-Shang Hong

Clinicopathlogical features and prognosis of drug rash with eosinophilia and systemic symptoms : a study of 30 cases in Taiwan

Background  Drug rash with eosinophilia and systemic symptoms (DRESS), a group of non‐blistering severe cutaneous adverse drug reactions (SCADRs), is characterized by skin rash and multiorgan involvement. Details of this reaction have not been reported in the literature so far.

Lupus lymphadenitis simulating Kikuchi's lymphadenitis in patients with systemic lupus erythematosus : A clinicopathological analysis of six cases and review of the literature

Kikuchis disease (KD) or Kikuchis lymphadenitis (KL) is a self‐limiting disease mostly affecting the cervical lymph nodes of young individuals. Whether the reported cases of KL associated with systemic lupus erythematosus (SLE) were genuine KL or lupus lymphadenitis (LL) simulating KL in SLE patients is not clear. We analyzed six cases of KD‐like lymphadenitis occurring in SLE patients and 12 reported cases to clarify the relationship between KL and SLE. We found that not all cases occurred simultaneously with SLE. Eight cases occured either before or after SLE. These cases might have true KL independent of SLE with the exception of two cases that occurred after SLE, but the patients still had lupus activity. The 10 cases that coexisted with SLE most likelty had LL rather than KL. This was supported by the immunohistochemical finding of sparse cytotoxic T cells in those lymph nodes in contrast to abundant cytotoxic T cells usually seen in a typical KL. We conclude that KL is not related to SLE, and KD‐like lymphadenitis coexisting with SLE should be regarded as LL. Pathologists should be aware of the possibility that LL can mimic KL in patients with SLE, especially necrotizing‐type KL.

Detection of Sporothrix schenckii in Clinical Samples by a Nested PCR Assay

ABSTRACT Cutaneous sporotrichosis is a chronic granulomatous fungal infection caused by Sporothrix schenckii with worldwide distribution. Its traditional diagnosis is time-consuming and difficult to differentiate from that of a clinical sporotrichoid lesion caused by various pathogens. In this study, a nested PCR assay for the detection of S. schenckii was evaluated by using a sequence of 18S rRNA gene as a target. For the examination of specificity and sensitivity, five clinical isolates with 1 ATCC 10213 strain of S. schenckii, 10 strains of clinical common fungi, 3 strains of Mycobacterium spp., Staphylococcus aureus, and normal human skin tissue were used. The expected fragment was amplified from six S. schenckii isolates in the first round and nested PCR but not from other microorganisms and human DNA. Their sequences were 100% identical to the S. schenckii 18S rRNA gene sequence deposited in GenBank. A detection limit of 40 fg of S. schenckii DNA extract was determined with ethidium bromide staining. Serial dilution studies demonstrated that the nested PCR could detect a DNA amount of 1 CFU of S. schenckii in tissue samples. We further investigated the nested PCR assay for the detection of S. schenckii from the tail tissues of 5 experimentally infected mice and from the clinical biopsy specimens of 12 patients with sporotrichosis confirmed by culture or histochemical staining. The nested PCR assay was positive in all 5 infected mice and in 11 of the 12 clinical specimens. The high sensitivity and specificity of this nested PCR indicate that the assay can provide rapid diagnosis with sufficient accuracy to be clinically useful for patients with sporotrichosis.

Granulomatous variant of chronic pigmented purpuric dermatoses : report of four new cases and an association with hyperlipidaemia

Four patients presenting with chronic pigmented purpuric dermatosis (CPPD) on the limbs were found to have granulomatous inflammation superimposed on the pathological changes of CPPD. Three of the four patients had hyperlipidaemia. Therefore, the granulomatous reaction observed could be associated with hyperlipidaemia. Whether it occurs only in Asian people or not needs further observation.

Association between the insertion/deletion polymorphism of the angiotensin I-converting enzyme gene and risk for psoriasis in a Chinese population in Taiwan

Background  Genetic factors play an important role in susceptibility for psoriasis. The angiotensin I‐converting enzyme (ACE) is expressed by keratinocytes. Administration of ACE inhibitors may induce or exacerbate psoriasis in clinical practice. Thus, ACE gene variants may contribute to the genetic background of psoriasis.

Aggressive digital papillary adenocarcinoma: a review.

Vigorous treatment of aggressive digital papillary adenocarcinoma (ADPA), including amputation, has been recommended by most authors, but the appropriateness and effectiveness of excision as an alternative to amputation has not been systematically evaluated. To evaluate the appropriateness and effectiveness of excision as an alternative to amputation in the treatment of ADPA, we reviewed the clinical presentations, treatments and patient outcomes presented in case reports on ADPA available on Ovid MEDLINE. We also assessed the results of immunohistochemical staining for proliferation markers in one patient in order to explain the nonaggressive nature of ADPA noted in that patient. Except for the duration of lesions, there was no significant difference in clinical outcome between the excision and amputation groups. We also found that p63 may be a useful marker for distinguishing primary ADPA from metastatic adenocarcinomas. In addition, the intensity of Ki67 expression in tumour cells may be a marker of aggressive behaviour and thus be helpful in therapeutic decision‐making. Wide excision with or without sentinel lymph‐node biopsy is a feasible alternative to amputation. It should be considered in patients who present with a long‐standing history of ADPA without evidence of underlying bone invasion or distant metastasis and with low‐intensity expression of proliferation markers.

Fatal sodium valproate–induced hypersensitivity syndrome with lichenoid dermatitis and fulminant hepatitis

Anticonvulsant hypersensitivity syndrome caused by sodium valproate (Depakene) has rarely been reported. Here we describe a patient who presented with nonspecific polymorphous eruptions, fulminant hepatitis, and jaundice. Lichenoid dermatitis was found in the skin biopsy specimen. Toxic hepatitis and microvesicular steatosis were found in the liver biopsy specimen, consistent with valproate hepatotoxicity.

Disseminated xanthogranulomas associated with adult T‐cell leukaemia/lymphoma: a case report and review the association of haematologic malignancies

Xanthogranuloma (XG) is rarely observed in adults and has been reported to be associated with chronic myelogenous leukaemia (CML) and/or neurofibromatosis type 1 (NF1). A 68‐year‐old woman with adult T‐cell leukaemia/lymphoma (ATLL) gradually developed disseminated XGs over the 3 years since disease onset. Histopathological examination of a skin biopsy revealed the presence of histiocytes in the dermis with a few Touton giant cells admixed with lymphoid cells. The lesions of XGs persisted despite chemotherapy with prednisolone and chlorambucil for her ATLL. This is the first report of disseminated XGs associated with ATLL. The association of disseminated XGs with haematologic malignancies was reviewed and the possible pathogenesis of this association will be discussed.

Treatment of a tufted angioma with intense pulsed light

Tufted angioma is a rare cutaneous angiomatous proliferation named because of its characteristic histologic pattern of grouped dermal capillary tufts. Lesions usually begin in infancy or early childhood but rarely are congenital. Clinical manifestations are dull red coalescent papules and plaques, most commonly located on the neck, shoulders, and upper back, and can sometimes be tender. Although transformation to malignancy has not been described, tufted angiomas do not tend to regress. Effective treatments reported in the literature are scarce. We report an adult case of tufted angioma, with unusual presentation as annular plaques, which was alleviated after treatment with intense pulsed light in terms of both cosmetics and discomfort.

Seeing the gene therapy: Application of gene gun technique to transfect and decolour pigmented rat skin with human agouti signalling protein cDNA

We developed a gene gun method for the transfer of human agouti signalling protein (ASP) cDNA to alter rat skin colour in vivo. Human ASP cDNA was cloned into a modified cytomegalovirus plasmid and delivered to the skin of Long–Evans rats by gene gun bombardment. Skin pigmentation, body weight and blood sugar of ASP cDNA-transfected rats were recorded against the control group, which were injected with plasmids encoding for green fluorescent protein. The treated skin showed lighter skin colour after 3 days of ASP gene transfection. This depigmentation effect was most prominent on day 14 and the skin gradually returned to its original pigmentation by day 28. Successful transfection of ASP gene in skin and hair follicles, as well as downregulation of melanocortin-1 receptor (MC1R) and tyrosinase expression upon treatment, was confirmed using immunohistochemistry and Western blot analysis. Body weight and blood sugar in the treated rats did not show statistically significant differences as compared to control groups. These observations demonstrate that gene transfer using the gene gun method can induce high cutaneous ASP production and facilitate a switch from dark to fair colour without systemic pleiotropic effects. Such a colour switch may be that ASP is acting in a paracrine fashion. In addition, this study verifies that ASP exerts its functions by acting as an independent ligand that downregulates the melanocyte MC1R and tyrosinase protein in an in vivo system. Our result offers new, interesting insights about the effect of ASP on pigmentation, providing a novel approach to study the molecular mechanisms underlying skin melanogenesis.