Clinical characteristics, differential diagnosis, and treatment outcome of subcutaneous panniculitis-like T-cell lymphoma: a literature review of published Japanese cases.

BACKGROUND: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of non-Hodgkin lymphoma that shows phenotypic features of cytotoxic T cells and preferentially involves subcutaneous tissue. SPTCL is believed to show an indolent clinical course, unless patients develop haemophagocytic syndrome. Various reported therapies include corticosteroids, immunosuppressive drugs, and chemotherapies. The use of chemotherapy as a first-line treatment remains controversial, and treatment approaches for SPTCL have not been established yet. OBJECTIVES: To investigate the clinicopathological features, treatment modalities, and outcomes of Japanese patients with SPTCL. MATERIALS & METHODS: We performed a literature review of Japanese cases of SPTCL. RESULTS: Twenty-two cases have been reported in the English and Japanese literature. Six cases were excluded due to a lack of sufficient clinical and immunohistological data, and treatment modalities and outcome were available in 16 cases. Clinical characteristics of the Japanese cases were generally similar to those of Western countries. Approximately half of the Japanese patients were treated initially with corticosteroids. Among them, more than half subsequently received chemotherapies due to a lack of response or recurrence of the disease. Overall, chemotherapy was used for approximately 80% of reported cases as a primary or secondary therapy. CONCLUSION: Our findings suggest that corticosteroids may be beneficial for some SPTCL patients, but most patients required chemotherapy during the course of their disease. Further investigations are needed to both establish appropriate treatment strategies for SPTCL and clarify predictive factors in order to identify patients who may benefit from corticosteroid therapy as a primary treatment.

Ichthyosiform sarcoidosis: report of three cases from Japan and literature review.

Cutaneous lesions of sarcoidosis present with various manifestations including specific and non-specific cutaneous lesions. Ichthyosiform sarcoidosis is a rare form of cutaneous sarcoidosis, presenting with asymptomatic, adherent, polygonal scales, mainly appearing on the lower limbs.  Ichthyosiform sarcoidosis has a predilection for dark-skinned races, and cases affecting Japanese patients have rarely been reported in English literature.  We herein describe three Japanese cases of ichthyosiform sarcoidosis on the lower limbs. All of the patients were female, with an age range of 57-69 years old.  Histologically, sarcoidal granulomas were located in the mid- to lower dermis.  All cases had scar sarcoidosis on the knees.  Furthermore, Case 1 presented with papular sarcoidosis on the back, and Case 3 presented with subcutaneous nodules on the buttock as well as erythema nodosum-like lesions on the lower legs.  All patients had lung sarcoidosis, but ocular sarcoidosis was seen in only Case 2. Case 3 showed Heerfordt syndrome with facial nerve paralysis. Histological features showed that the granular layers were scarcely detected in the overlying epidermis; however, filaggrin expression was not decreased.  Sarcoidal granulomas accumulated around the sweat glands in one case, whereas those features were not detected in the other two cases. In conclusion, ichthyosiform cutaneous sarcoidosis may be overlooked or misdiagnosed as xerotic dry skin which is frequently found in elderly people, and ichthyosiform cutaneous lesions may be more prevalent than previously estimated.

Perforating pilomatricoma with anetodermic epidermis in an adolescent with lymphoma.

Pilomatricoma is a benign neoplasm originating from primitive cells of the hair matrix. Occasionally the skin overlying pilomatricomas assumes peculiar clinical features, and anetodermic change of the skin overlying pilomatricomas is sometimes seen, but perforation in pilomatricoma is a rare event. We report a case of perforating pilomatricoma with anetoderma in an adolescent with lymphoma.

Eruptive molluscum contagiosums in a patient with rheumatoid arthritis and lung cancer.

A 67-year-old woman with rheumatoid arthritis (RA) treated with systemic prednisolone and methotrexate over 20 years developed eruptive molluscum contagiosums on the trunk and extremities. Investigation revealed lung cancer 2 years later. Newly development of molluscum contagiosums ceased after the surgical operation of lung cancer. Immunologic dysfunctions have been shown in RA, and especially patients under long-term methotrexate therapy are susceptible to miscellaneous skin conditions. Eruptive molluscum contagiosums are induced in association with hematologic malignancies such as lymphoma, leukemia, and HIV infection; however, it is important to investigate internal malignancies, not only hematologic malignancies but also solid cancers, when patients with RA under immunosuppressive therapies presented eruptive or disseminated molluscum contagiosums.