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Background Generalized bullous fixed drug eruption (GBFDE), a particular form of fixed drug eruption (FDE), is characterized by widespread blisters and erosions and can be confused with Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). Objective We sought to analyze specific features of GBFDE and differentiate it from SJS/TEN. Methods We retrospectively studied patients with GBFDE and SJS/TEN during a period of 10 years. GBFDE was defined as typical FDE lesions with blisters involving at least 10% body surface area on at least 3 of 6 different anatomic sites. Clinical presentations; histopathological features; immunohistochemical patterns of cluster-of-differentiation (CD)3, CD4, CD8, CD56, Fas, Fas ligand, granzyme B, perforin, granulysin, and forkhead box P3 (Foxp3); and serum granulysin levels were compared. Results Twenty-three cases of GBFDE were collected. Patients with GBFDE had shorter latent periods, less mucosal involvement, more eosinophil infiltration, and dermal melanophages. Lesional infiltrates in GBFDE had more dermal CD4 + cells including Foxp3 + regulatory T cells, fewer intraepidermal CD56 + cells, and fewer intraepidermal granulysin + cells. The serum level of granulysin in GBFDE was also significantly lower than in SJS/TEN. Limitations The number of cases in this study is small. Conclusion GBFDE is a distinct disease distinguishable from SJS/TEN by particular features such as granulysin, CD56, and Foxp3 expressions.

Disseminated cutaneous nontuberculous mycobacteria infection is rare in immunocompetent hosts. We report a case of Mycobacterium intracellulare infection in an immunocompetent patient presenting with simultaneously developing multiple asymptomatic cutaneous papulonodules. The possibility of lung lesions as the primary focus is suspected. We review the literature for other cases of multiple cutaneous M avium complex infections in immunocompetent hosts. There are differences in the virulence of M avium and M intracellulare, and hence in the underlying immune status of the hosts.

Hepatosplenic abscess caused by Actinomyces is rare and often misdiagnosed as malignancy. Herein, we report a case of hepatosplenic actinomycosis in a 37-year-old immunocompetent man with a 2-month clinical history of intermittent fever and upper left abdominal pain. Physical examination revealed a mildly ill-appearing man with a low-grade fever (38°C) and upper left quadrant abdominal tenderness. Abdominal sonographic examination showed the presence of a 6.3 cm × 6.5 cm heterogeneous abscess with a hypoechoic center and honeycomb appearance in an enlarged spleen (8 cm × 5 cm). Computerized tomography of the abdomen revealed a multiloculated splenic lesion, and laparotomy showed multiple hepatic nodules and a splenic abscess. Histopathological examination of the biopsy revealed filamentous branching bacilli and sulfur granules in the hepatosplenic abscess. The patient successfully underwent splenectomy accompanied by intravenous and oral penicillin treatment. Proper and prompt diagnosis of hepatosplenic actinomycosis is important because the therapeutic plan and prognosis of this pathogen are quite different from other microorganisms and malignancies.

Introduction The aim of this study was to investigate the diagnostic value of percutaneous computed tomography (CT)-guided coaxial transthoracic needle biopsy (TNB) for small pulmonary lesions (≤3 cm) with persistent ground-glass opacity (GGO). Methods From January 2004 to February 2010, consecutive patients with persistent small GGO lesions (≤3 cm) who underwent CT-guided TNB were analyzed. The pathologic results of CT-guided TNB were compared with final diagnoses, and the GGO percentage on CT was correlated with the stromal invasion in surgical pathology. Results We performed CT-guided TNB on 1612 patients during the study period. Among them, 55 patients had persistent small GGO lesions (size range 0.5–3.0 cm; 1.72 ± 0.73 cm), 47 were diagnosed with lung adenocarcinomas, and 8 with benign nonspecific lesions. Minor procedure-related complications occurred in 26 patients (47.3%) with small pneumothorax and 11 patients (20.0%) with self-limited mild hemoptysis. The final diagnoses of the 43 patients receiving lobectomy were invasive adenocarcinoma ( n = 23), pure bronchioloalveolar carcinoma ( n = 19), and mucosa-associated lymphoid tissue lymphoma ( n = 1). The diagnostic accuracy was 93.0% (40/43) using CT-guided TNB. Pure GGO lesions had a higher incidence of pure bronchioloalveolar carcinoma than GGO-dominant lesions (70.0% versus 21.7%; p = 0.004). Compared with surgical pathology, stromal invasion was underestimated in 43.5% (10/23) of the TNB specimens, especially in pure GGO lesions (83%, 5/6). In logistic regression analysis, the GGO percentage correlated inversely with stromal tumor invasion ( p = 0.0028). Conclusions CT-guided coaxial TNB is a safe and useful method for diagnosing small (≤3 cm) persistent GGO lesions. Stromal invasion may be underestimated by TNB in GGO lesions.

The clinical, histologic, and radiographic presentations of nontuberculous mycobacterial (NTM) lung disease are usually indistinguishable from those of reactivated pulmonary tuberculosis (TB), so it remains a great challenge for the clinician to make treatment decisions for patients with old TB and a positive culture result for NTM. This study investigated whether the mycobacterial specific heat shock protein 65 (hsp65) and Mycobacterium tuberculosis (MTB)-specific IS6110 gene would present in pulmonary lesions of patients with NTM pulmonary infection. Formalin-fixed and paraffin-embedded (FFPE) tissue blocks of 24 patients with NTM infections treated at the hospital from 1998 to 2008 were included. Mycobacterial hsp65 gene was amplified in 20 of the 24 patients, and the species identified by sequencing was consistent with corresponding culture results in 12 of these patients. MTB-specific IS6110 gene was detected in 3 of the 7 patients who had old TB and a subsequent diagnosis of fibrocavitary NTM lung disease. Polymerase chain reaction (PCR) analysis of hsp65 gene also confirmed the presence of MTB genes in 2 of these 3 patients. Our results indicate that PCR amplification and sequencing of the mycobacterial hsp65 gene is a sensitive assay for identification of NTM species in FFPE materials. However, consistent results of PCR analysis, microbiology study, histologic manifestations, radiology, and clinical presentation are important for correct diagnosis of NTM pulmonary infection. The presence of MTB gene in patients with fibrocavitary NTM lung lesions poses a clinical dilemma for deciding concurrent treatment TB and NTM infection.

Nephrogenic systemic fibrosis (NSF) is an idiopathic, progressive, systemic fibrosis that occurs in patients with renal diseases. Recently, gadolinium-containing contrast (Gd-contrast) has become a suspected causal factor for NFS. This report discusses two female patients with end-stage renal disease, aged 70 and 51 years, respectively, who developed histologically proven NSF after exposure to Gd-contrast. Clinically, both patients were characterized by fibrosis and induration of skin and muscle mainly in the limbs with joint contracture. In the first case, NSF developed gradually after undergoing evaluation by Gd-contrast magnetic resonance imaging (MRI) and subsequent surgery for her urothelial carcinoma. In the second patient, NSF developed after undergoing evaluation by Gd-contrast MRI for her right shoulder bursitis with calcification, and the conditions of NSF continued to worsen after the surgical treatment of this right shoulder lesion. Although the role of Gd-contrast in NSF is still not well known, the correlation in our cases strongly suggests that it should be used with cautioned in patients with end-stage renal disease. Both of our patients underwent surgery before or during the development of NSF, indicating that the surgical procedure may be a contributing factor.

To compare the differences in DNA aberrations between arsenic-exposed and non-arsenic-exposed transitional cell carcinoma (TCC), we analyzed 19 arsenic-exposed and 29 non-arsenic-exposed urinary TCCs from Chi-Mei Hospital using comparative genomic hybridization. DNA aberrations were detected in 42 TCCs including 19 arsenic-exposed and 23 non-arsenic-exposed TCCs. Arsenic-exposed TCCs had more changes than unexposed TCCs (mean ± SD, 6.6 ± 2.9 vs. 2.9 ± 2.2). Arsenic exposure was significantly associated with the number of DNA aberrations after adjustment for tumor stage, tumor grade and cigarette smoking in multiple regression analysis. The most frequent DNA gains, which were strikingly different between arsenic-exposed and non-arsenic-exposed TCCs, included those at 1p, 4p, 4q and 8q. A much higher frequency of DNA losses in arsenic-exposed TCCs compared with non-arsenic-exposed TCCs was observed in 10q, 11p and 17p. Chromosomal loss in 17p13 was associated not only with arsenic exposure, but also with tumor stage and grade. The p53 immunohistochemistry staining showed that chromosome 17p13 loss was associated with either p53 no expression (25%) or p53 overexpression (75%). The findings suggest that long-term arsenic exposure may increase the chromosome abnormality in TCC, and 17p loss plays an important role in arsenic-induced urinary carcinogenesis.

Dermatofibroma (DF) is a common, benign, dermal tumor, often occurring as a single lesion. Multiple eruptive DFs are rare and usually associated with autoimmune diseases, immunosuppressant therapy, or both. We present the case of a 28-year-old woman with dermatomyositis who developed multiple eruptive DFs after undergoing methotrexate and corticosteroid treatment. Immunosuppressants such as methotrexate and corticosteroids might cause multiple eruptive DFs.

Primary tumor or tumor-like lesions of the spleen are rare. Among them, vascular lesions are the most common. Vascular tumor of the spleen is different from the usual hemangioma of soft tissue because the vascular structure of the spleen is unique. Sclerosing angiomatoid nodular transformation (SANT) is a recently described vascular lesion of the spleen. Grossly, it is a multinodular, well-circumscribed tumor containing a hypervascular core. Microscopically, it comprises three types of vessels, and each type recapitulates the immunohistochemical characteristics of the normal vascular elements of the splenic red pulp, i.e. capillaries, sinusoids, and small veins, respectively. Because of the rarity of this entity, its actual pathogenesis is still unknown. In this study, we report a case of SANT occurring in a 43-year-old woman, in whom there were also multiple calcifying fibrous pseudotumors (CFPTs) in the abdominal cavity. Both SANT and CFPT are thought to be variants of inflammatory pseudotumor. Coexistence of these two rare entities in a patient has never been reported, and this fact suggests that there might be a common mechanism contributing to the formation of these two types of lesions. [J Formos Med Assoc 2007;106(3):234-239]

Background/purpose Early mycosis fungoides (MF) is difficult to distinguish from other benign inflammatory dermatoses. We evaluated clonal T-cell receptor (TCR) γ Gene rearrangement by polymerase chain reaction (PCR) as a surrogate to histologic diagnosis in early MF. Methods Twenty paraffin-embedded skin biopsies from nine patients diagnosed with MF were included. Two multiplex PCR encompassing various Vγ and Jγ regions were used to detect TCRγ Gene rearrangements. Histologic diagnoses were categorized as “diagnostic”, “consistent”, “suggestive”, or “nondiagnostic”. We compared TCRγ PCR results with histologic parameters to determine the differences between PCR-positive and PCR-negative groups. Results TCRg PCR was positive in 53% (8/15) of the patch stage, in 100% (2/2) of the plaque stage, and in 100% (3/3) of the tumor stage. TCRγ PCR was positive in 50% (4/8) of the specimens in both the diagnostic and consistent of MF groups, 71% (5/7) in the suggestive of MF group. We found that inflammation was more severe in PCR-negative specimens. Papillary dermal fibrosis was common, and differed significantly between PCR-positive and PCR-negative groups ( p = 0.01). T-cell monoclonality was detected in one nondiagnostic lesion in a patient with psoriasis and MF. Conclusion TCRγ PCR allows the diagnosis of MF in patients with lymphocyte-poor lesions, suggestive of MF pathologically. TCRg PCR is more likely to be negative with moderate to severe inflammation, particularly with papillary dermal fibrosis. We suggest that the ratio of malignant clonal to reactive T-cells is critical for MF diagnosis. [ J Formos Med Assoc 2007;106(4):265-272]

We describe the case of a 13-year-old boy with stage I hypopigmented mycosis fungoides in whom minimal residual disease was detected with T-cell receptor gamma-polymerase chain reaction after the disease was in complete clinical remission. We further cloned and sequenced the T-cell receptor gamma-polymerase chain reaction product of the lesion in remission and found that the original T-cell clone still existed in decreased amounts. The patient was followed up for 3 1/2 years without any new lesions developing. The clinical significance of this residual malignant T-cell clone in mycosis fungoides remains to be elucidated.

Summary This article describes the pathological studies of fatal severe acute respiratory syndrome (SARS) in a 73-year-old man during an outbreak of SARS in Taiwan, 2003. Eight days before onset of symptoms, he visited a municipal hospital that was later identified as the epicenter of a large outbreak of SARS. On admission to National Taiwan University Hospital in Taipei, the patient experienced chest tightness, progressive dyspnea, and low-grade fever. His condition rapidly deteriorated with increasing respiratory difficulty, and he died 7 days after admission. The most prominent histopathologic finding was diffuse alveolar damage of the lung. Immunohistochemical and in situ hybridization assays demonstrated evidence of SARS-associated coronavirus (SARS-CoV) infection in various respiratory epithelial cells, predominantly type II pneumocytes, and in alveolar macrophages in the lung. Electron microscopic examination also revealed coronavirus particles in the pneumocytes, and their identity was confirmed as SARS-CoV by immunogold labeling electron microscopy. This report is the first to describe the cellular localization of SARS-CoV in human lung tissue by using a combination of immunohistochemistry, double-stain immunohistochemistry, in situ hybridization, electron microscopy, and immunogold labeling electron microscopy. These techniques represent valuable laboratory diagnostic modalities and provide insights into the pathogenesis of this emerging infection.

We describe a 69-year-old female farmer with diabetes mellitus who developed subcutaneous infection due to a plant pathogen, Corynespora cassiicola. The organism was identified based on characteristic morphotypes and confirmed by sequence analysis of the internal transcribed spacer (ITS) regions. The patient was treated successfully with amphotericin B therapy.

Histiocytoid Sweet syndrome is a recently described variant of acute febrile neutrophilic dermatosis. The histiocytoid cells are easily misinterpreted as histiocytes, when in fact they are immature myeloid cells. The recognition of myeloperoxidase expression in these cells is important in avoiding confusion with histiocyte-rich dermatoses. Herein, we report a case of histiocytoid Sweet syndrome with neutropenia that had an unusual presentation. The recognition of this rare combination helps expand the spectrum of Sweet syndrome with histiocytoid infiltrate.

Chow-In Ko, MD, Chien-Ching Hung, MD, Mao-Yuan Chen, MD, Po-Ren Hsueh, MD, Cheng-Hsiang Hsiao, MD, Jau-Min Wong, MD Diarrhea is the most common GI presentation of immunocompromised patients, especially those with acquired immunodeficiency syndrome (AIDS).1,2 More than 50% of patients with AIDS have diarrhea caused by a variety of pathogens during their illness.1,2 Intestinal mycoses are uncommon in immunocompromised patients, however, and may be caused by Cryptococcus neoformans3 and Histoplasma capsulatum.4 Penicillium marneffei, an emerging facultative intracellular pathogen and the only thermally dimorphic fungus of the genus Penicillium, can cause disseminated infection in patients residing in or traveling to areas where the organism is endemic, which include southeast Asia, southern China, and Hong Kong.5-17 With the human immunodeficiency virus (HIV) pandemic, the number of cases of penicilliosis marneffei has increased markedly during the past 5 years, from 30 cases during the period between 1973 and 1990 to more than 160 by the end of 1995.16 After extrapulmonary tuberculosis and cryptococcosis, penicilliosis marneffei ranks as the third most common opportunistic infection in HIV-infected patients in Thailand.14 Although P marneffei has become an increasingly important cause of invasive mycoses in immunocompromised hosts, it has been rarely implicated as a cause of intestinal mycoses. As of December 1997, only four cases of intestinal penicilliosis marneffei have been reported in English language publications and only one was diagnosed antemortem by endoscopy.8,9,12,17 Here we report three cases in which immunocompromised patients, two with AIDS and one after renal transplantation, presented with intestinal penicilliosis marneffei where early diagnosis was made possible by endoscopy.

N eutrophilic dermatoses (ND) are characterized histologically by an epidermal and/ or dermal infiltrate of polymorphonuclear leukocytes, absence of microorganisms on special stains and cultures, and clinical improvement after treatment with systemic steroids. ND includes subcorneal pustular dermatosis, Sweet’s syndrome, erythema elevatum diutinum (EED), pyoderma gangrenosum (PG), neutrophilic eccrine hidradenitis, and granuloma faciale. We describe a pediatric patient with glomerulonephritis who successively developed 3 types of ND: EED-like dermatosis; subcorneal vesicular neutrophilic eruption; and acute form of PG.

BACKGROUND & AIMS: Bowel ischemia and perforation is an infrequent but ominous event in patients with malignant lymphoma. The underlying pathogenesis of this association remains to be clarified in most cases. Seven unusual cases of angiocentric T-cell lymphoma of the intestine presenting with bowel ischemia or perforation are reported. Their clinicopathologic features were analyzed. METHODS: Clinical records and histopathology were reviewed. Immunophenotypic studies and EBER1 in situ hybridization for Epstein-Barr virus were performed. RESULTS: All patients (3 men and 4 women; mean age, 42 years) presented with acute abdominal pain, bloody stool, or bowel perforation. The intestinal lesions were ulcerated and transmurally necrotic or gangrenous. The angiodestruction of the tumor cells appeared to be responsible for the presenting bowel ischemia. The neoplastic cells expressed a pan-T-cell antigen CD45RO; 4 of them contained Epstein-Barr virus transcripts EBER1. The prognosis was grave; 6 patients died within 3 months of onset. CONCLUSIONS: Intestinal angiocentric T-cell lymphoma represents a distinct etiology of bowel ischemia. The condition should be taken into consideration, particularly in young adults with ischemic bowel disease. (Gastroenterology 1996 Apr;110(4):985-90)

Nontuberculous mycobacteria (NTM) are unusual pathogens causing ocular infections, mostly seen as keratitis, scleral buckle infections, and implantation infections, which are usually related to ocular trauma, or surgery. Conjunctival NTM infections are even rarer. We present a case of conjunctival NTM granuloma without surgical history, confirmed by acid-fast stain and nested polymerase chain reaction (PCR) analysis in the formalin fixed and paraffinembedded (FFPE) tissue block. A 49-year-old otherwise healthy woman suffered from a large, reddish conjunctival nodule in the left eye for 2 months. She was a frequent wearer of daily disposable soft contact lenses (SCL) for high myopia and raised three pet parrots (Melopsittacus undulatus). On examination, a large, elevated, fleshy, and solid mass (15 mm 10 mm), containing some yellowish nodules within, was noted at the temporal bulbar conjunctiva of the left eye (Fig. 1A). Her systemic and other ocular examinations were normal. The

Cutaneous non-mycosis fungoides (MF) T- and NK/T-cell lymphomas are heterogeneous with different degrees of aggressiveness. Extranodal natural killer/T-cell lymphoma (ENKTL) is a predominantly extranodal lymphoma associated with EBV. In our recent Asian collaborative study on primary cutaneous ENKTL, we identified five patients initially misdiagnosed as various types of PTCL because EBER was not performed. Three patients were males and two were females, with a median age of 45 years (range 28~71 years). Based on the initial diagnoses (two as PTCL, two as primary cutaneous CD30-positive T-cell lymphoproliferative disorders and one as subcutaneous panniculitis-like T-cell lymphoma), all patients received CHOP chemotherapy and three with additional radiotherapy. All patients experienced persistent disease or relapse despite treatment (mean duration 11 months, range 1~24 months). These rare tumors often pose significant diagnostic challenges to the practicing dermatopathologists and (hemato)pathologists. We proposed to include EBER as a screening assay for primary cutaneous non-MF T-cell lymphoma, at least in East Asia where the relative frequency of such tumors is higher than in the West. As novel chemotherapy regimen SMILE (L-asparaginase, methotrexate, ifosphamide, etoposide, and dexamethasone) has been shown to be more effective than CHOP for ENKTL, a correct diagnosis of ENKTL is very important.

Purpose To evaluate the effectiveness of conjunctival resection combined with Tenon layer excision in treating superior limbic keratoconjunctivitis (SLK) and the involvement of mast cells in SLK. Design Retrospective, interventional case series. Methods Forty eyes of 30 SLK patients who were unresponsive to medical treatment received superior bulbar conjunctival resection, and another 20 patients who underwent cataract and retinal surgery served as a control group. The conjunctiva specimens from study and control patients were examined by hematoxylin and eosin staining and immunohistochemistry using antibodies against mast cell tryptase. Results In all operated eyes, the clinical symptoms and signs, including irritation and redness and superior bulbar conjunctival hyperemia and superior tarsal conjunctival papillary hypertrophy, subsided significantly three months after the operation. Only three eyes had recurrence from the margin of conjunctival resection, and this was relieved after reoperation. Keratinized conjunctival epithelium, loss of goblet cells, and increased mast cell numbers ( P Conclusions Our cases demonstrate that superior bulbar conjunctival resection combined with Tenon layer excision is an effective treatment for SLK. The pathologic findings suggest that mast cells may play a role in the pathogenesis of SLK.