Adult T-cell leukemia/lymphoma with follicular mucinosis: an unusual histopathological finding and a commentary

Follicular mucinosis is currently recognized as a histopathological finding characterized by the accumulation of mucin within follicular epithelium and is commonly associated with follicular mycosis fungoides (MF). We report the finding of follicular mucinosis in a cutaneous nodule of human T-lymphotropic virus type 1 (HTLV-1) associated adult T-cell leukemia/lymphoma (ATLL). The patient was a 69-year-old female of Caribbean descent with a history of ATLL who presented with erythematous nodules on the chest and abdomen. Histopathologic examination showed a pan-dermal infiltrate of medium-to-large sized atypical lymphocytes extending into follicular epithelium where they associated with large mucin deposits. Immunohistochemical stains showed that the atypical lymphocytes were positive for CD3, CD4 and CD25 and negative for CD30. Cutaneous lesions of ATLL, which often present histopathologically as an epidermotropic lymphoma with Pautrier-type collections, are often difficult to distinguish from MF. Until recently, lymphoma-associated follicular mucinosis seemed specific to MF and Sézary syndrome (SS), being reported only once in a lesion of ATLL. We report a second case of ATLL-associated follicular mucinosis to increase awareness of this possible association, and briefly review the literature of follicular mucinosis-associated hematologic malignancies, ultimately cautioning against the interpretation of all cutaneous lymphoma-related follicular mucinosis as MF/SS.     

Primary hyperparathyroidism and cutaneous T-cell lymphoma: fortuitous association?

This is the third report of an association between T-cell cutaneous lymphoma (mycosis fungoides) and primary hyperparathyroidism (adenoma). Some studies support the concept that hyperparathyroidism may have promotional activity for the development of certain malignant tumors. A high risk for successive or concurrent neoplasms has been reported in patients with parathyroid adenomas. Primary hyperparathyroidism in a neoplastic context may be underreported. Patients with tumor-associated hypercalcemia should be evaluated for the possibility of primary hyperparathyroidism.     

Febrile ulceronecrotic Mucha-Habermann disease with clonality: a cutaneous T-cell lymphoma entity?

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a severe variant of pityriasis lichenoides et varioliformis acuta (PLEVA). PLEVA patients only very rarely have systemic signs; the cutaneous lesions are usually asymptomatic, but may be pruritic and may heal with scarring. FUMHD often starts out as classic PLEVA, but goes on to develop widespread ulceronecrotic lesions and is associated with a high mortality rate. Whether Pityriasis lichenoides chronica (PLC) and PLEVA form a spectrum rather than single entities of clonal lymphoproliferative diseases has been discussed. Recently, it has been proposed that FUMHD, too, is a clonal lymphoproliferative disorder. Here, we report two cases of FUMHD with monoclonal T-cell population, as detected by Southern blot analysis. We propose that clonal FUMHD represents a cutaneous T-cell lymphoma entity.     

Guillain-Barré syndrome in a child with systemic lupus erythematosus and anti-Ro/SSA and anti-La/SSB autoantibodies

We report a 9‐year follow‐up of a girl with systemic lupus erythematosus (SLE) and probable Sjögren’s syndrome. At the age of 7 years, the patient developed a chilblain‐like eruption with features of SLE, including leucopenia, oral ulcers, positive rheumatoid and antinuclear antibodies and positive anti‐dsDNA, anti‐Ro/SSA and anti‐La/SSB antibodies. At the age of 13 years she developed Guillain–Barré syndrome, which completely resolved with aggressive treatment, including high‐dose corticosteroids and the use of plasma exchange followed by intravenous gammaglobulin.     

Elevated levels of soluble CD8 antigen in sera of patients with psoriasis — a possible sign of suppressor/cytotoxic T-cell activation

Summary Increased levels of the soluble CD8 antigen probably represent a sign of suppressor T-cell activity. The concentration of CD8 was measured in the sera of patients with psoriasis without psoriatic arthritis (n=25) and atopic dermatitis (n=26), exhibiting moderate to severe disease activity, in comparison with nonatopic healthy controls (n=31) using an ELISA technique. CD8 levels were found to be significantly elevated in psoriatic patients as compared with healthy controls (p0.002). Increase in CD8 may be due to suppressor T-cell activation in the skin of patients with psoriasis.Presented at the 1st ESDR-JSID-SID Tricontinental Meeting, 26–30 April 1989, Washington, D.C.     

Epstein-Barr virus-associated primary central nervous system lymphoma in a patient with adult T-cell leukemia / lymphoma

We present a case of Epstein-Barr virus (EBV)-associated primary central nervous system lymphoma (PCNSL) arising from a patient with cutaneous-type adult T-cell leukemia/lymphoma (ATLL). Extranodal sites affected by ATLL include the skin, lung, liver, gastrointestinal tract and central nervous system (CNS). CNS involvement usually occurs as an acute and lymphoma-type ATLL. PCNSL is a rare type of tumor and the vast majority of PCNSL are of B-cell lineage. Individuals with acquired, iatrogenic or congenital immunodeficiency are at increased risk of PCNSL, which is commonly associated with EBV. In our patient, the expression of latent infection membrane protein 1 (LMP1), EBV nuclear antigen 2 (EBNA2), and EBV-encoded small RNA (EBER) in tumor cells confirmed a type III latency of EBV infection. Human T-cell lymphotropic virus type I (HTLV-I) can induce immunodeficiency before the overt development of ATLL. The HTLV-I infection led to suppression of the immune system and the development of EBV-associated PCNSL. This is the first reported case of the clinicopathological features of EBV-associated PCNSL arising from a patient with ATLL.     

Erythroderma and non-Hodgkin T-cell lymphoma: what else,apart from Mycosis Fungoides and Sézary syndrome?

Background

Peripheral T-cell lymphomas, not otherwise specified (PTCL-NOS), are a rare condition characterised by specific histology, nodal presentation, and a poor prognosis. In total, 10-18% of patients present with cutaneous involvement which is regarded as a poor prognostic marker. However, cutaneous PTCL-NOS lesions have been rarely reported in the literature.

Objectives

We sought to describe PTCLNOS cases characterised by erythrodermic dissemination to the skin.

Materials & methods

Three cases of PTCL-NOS were investigated; all male, with a mean and median age of 55 and 51 years, respectively.

Results

All patients underwent aggressive chemotherapeutic protocols with only transient improvement of the disease, and died within two years of follow-up.

Conclusions

Dermatologists should be reminded that erythroderma and lymph node enlargement do not represent an exclusive paradigm for erythrodermic cutaneous T-cell lymphoma, and that these features can be due to a systemic lymphoma that should be considered in the differential diagnosis.

     

Novel treatment of Sézary-like syndrome due to adult T-cell leukaemia/lymphoma with daclizumab (humanized anti-interleukin-2 receptor alpha antibody)

We describe a patient with erythrodermic adult T-cell leukaemia/lymphoma resistant to multiple systemic therapies who, on the commencement of daclizumab, a humanized anti-interleukin-2 receptor antibody, developed a rapid and sustained complete response with resolution of previously debilitating erythroderma, suggesting significant activity of this agent in this disease process.     

Expression of T-cell receptor-γδ in normal human skin, inflammatory dermatoses and mycosis fungoides

Background: T cells expressing the γδ T‐cell receptor (TCR) (γδ T cells) are found in normal epithelial tissues such as the skin. However, the proportions of γδ T cells that may be observed in commonly encountered cutaneous diseases with a prominent lymphocytic infiltrate have not been elucidated. Methods: Our pathology database was searched for cases of mycosis fungoides, erythema multiforme, graft‐versus‐host disease, lichen planus, lupus panniculitis and spongiotic dermatitis. Immunostaining for CD3, βF1 and the TCR γ chain was performed on formalin‐fixed paraffin‐embedded specimens retrieved from these cases to determine the normal range of γδ T cells in these diseases. Results: In 100 of the 101 cases studied (99.0%), γδ T cells accounted for less than 10% of the T‐cell infiltrate. Furthermore, γδ T cells were essentially absent in 74 cases (73.3%). Conclusions: These results suggest that γδ T cells very rarely account for more than 10% of the lymphocytic infiltrate in common inflammatory or infiltrative processes of the skin. Any case of suspected mycosis fungoides or interface dermatitis that possesses more than 10% γδ T cells should raise consideration for further investigation. Hocker TL, Wada DA, el‐Azhary R, Gibson LE. Expression of T‐cell receptor‐γδ in normal human skin, dermatitis and mycosis fungoides.     

Onychomycosis with onychodystrophy or acrolentiginous melanoma with onychomycosis and onychodystrophy?

A 68-year-old female patient had been treated locally and systemically for onychomycosis of the left thumbnail for 1 year. During the course of treatment there was increasing destruction of approximately 50% of the nail without Hutchinson's sign. Dermoscopically there were yellow to brown vertical stripes of varying width in the remaining parts of the nail. In the visible nail matrix reddish, brownish and grey-black colored components with varying differential structures could be detected. An acrolentiginous melanoma with a diameter of 1.04?mm could be identified histologically and the associated onychomycosis was confirmed by culturing.