A case of classic Kaposi's sarcoma with multiple organ involvement

Kaposi's sarcoma is a systemic, multifocal, angiomatous tumor which can be classified into four clinical variants. Among them, classic Kaposi's sarcoma is rare in Oriental people, and the involvement of internal organs in this variant is reported to be very rare. We present a case of classic Kaposi's sarcoma in a 67-year-old Korean male, who had multiple organ involvement in the skin, lymph node, and stomach. Lesions of Kaposi's sarcoma cleared after treatment with etoposide, but the skin lesions recurred after a three-month symptom-free period. He was treated with doxorubicin, bleomycin, and vincristine, and all the skin lesions were cleared.     

Primary cutaneous anaplastic large cell lymphoma with subsequent leg involvement

Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is regarded as an indolent type of cutaneous T-cell lymphoma. However, a few recent publications revealed that C-ALCL patients with initial leg involvement had significantly worse survival than those without initial leg involvement. Herein, we report a case of C-ALCL with subsequent leg involvement, which led to death after chemoradiation therapy. A 75 years old Japanese man presented with multiple erythematous nodules in his left arm and the side of his left chest. Histopathological and immunohistochemical studies led to the diagnosis of primary C-ALCL. At the initial diagnosis, no leg lesion was found. One year after the initial diagnosis, C-ALCL appeared in his right lower thigh and left hip. Radiation therapy, low-dose etoposide and CHOP therapy were performed; however, the patient died of malignant lymphoma 4 years after the initial diagnosis. We speculated that the occurrence of subsequent leg involvement may also be indicative of a worse prognosis, as in the case with initial leg involvement in C-ALCL. Therefore, we propose that C-ALCL patients with initial or subsequent leg involvement should be classified as a distinct clinicopathological variant of C-ALCL (“leg-type” involvement) and that they may require intense therapy.     

Late lethal hepatitis B virus reactivation after rituximab treatment of low-grade cutaneous B-cell lymphoma

The chimeric anti-CD20 monoclonal antibody, rituximab, is a promising treatment for cutaneous B-cell lymphomas. Classically used in combination with a multiagent-chemotherapy regimen, it can sometimes give excellent results alone. Because of its selective action on B lymphocytes, it is considered a moderate immunosuppressant in terms of infection. We describe a woman with relapsed cutaneous follicular centre B-cell lymphoma and secondary lymph-node involvement treated with rituximab alone, which induced a complete remission. One year later, she experienced a fatal hepatitis B virus (HBV) reactivation. Several such HBV reactivations were reported after combined rituximab and multiagent chemotherapy for B-cell lymphomas. This is the first case of HBV reactivation occurring during the year following rituximab monotherapy in the absence of any other immunosuppressive factor.     

CD30+ cutaneous lymphoproliferative disorders: the Stanford experience in lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma

BACKGROUND: CD30+ cutaneous lymphoproliferative disorders (CLPDs) include lymphomatoid papulosis, borderline cases of CD30+CLPDs, and primary cutaneous anaplastic large cell lymphoma (PCALCL). Prior studies have shown CD30+CLPDs have an excellent prognosis. OBJECTIVE: We sought to present the single-center experience of Stanford University, Stanford, Calif, in the management of CD30+CLPDs. METHODS: A retrospective cohort analysis of 56 patients with CD30+CLPDs treated at our institution was performed. RESULTS: No patients with lymphomatoid papulosis died of disease, and overall survival was 92% at 5 and 10 years. Disease-specific survivals at 5 and 10 years for PCALCL were 85%. Disease-specific survival at 5 years for localized versus generalized PCALCL was 91% versus 50% (P =.31). PCALCL was highly responsive to treatment, but the relapse rate was 42%. In all, 3 patients progressed to extracutaneous stage of disease. No clinical or histologic factors analyzed were predictive of worse outcome in lymphomatoid papulosis and PCALCL. CONCLUSION: Similar to prior reports from multicenter European groups, the single-center experience at our institution demonstrates CD30+CLPDs have an overall excellent prognosis; however, cases of PCALCL with poor outcome do exist.     

Primary cutaneous anaplastic large‐cell lymphoma: a case report

Primary cutaneous anaplastic large‐cell lymphoma (PCALCL) is a part of the spectrum of CD30+ lymphoproliferative cutaneous processes. The characteristics include single or multifocal nodules that ulcerate as skin lesion, slow disease progression, autoregressive, and recurrent in few years. The present study report the case of a 16‐year‐old boy presenting PCALCL with single nodules, ulcer, keloid, and scab in his right‐side face. He showed a good response to the treatment with systemic chemotherapy and dermatoplasty, and regained confidence after the appearance of recovery. There is no relapse of the primary lesion and organs involved till now. The chemotherapy combining with surgical excision and dermatoplasty is a good method for PCALCL, per the lesion biopsy and positron emission tomography‐computed tomography before and after treatment.     

Bullous and haemorrhagic lichen sclerosus with scalp involvement

We describe a patient who developed a generalized blistering eruption due to lichen sclerosus and who was observed to have scalp involvement. Both are unusual manifestations of this disease which merit consideration. Lichen sclerosus is an uncommon disease that most frequently affects the external genitalia of perimenopausal women. The aetiology is unknown. Approximately 20% of affected patients have extragenital lesions that present as small, ivory, shiny round macules or papules that later become atrophic; extragenital lesions are generally asymptomatic. Bullous and haemorrhagic forms may occur but these are generally localized and reports of extensive or generalized involvement are rare. We describe an elderly woman with generalized bullous lichen sclerosus. As an incidental finding, she was observed to have lichen sclerosus affecting her scalp. This has rarely been described and it would appear that she is the third reported case of scalp involvement.     

Induction of durable responses by oral etoposide monochemotherapy in patients with metastatic Merkel cell carcinoma

Merkel cell carcinoma (MCC) is an aggressive, rare tumour of the skin. For advanced cases with distant organ metastases several different regimens of chemotherapeutics have been described. Disease specific 5-year survival rates for these patients are approximately 11%. In this case series we report our experience with orally administered etoposide (100?mg at days 1 to 10 in a 31 day cycle) in 4 patients. We treated two male and two female patients with a median age of 68.5 years. In our four treated patients the disease control rate (complete remission, partial remission, stable disease) was 100%. Three out of four patients reached complete remission. Promisingly, two of our patients had long lasting, durable responses which, until now, have lasted for 16 and 36 months, respectively. The mean follow up time after start of therapy was 14.25 months (range 1-36 months). Etoposide treatment was generally well tolerated, the most common side effect was neutropenia, in one case CTC grade 3. In conclusion, orally administered etoposide in metastatic Merkel cell carcinoma was highly effective and well tolerated.     

Treatment of recalcitrant psoriatic arthritis with anti-tumor necrosis factor-α antibody

Currently available treatments for psoriatic arthritis are either not completely effective or toxic in some patients. As tumour necrosis factor (TNF)-alpha is involved in both the joint and skin involvement in psoriatic arthritis, blockade of TNF-alpha seems a reliable way to treat patients with this disease. We report two patients with progressive recalcitrant psoriatic arthritis treated with low-dose methotrexate (7.5 mg, once per week) in combination with intravenous chimeric monoclonal anti-TNF-alpha antibody (infliximab, 3 mg/kg body weight). Both showed a dramatic and rapid response in the reduction of pain, followed by improvement of laboratory and clinical signs of joint inflammation. Skin disease also responds after a short delay. The observation shows that infliximab is effective and well tolerated in patients with recalcitrant progressive psoriatic arthritis. Different kinetics of symptom release during treatment suggest a variable role for TNF-alpha in disease pathways of pain, joint inflammation and skin involvement.     

Cutaneous gamma‐delta T‐cell lymphoma with central nervous system involvement: report of a rarity with review of literature

Primary cutaneous gamma‐delta (γδ) T‐cell lymphoma is an extremely rare and aggressive variant of cutaneous lymphoma. Central nervous system (CNS) involvement, a rare finding, and hemophagocytic syndrome are two complications that are commonly fatal. We describe a 58‐year‐old patient presenting with skin plaque who subsequently developed subcutaneous nodules diagnosed as cutaneous T‐cell lymphoma (CTCL), clinically resembling ‘mycosis fungoides’. The patient was treated with repeat topical radiation therapies but had frequent relapsed disease. Approximately 4.5 years after, the patient presented with third and sixth cranial nerve palsies and was found to have CNS involvement by lymphoma per positron emission tomography—computed tomography (PET/CT) and a biopsy of foramen magnum. Phenotypically, the tumor cells were CD3(+)/CD4(?)/CD8(?)/CD7(+)/CD5(?)/CD30(?)/TCRαβ(?)/TCRγδ(+). Despite aggressive strategies taken, the patient expired 3 months after the diagnosis of the CNS lesion. A retrospective investigation proved the original CTCL to be γδ T‐cell in origin, confirming an indolent cutaneous γδ T‐cell lymphoma with eventual CNS manifestation. We present this case to draw attention to the entity, which can occasionally present with misleading histopathologic and clinical features. In addition, we provide a review of the literature to summarize clinical and pathologic features of the reported similar cases.     

Blastic natural killer cell leukaemia with skin involvement: a case report

Natural killer cell leukaemia is generally accompanied by extramedullary involvement. CD4+ natural killer cell leukaemia frequently manifests with cutaneous involvement. The disease pursues a very aggressive course with no long-term survivors reported. We present a patient with CD4+ natural killer cell leukaemia with skin, bone marrow and peripheral blood involvement who is still on remission at the end of 2 years.     

Cutaneous sarcoidosis with cardiac involvement.

Despite the clinical importance of cardiac involvement in the prognosis of sarcoidosis, it is often overlooked because of the subclinical disease progression and difficulty in diagnosis. We report here five patients with cutaneous sarcoidosis lesions where cardiac involvement was detected with the appearance of mild cardiac symptoms on a careful examination of the heart after cutaneous sarcoidosis was diagnosed. In four of the five cases, the patients had annular lesions while the fifth case showed a nodular eruption on the face. Three of the five patients showed complete AV block while one showed complete right bundle block and left bundle anterior branch block. All four patients with conduction disturbances underwent a permanent pacemaker implantation with prednisolone tapering therapy. The remaining patient had congestive heart failure and was treated with prednisolone alone. The present findings support the belief that sarcoidosis patients with cutaneous lesions, especially facial annular lesions, should be carefully examined and monitored for cardiac involvement, even in cases without apparent cardiac symptoms.     

Suspected cardiac toxicity to intravenous immunoglobulin used for treatment of scleromyxedema

Scleromyxedema is a rare, generalized form of lichen myxedematosus, which may be associated with systemic involvement and can be fatal. The therapeutic options available provide partial or inconsistent response and are associated with significant adverse effects. We report a case of scleromyxedema with cardiac involvement, treated with low-dose intravenous immunoglobulin, with almost complete clearing of the skin lesions. The patient died after three cycles of treatment, possibly due to myocardial infarction.     

Management of multifocal primary cutaneous CD30 anaplastic large cell lymphoma

Primary cutaneous CD30(+) anaplastic large cell lymphoma in adults is rare, but the prognosis is generally excellent. Skin lesions may be localized or, less commonly, multifocal. Although not extensively reported, multifocal primary cutaneous anaplastic large cell lymphoma tends to relapse after systemic chemotherapy and is generally considered more prone to progress to extracutaneous involvement than the localized disease. We report the case of a 21-year-old woman with primary cutaneous CD30(+) anaplastic large cell lymphoma manifesting as widespread papules and nodules. Despite remaining localized to the skin, the disease relapsed after multiple chemotherapy regimens and autologous stem-cell transplantation. Treatment with an experimental anti-CD30 monoclonal antibody was successful. Review of this case and similar cases illustrates that traditional combination chemotherapy may not be best. Newer treatments, including anti-CD30 monoclonal antibodies, show promise. However, further study is needed to develop optimal therapeutic strategies.     

Necrobiotic xanthogranuloma with cardiac involvement

A patient with necrobiotic xanthogranuloma (NXG) and paraproteinaemia, who was followed-up for several years, and treated with low-dose chlorambucil, died as a result of a respiratory illness. The significant findings at autopsy were a xanthogranuloma of the spleen and giant cell myocarditis. The myocardial lesions were composed of macrophages, giant cells and lymphocytes. This finding is important because four of five known autopsied patients with NXG have had giant cell myocardial disease, and an effort at antemortem diagnosis should be made.     

Xanthoma disseminatum with hepatic involvement in a child

Xanthoma disseminatum is a rare, usually self-healing dermatologic disease of unknown etiology. Involvement of other organs and tissues including bone marrow, bone, and brain may be seen rarely in children. However, to date, hepatic involvement has not been reported. We describe a child with xanthoma disseminatum who had hepatic involvement, and discuss his course and treatment with steroid and azathioprine.     

Post-kala-azar dermal leishmaniasis with mucosal involvement in a kidney transplant recipient: treatment with liposomal amphotericin B

Post-kala-azar dermal leishmaniasis (PKDL) is a rare clinical variant of cutaneous leishmaniasis. It is very common in the Indian subcontinent and less frequent in East Africa, but exceptional in the American and European continents. We have observed a case of PKDL in a renal transplant recipient. No systemic symptoms were present. The patient was treated with liposomal amphotericin B. We emphasize the unusual aspects of this case: the appearance of PKDL in Europe, its relationship with immunosuppression, the severe mucosal involvement and the excellent response to liposomal amphotericin B, a newly described treatment for the disease.     

Treatment of adult Langerhans cell histiocytosis with etoposide.

BACKGROUND: Langerhans cell histiocytosis (LCH) is a reactive disease in which abnormal Langerhans cells accumulate in various body sites and cause damage to affected organs. Adults often do not respond to conventional therapy with local or systemic steroids. OBJECTIVE: Our purpose was to investigate intravenous etoposide as monotherapy in the treatment of adult patients with severe or resistant LCH. METHODS: In an open study, three adult patients with LCH (one with single-system skin disease and two with multisystem disease) were treated with etoposide, 100 mg/m2/day, for 3 days. This was repeated every 3 or 4 weeks for three or four cycles. RESULTS: All patients achieved clinical remission that persisted during a 12- to 14-month follow-up. No serious side effects were noted. CONCLUSION: We recommend the use of etoposide monotherapy in severe or restricted LCH in adults.     

Intralesional rituximab for cutaneous B-cell lymphoma

Rituximab, a chimeric anti-CD20 monoclonal antibody, has been approved for systemic treatment of relapsed or refractory CD20-positive B-cell non-Hodgkin's lymphoma. As cutaneous B-cell lymphoma (CBCL) also expresses the CD20 molecule, three patients with histologically and immunohistochemically confirmed CBCL without systemic involvement were treated with low-dose intralesional rituximab in a pilot study. Single doses applied ranged from 10 to 30 mg per lesion, according to lesion extent, with a cumulative dose of up to 350 mg. Injections were given two or three times weekly for 3-5 weeks, with a second cycle after 6 weeks in one patient with incomplete remission. Complete and lasting remission was achieved in each patient; this has persisted for up to more than 1 year. The observed adverse events were of grade 1 severity. Results suggest that intralesional rituximab may be a safe and effective new therapy modality for CBCL.     

The morphologic spectrum of primary cutaneous anaplastic large T-cell lymphoma: a histopathologic study on 66 biopsy specimens from 47 patients with report of rare variants

Background:  Primary cutaneous anaplastic large T-cell lymphoma (PCALCL) is a well-defined entity with prognostic differences from the nodal counterpart [nodal anaplastic large cell lymphoma (NALCL)]. Several histological variants of NALCL have been characterized (common, lymphohistiocytic and small cell). However, studies on morphological variants of PCALCLs are lacking.
Methods:  We analyzed retrospectively the clinicopathologic features of 66 biopsies from 47 patients (M : F = 27 : 20; median age: 53 years; mean age: 51.8 years; range: 14–82) with PCALCL, in order to better characterize the spectrum of this unusual neoplasm.
Results:  The 'common variant' was the most frequent (40.4%). In contrast to NALCL, in PCALCL, marked reactive infiltrates are more commonly present. In fact, 26 cases were classified as 'inflammatory type' (15 cases) and 'lymphohistiocytic' (11 cases). Concerning the predominant cell morphology, large anaplastic cells (33%) were almost as frequent as large pleomorphic (36%) and small to medium-sized cells (26%). We reported for the first time in the skin 2 rare cases with the predominance of large cells with a 'signet-ring'-like appearance. Epidermotropism and presence of eosinophils were found in a proportion of cases in all PCALCL variants.
Conclusions:  PCALCL is characterized by variable histopathological presentations and a broad cytomorphologic spectrum.     

Splenic marginal zone B-cell lymphoma with epidermotropic skin involvement

Marginal zone B-cell lymphoma (MZL) is subclassified into extranodal MZL of mucosa-associated lymphoid tissue (including cutaneous lymphomas), splenic MZL, and nodal MZL. We report the case of a 68-year-old man with erythematous-violaceous plaques and nodules. Skin biopsy showed an epidermotropic lymphocytic infiltration and cytology and immunohistochemistry were consistent with MZL. The workup revealed disease in the peripheral blood and bone marrow and massive splenomegaly. Splenectomy confirmed the diagnosis of splenic MZL and led to resolution of the skin lesions. Cutaneous recurrence was treated successfully with chemotherapy and rituximab but caused fatal hepatitis due to hepatitis B virus reactivation. Skin involvement by splenic MZL is uncommon; this form of the disease can present epidermotropism, a very rare finding in primary cutaneous MZL. Treatment consists of splenectomy, which may be associated with chemotherapy and/or rituximab; this treatment may lead to reactivation of latent hepatitis B infection and screening for hepatitis should therefore be performed prior to starting therapy.