, consistent with a diagnosis of lupus erythematosus panniculitis. Three cases of linear lupus erythematosus panniculitis of the scalp presenting as alopecia along Blaschko's lines have been documented in Korean dermatologic literature. However, there have been no reports of doughnut-shaped alopecia until now. Herein, we present an interesting case of a pediatric patient with a unique feature of doughnut-shaped alopecia along Blaschko's lines.

Lupus Erythematosus Panniculitis with Xanthomatous Reaction / 대한피부과학회지

Xanthoma may or may not be associated with abnormal serum lipids. Normolipidemic xanthomas can occur in association with underlying lymphoproliferative disease, or they can result from lipid deposition with damaged or altered skin. A 56-year-old woman presented with multiple, yellowish to erythematous papules and plaques on the periocular area and forehead. She had no evidence or history of hyperlipidemia. The biopsy specimen of the forehead showed a diffuse histiocytic infiltrate with abundant bubbly cytoplasm, associated with lobular lymphocytic panniculitis and mucin deposition. Based on the clinical and histopathologic findings, we diagnosed this case as lupus erythematosus panniculitis with xanthomatous reaction. The patient was treated with low dose oral steroids and antimalarials, and her skin lesions were improved after 2 weeks.

Clinical and Histopathologic Studies of Lupus Erythematosus Panniculitis in Korea / 대한피부과학회지

BACKGROUND: Lupus erythematosus panniculitis (LEP), (or lupus erythematosus profundus), is an unusual variant of lupus erythematosus which primarily affects subcutaneous fat. LEP may occur with or without other manifestations of cutaneous or systemic lupus erythematosus. Only a few studies on the clinical and histopathologic manifestation of LEP have been conducted in Korea. OBJECTIVE: The purpose of this study was to investigate clinical, laboratory, and histopathologic features of LEP in Korean patients. METHOD: A total of 30 patients confirmed as having LEP at the Chungbuk National University Hospital between 1 July 1994 and 31 June 2004 were included in this study. The medical records and histopathologic findings were reviewed. RESULTS AND CONCLUSION: 1. LEP occurred predominantly in females, with the female to male ratio being 4: 1. The mean age at diagnosis was 38.5 years. 2. The most common clinical features were nodules or plaques, and 60% of the patients had pain or tenderness. Skin lesions affected the proximal arm area, face, trunk, scalp, and buttocks. In 26% of cases, LEP was associated with discoid lupus erythematosus (DLE), and 13% had evidence of mild systemic lupus erythematosus (SLE). The antinuclear antibody (ANA) was positive in 30% of cases. 3. The main histopathologic characteristics of LEP were predominant lymphocytic lobular panniculitis, hyaline necrosis, mucin deposit, and lymphoid aggregates. 4. Treatments included antimalarial drugs, systemic corticosteroids, intralesional corticosteroids, or topical potent corticosteroids under occlusion. They produced some improvement in most cases, however LEP exhibited a chronic nature with a high relapse rate.

A Case of Lupus Erythematosus Panniculitis with Infiltration of Atypical Lymphocytes / 대한피부과학회지

Lupus erythematosus panniculitis is a rare variant of lupus erythematosus primarily involving a panniculus with or without discoid lupus erythematosus in the overlying skin. A 34-year-old woman showed multiple, variable-sized, indurated subcutaneous nodules on both her upper arms and axillae. A Histopathological examination revealed lobular panniculitis with infiltration of several hyperchromatic atypical large lymphocytes. The infiltrated cells were positively stained with LCA, UCHL-1, CD4, CD8, but not with CD20 and CD68. A T cell receptor -chain gene and immunoglobulin heavy chain gene rearrangement study showed no monoclonality. The result of an ANA test was positive at 1: 40 in a homogeneous pattern. Skin lesions were improved by combined therapy with daily hydroxychloroquine 400mg and prednisolone 10mg in a week and after 6weeks, resolved with atrophic depressed scars. After 4 months, she complained of symptoms of the Raynaud phenomenon and was managed by nifedipine. There has been no recurrence during follow-up for 7 months.