Sweet Syndrome due to Myelodysplastic Syndrome: Possible Therapeutic Role of Intravenous Immunoglobulin in Addition to Standard Treatment.

We report an 82-year-old lady who developed sudden onset nodular and erythematous lesions and neutrophilia following an episode of urinary tract infection. Skin biopsy confirmed the diagnosis of Sweet syndrome. Response to the use of prednisolone alone was not satisfactory. The skin lesions however showed a sustained response to the regular use of intravenous immunoglobulin (IVIG) and prednisolone was slowly weaned off. Our case highlights the possible therapeutic role of IVIG in managing this condition.

Diffuse large B-cell lymphoma of the central nervous system in mycophenolate mofetil-treated patients with systemic lupus erythematosus.

Patients with systemic lupus erythematosus (SLE) are susceptible to the development of lymphoproliferative disorders and postulated causes include intrinsic defects in immune surveillance and iatrogenic administration of immunosuppressants. Since the introduction of mycophenolate mofetil (MMF) to the immunosuppressive regimen for the management of post-organ transplantation, there have been reports of primary lymphoma of the central nervous system (PCNSL). MMF has been widely used to treat active SLE patients with Class IV lupus nephritis. In addition to two previously reported cases of PCNSL among SLE patients on long-term MMF, we report a third patient who has been on treatment with MMF for 8 years. The histology showed features compatible with diffuse large B-cell lymphoma with strong immunohistochemical staining for CD20 and positive signal for Epstein-Barr virus (EBV)-encoded RNA by in-situ hybridization that is similar to other case reports, suggesting EBV driven B-cell lymphoproliferative disease. The patient responded to withdrawal of MMF, intravenous methotrexate, rituximab and whole brain radiotherapy. With the increasing use of MMF in active renal as well as non-renal exacerbations of SLE, PCNSL should be included in the differential diagnosis in patients who present with gradual onset of focal neurological deficit.

Vulvar basal cell carcinoma in China: a 13-year review.

OBJECTIVE: We conducted a 12-year retrospective review of vulvar basal cell carcinoma (BCC) in a Chinese population. STUDY DESIGN: Medical records and histopathologic reports were examined from 5 major Hospitals in Hong Kong to list all patients diagnosed with vulvar BCC. Clinical data and histologic materials were reviewed. RESULTS: Sixteen vulvar BCCs were diagnosed. Most of them were pigmented. They were removed by simple excision or wide local excision. All the carcinomas were identified in the reticular dermis. The predominant histologic pattern was nodular, which may be mistaken as adenoid cystic carcinoma. CONCLUSION: The high proportion of pigmented vulvar BCCs suggested that biopsy should be performed for any pigmented lesion in a Chinese patient. The BCCs are superficial and tissue-preserving treatment approach is recommended. The tumor depth estimation is difficult and intraoperative frozen section consultation may be helpful. Formal histopathologic assessment should be used to reach an objective diagnosis.

Penile lichen sclerosus after allogeneic stem cell transplantation.

Graft-versus-host disease (GVHD) often complicates allogeneic stem cell transplantation (SCT) and affects mainly the gut, liver, lung and skin. The microscopic morphological features of late-phase sclerodermatous chronic GVHD in the skin, namely epidermal atrophy, lymphoplasmacytic infiltration, dense dermal fibrosis and adnexal atrophy, are histologically indistinguishable from those in sporadic systemic sclerosis, morphoea and the related condition of lichen sclerosus. Mucosal orifices including those of the genitourinary system may be severely affected. We present three SCT recipients with chronic GVHD and severe posthitis leading to phimosis requiring surgery. The excised prepuces showed features of lichen sclerosus including epidermal atrophy and a subepidermal zone of eosinophilic, homogeneous and hyalinized collagen above a band-like lymphoplasmacytic infiltrate. These cases add further evidence to support the notion that penile lichen sclerosus should be included within the expanding sclerodermoid spectrum of late-stage cutaneous chronic GVHD.

Pulmonary iron overload in thalassemia major presenting as small airway disease.

Lung function abnormalities that are associated with thalassemia major are variable with etiology that is yet undetermined. Some studies have suggested that pulmonary iron deposition is a probable cause for these lung defects although there has been no antemortem histopathological and radiological evidence for this. We report a case of thalassemia major with biopsy-proven pulmonary iron overload, in which thoracic high-resolution computed tomography revealed a morphological-functional correlation consistent with small airway disease.

Primary extramedullary plasmacytoma of the lacrimal gland.

Primary extramedullary plasmacytoma (EMP) of the lacrimal gland is rare. Here we describe the histopathologic and imaging findings in a 77-year-old patient with primary extramedullary plasmacytoma of the lacrimal gland. The usefulness of CD79a in the diagnosis of plasmacytoma and the good response of the tumor to combined surgery and radiotherapy are illustrated. Plamacytoma should be included in the differential diagnosis of lacrimal tumors.

Adenoid cystic carcinoma of the breast: a tumour commonly devoid of oestrogen receptors and related proteins.

AIM: Adenoid cystic carcinoma (ACC) of the breast is an uncommon well-differentiated tumour with good prognosis, and sometimes difficult to distinguish from in-situ and invasive cribriform carcinoma (ICC) which are relatively more common. Recently, we encountered a case of ACC that proved to be totally oestrogen receptor (ER) negative by immunohistochemistry. We investigated the possibility that this may be a consistent feature that can help in differentiating ACC from ICC which are usually ER positive. METHODS AND RESULTS: The immunoperoxidase technique was used to study the expression of ER and other related proteins in six cases of ACC and two cases of ICC. All ACC cases were negative for oestrogen (ER) and progesterone (PgR) receptors whereas the two ICC were strongly positive for ER and showed a variable degree of PgR positivity. In addition, ACC cases were pS2 negative and showed minimal expression of prolactin receptors (PrlR), while the two ICC showed widespread and strong staining for pS2 and PrlR. The percentages of cells staining positively for Ki67 and p27 were generally lower in ACC than in ICC. Both tumour types were c-erbB-2 negative, but p53 was weakly to moderately positive. CONCLUSIONS: The findings suggest that a negative immunoperoxidase staining for ER would confirm the diagnosis of ACC in contrast to the positive staining which is always seen in ICC. The findings also raise the issue of the presence of a specific class of ER negative breast carcinomas which are negative not because of poor differentiation, but because of their derivation from, or differentiation along, an ER negative cell lineage.

The evaluation of salivary gland tumours using proliferating cell nuclear antigen.

In order to assess its discriminating and prognostic value, we studied the immunoreactivity for proliferating cell nuclear antigen (PCNA) in tissue from 52 human salivary gland tumours using the murine monoclonal antibody PC10. The PCNA percentage count, namely, the average number of positive nuclei counted per 100 randomly selected tumour cells was recorded for each tumour. Anaplastic carcinoma was used as a positive control and histologically 'normal' salivary gland and tonsil served as a negative control. A PCNA count of 30 per cent was postulated to predict malignancy within a given salivary gland tumour i.e. a PCNA count of 30 per cent or above would indicate malignant potential. This gave a sensitivity of 96.9 per cent and a specificity of 95.2 per cent and a positive predictive value of determining malignancy of 96.8 per cent. We conclude that PCNA immunoreactivity is useful in discriminating between benign and malignant salivary gland tumours and that it may have prognostic value in this diverse group of neoplasms.

Absence of Epstein-Barr virus in testicular germ cell tumours: a study of 21 cases using in situ hybridisation.

Aim-To establish whether testicular germ cell tumours contain Epstein-Barr virus (EBV) and if so to provide further evidence for the hypothesis that EBV plays a direct role in the pathogenesis of testicular germ cell tumours.Method-Paraffin wax embedded tissue blocks from 21 germ cell tumours including 12 teratomas and nine classic seminomas were examined by in situ hybridisation for the expression of the small EBV encoded nuclear RNA transcripts (EBER-1 and -2) using isotopic and nonisotopic probes.Results-There was no EBER specific signal detectable in any of the testicular germ cell tumours examined by in situ hybridisation whilst a strong signal was observed in appropriate control sections.Conclusion-The absence of demonstrable EBER transcripts in testicular germ cell tumours make a direct role for EBV in the pathogenesis of these tumours unlikely. Other explanations for the epidemiological and serological evidence linking EBV with germ cell tumours need to be explored.

An unusual case of colonic angiodysplasia.

An unusual case of a colonic vascular anomaly resembling angiodysplasia associated with right sided diverticular disease is presented. The patient, a 74 year old man, presented with a four day history of rectal bleeding and subsequently underwent hemicolectomy. The resected specimen was flushed out with heparin-saline solution and injected with a barium-gelatine mixture. Preoperative barium enema revealed right sided diverticula, whereas post-resection angioradiography revealed the "coral reef" vascular anomaly consistent with angiodysplasia. Histology confirmed the presence of both diverticular disease and angiodysplasia. This case report highlights the importance of considering a vascular anomaly in patients presenting with rectal bleeding despite the presence of another radiologically demonstrable anatomical lesion.