Chromoblastomycosis in Taiwan: A report of 30 cases and a review of the literature.

Chromoblastomycosis (CBM) is an implantation mycosis characterized by the presence of pigmented muriform cells in tissue. CBM is endemic in Taiwan, but only three formal cases have been reported to date because of underreporting. To describe and update its epidemiologic features, we report a series of 30 cases between 2003 and 2016 at a single medical center. Patients were predominately male (2.75:1). The mean age of onset was 65.9 years, and disease duration ranged from 2 months to 20 years. Diabetes was the most common comorbidity, and extremities were the most frequent sites of involvement. The lesions presented as papuloplaque, verrucous, cicatricial, targetoid, or mixed types. The dermoscopic features were variable, including red dots, white vague areas, black globules, and sand-like patterns. Among 10 Fonsecaea isolates further identified by sequencing the ITS regions of ribosomal DNA, nine were F. monophora and one was F. nubica. All but one patient received either systemic antifungal agents, surgical excision, or both. Surgical excision achieved a higher complete remission rate than the other forms of treatment did.

Medical genetics: a marker for Stevens-Johnson syndrome.

Stevens-Johnson syndrome and the related disease toxic epidermal necrolysis are life-threatening reactions of the skin to particular types of medication. Here we show that there is a strong association in Han Chinese between a genetic marker, the human leukocyte antigen HLA-B*1502, and Stevens-Johnson syndrome induced by carbamazepine, a drug commonly prescribed for the treatment of seizures. It should be possible to exploit this association in a highly reliable test to predict severe adverse reaction, as well as for investigation of the pathogenesis of Stevens-Johnson syndrome.

Cutaneous normolipemic plane xanthoma with supraglottic involvement in a patient with hand-Schüller-Christian Disease: a case report.

Normolipemic plane xanthoma (NPX) is a histiocytic disorder characterized by yellow-orange plaques in the periorbital areas, neck, upper trunk, and flexural folds. Association with systemic disease or paraproteinemia has been reported previously, but rarely with Langerhans cell histiocytosis (LCH). We report a case of Hand-Schüller-Christian disease (a type of LCH) in a patient who developed NPX with supraglottic involvement. NPX developed after several courses of chemotherapy and the supraglottic xanthoma occurred about 2 years later. The coexistence of LCH and non-LCH histiocytic lesions in this patient could be a result of chemotherapy-induced changes or may be just coincidental.

Bullous pemphigoid in a renal transplant recipient: a case report and review of the literature.

Bullous pemphigoid (BP) is an autoimmune disease with chronic, recurrent bullous eruptions. BP has been reported to be associated with drugs, physical stimuli, malignancies, and immune abnormalities. Its association with renal transplant is rare and only four cases have been reported. We present a case of BP in a 52-year-old man with chronic hepatitis B and C infection who underwent a cadaveric renal transplant 13 years earlier. His graft was still functioning well when BP appeared. The occurrence of BP in our patient might be a result of drugs (furosemide or tacrolimus), viruses, or renal allograft. As the patient was receiving regular T-cell immunosuppressant therapy, his BP lesions were recalcitrant to corticosteroid treatment. We discuss the pathogenesis and treatment of such patients.

Intravenous immunoglobulin-induced, non-eczematous, vesiculobullous eruptions in Stevens-Johnson syndrome.

Intravenous immunoglobulin (IVIG) has emerged as a promising treatment that interrupts the progression of Stevens-Johnson syndrome (SJS). Our patient experienced an uncommon adverse effect, non-eczematous, vesiculobullous eruptions, after treatment with IVIG. These new lesions developed rapidly on the palms while most previous SJS bullous lesions subsided. A skin biopsy of these new lesions showed an intracorneal vesicle, without epidermal necrosis, with inflammatory cell infiltration. IVIG-induced, vesiculobullous eruptions are discussed, along with their possible pathogenesis. With the increasing use of IVIG for treatment of bullous dermatoses, recognition of this rare adverse effect is important for prompt differential diagnosis.

Bilateral sporotrichoid lymphocutaneous dermatosis in a drug abuser: case report and review of the literature.

Bilateral sporotrichoid mycobacterial cutaneous infection is unusual and has been reported in only three cases involving different species and with an unknown route of infection in the literature.We report a case of bilateral sporotrichoid dermatosis in an immunocompetent patient with a history of intravenous heroin injection before development of the skin lesions. Both special stain and culture of biopsy specimen were negative. Finally, Mycobacterium fortuitum was identified by a polymerase chain reaction-based method. The patient responded well to clarithromycin and ciprofloxacin therapy. This case represents an unusual primary cutaneous M. fortuitum infection manifested as bilateral sporotrichoid lesions of the limbs. Review of previous reported cases of bilateral sporotrichoid mycobacterial infection shows different isolated organisms and routes of infection to that found in our case.

Metastasis of mediastinal epithelioid angiosarcoma to the finger.

Epithelioid angiosarcoma (EA) is a rare malignant, vascular tumor that is usually observed in middle-aged and elderly males. Cutaneous metastasis of EA is extremely rare. We report the case of a 41-year-old woman presenting with a painful bluish, bulla-like lesion on the distal extent of the left third finger. The patient had recently been diagnosed with mediastinal EA with disseminated metastases. The skin biopsy specimen revealed metastatic EA. This is thought to be the first reported metastasis of EA to the finger. Whenever a patient has metastatic disease, acrometastases should be considered in the differential diagnosis of inflammatory lesions of the digits and a skin biopsy should be performed.

Bullous amyloidosis in a hemodialysis patient is myeloma-associated rather than hemodialysis-associated amyloidosis.

We report a 78-year-old woman on hemodialysis who presented with refractory multiple pruritic vesicles and bullae on her trunk and extremities for 2 months. Histopathologic examination of skin biopsy specimen showed subepidermal bullae with many amyloid deposits in the papillary dermis. No evidence of systemic amyloidosis could be found on physical examination. While the initial clinical diagnosis was bullous pemphigoid, the histopathology and direct immunofluorescence result favored hemodialysis-associated amyloidosis. However, immunochemical study for beta(2)-microglobulin was negative. Further hematologic and immunologic work-up revealed the presence of multiple myeloma and that the deposit was AL amyloid. This is the first case of bullous amyloidosis in a hemodialysis patient and should remind dermatologists that bullous amyloidosis should be considered in addition to the usual presentation of porphyria cutanea tarda and pseudoporphyria for bullous dermatosis in the hemodialysis patient. We also suggest that hemodialysis-associated amyloidosis should not be taken for granted in the hemodialysis patient with cutaneous amyloidosis without systemic signs and symptoms. Further testing for other types of amyloid should be performed.

Cellulitis-like cutaneous metastasis of uterine cervical carcinoma.

Metastasis to the skin from cervical carcinoma is relatively uncommon. Herein we present a 41-year-old woman with a history of cervical carcinoma with severe facial erythematous swelling and telangiectasia. She was initially treated for cellulitis without improvement. A skin biopsy specimen revealed widespread intravascular tumor emboli in the dermis and subcutis, resembling the so-called inflammatory carcinoma of the breast. As this is an unusual clinical presentation for the metastasis of cervical carcinoma, this case is reported.

Malignant cutaneous tumors of the scalp: a study of demographic characteristics and histologic distributions of 398 Taiwanese patients.

BACKGROUND: The scalp is a unique anatomic region, in which pilosebaceous follicles are concentrated. OBJECTIVE: We sought to investigate demographic characteristics and histologic distributions of malignant scalp tumors. METHODS: Primary and metastatic scalp malignancies diagnosed histopathologically between 1983 and 2003 were reviewed. Age at diagnosis, sex, and histologic types were analyzed. RESULTS: A total of 398 Taiwanese patients (200 males, 198 females) were selected. Age at diagnosis ranged from 3 to 103 years. Most malignant scalp tumors (69.8%) occurred in those 50 years or older. Basal (41.2%) and squamous (16.6%) cell carcinomas were the most common histologic types. Surprisingly, metastatic tumors (12.8%) came in third, in which lung cancers were the most frequent primary tumor in both male and female patients. LIMITATIONS: In our series, the case number of metastatic scalp malignancies was underestimated because not all patients with metastatic scalp tumors received a scalp skin biopsy. CONCLUSION: Because a wide spectrum of primary and metastatic malignancies can occur on the scalp, scalp inspection should be included in general screening for either skin or internal cancers.

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.

Early congenital syphilis and erythema multiforme-like bullous targetoid lesions in a 1-day-old newborn: detection of Treponema pallidum genomic DNA from the targetoid plaque using nested polymerase chain reaction.

A 1-day-old male newborn was born with respiratory distress, low birth weight, hepatosplenomegaly, and bullous targetoid skin lesions over the face, back, buttocks, and extremities. A diagnosis of early congenital syphilis was made based on a treponemal serologic test. Pathologic examination of the skin lesion showed scattered dyskeratotic cells in the epidermis and interface dermatitis consistent with erythema multiforme. No spirochete could be found in the skin sections staining with Warthin-Starry stain. Using nested polymerase chain reaction, treponemal genomic DNA fragments encoding DNA polymerase I were detected.

Fulminant type 1 diabetes mellitus caused by drug hypersensitivity syndrome with human herpesvirus 6 infection.

Drug hypersensitivity syndrome (DHS) is an idiosyncratic and life-threatening adverse drug reaction characterized by skin rash and multiorgan involvement. In rare cases, fulminant type1 diabetes mellitus (DM) may develop after DHS. Among proposed pathogenesis, human herpesvirus 6 (HHV-6) infections may play a role in the development of DHS. We report a case of DHS associated with HHV-6 reactivation, complicated with a rare sequela of irreversible fulminant type 1 DM. No diabetes-related autoantibodies were detected. Early detection and intervention for this serious complication should be given in patients with DHS. Fulminant type1 DM associated with DHS is reviewed. The role of HHV-6 in DHS associated with fulminant type 1 DM is also discussed.

Linear allele-specific long-range amplification: a novel method of long-range molecular haplotyping.

Haplotypes have been repeatedly shown to be more powerful than collections of single-locus markers in gene-mapping studies. Various haplotyping methods including statistical estimation are employed but molecular haplotyping, the acquisition of information directly on physical DNA sequences, has been in demand for its accuracy and independence from family pedigrees. We investigated the allelic specificity of long-range PCR, which was successful for long-range haplotyping in recent reports, and found problems of initial mispriming and crossover amplification significantly confounded its application. Based on these observations, we designed a novel method based on linear amplification of a hemizygous DNA segment with a single phosphorothioate-modified oligonucleotide. Our results revealed, with a single nucleotide polymorphism as the discriminative marker, downstream haplotypes of 14-15 kb DNA segment could be confidently scored. With two rounds of the method and five single nucleotide polymorphisms, molecular haplotypes of 29.3 kb spanning the HCR and CDSN genes, two genes associated with the susceptibility of psoriasis, of 11 members, belonging to a CEPH family, were revealed. Clear Mendelian segregation of 35 highly heterozygous SNPs confirmed the accuracy of the method. Problems of low specificity associated with long-range PCR were not observed. The simplicity, along with long-sequence accessibility and feasibility of a single nucleotide difference as the discriminative marker indicated our method holds promise for future gene-mapping studies.

Childhood neutrophilic eccrine hidradenitis: a clinicopathologic and immunohistochemical study of 10 patients.

BACKGROUND: Neutrophilic eccrine hidradenitis (NEH) is occasionally reported in patients who have not received chemotherapy. OBJECTIVE: The purpose of this study was to describe the clinicopathologic features of NEH occurring in healthy children and to investigate the interleukin (IL)-8 expression in the cutaneous lesions. METHODS: Ten children with characteristic histologic features of NEH were collected from the Chang Gung Memorial Hospital. Their formalin-fixed, paraffin-embedded specimens were examined by immunohistochemical staining for IL-8. RESULTS: The age of first presentation at our clinic ranged from 6 months to 14 months with a median age of 9.1 months. The onset of the disease clustered in the summertime. The most common clinical appearance was multiple erythematous papules and nodules on the limbs. Two of 7 biopsy specimens grew coagulase-negative Staphylococcus . None of patients had underlying systemic disease and all had complete resolution of the lesions within 3 weeks. Immunohistochemical staining for IL-8 was negative in the 10 cases studied. CONCLUSION: Childhood NEH appears as urticaria-like erythematous nodules and plaques on the limbs, trunk, or scalp. This benign and limited disease occurs with a predilection for summer months. In our study, onset was in children less than 15 months of age. IL-8 was not detected in the cutaneous lesions.

A bullous neutrophilic dermatosis in a patient with severe rheumatoid arthritis and monoclonal IgA gammopathy.

A 35-year-old man presented with a 2-month history of itchy grouped vesiculobullae with crusted papules and confluent plaques, distributed symmetrically on the thighs, knees, and elbows. The man had experienced severe disabling seropositive rheumatoid arthritis for 18 years previously. Histologically, subepidermal vesicles were noted, and a dense, diffuse neutrophilic infiltration of the dermis. Direct immunofluorescence studies did not detect IgA deposits. The patient responded well to dapsone (100 mg/d) but not to systemic steroids. This patient's eruption might have been an unusual manifestation of rheumatoid neutrophilic dermatitis.

Distant cutaneous metastases of cholangiocarcinoma: report of two cases of a previously unreported condition.

Cutaneous metastases develop in 2% to 10% of patients with internal malignancies. Here we present two cases of bile duct carcinoma with distant cutaneous metastases, a condition previously unreported. Scalp tumors were the initial presentation in both cases.

Clinical and histopathologic spectrum of cutaneous Rosai-Dorfman disease in Taiwan.

BACKGROUND: Cutaneous Rosai-Dorfman disease (RDD) is a rare entity of unknown origin and is not well documented, especially in Asian populations. OBJECTIVE: The purpose of this study was to evaluate the clinical manifestation, diagnostic histopathology, clinical course, and response to treatment of cutaneous RDD in Taiwan. MATERIALS AND METHODS: This study included 21 patients with cutaneous RDD who presented at our institution from 1995 to 2003. Pathologic examinations with both hematoxylin-eosin and immunohistochemical stains were reviewed, as were associated clinical features and therapeutic methods. RESULTS: None of the 21 patients with cutaneous RDD had nodal lesions. The clinical manifestation was variable, but most commonly involved a central noduloplaque with satellite papules. One patient manifested as an ulcerated nodule, something not reported previously. Multifocal involvement only occurred in 4 patients. Concurrent involvement of uvea or vocal cord occurred in two patients. The most prominent histologic feature was a florid and mixed inflammatory infiltration. The phagocytosis of inflammatory cells into the cytoplasm of histiocytes, a process called "emperipolesis," is a characteristic finding of nodular RDD but usually only focally presented in cutaneous ones. Positivity for S-100 protein helped to confirm the diagnosis. The most effective treatment was surgical excision of solitary lesions. High-dose thalidomide (300 mg/d), but not low-dose, was effective to control the extensive cutaneous diseases in two patients. A total of 3 patients experienced spontaneous remission 1 to 2 years after diagnosis. CONCLUSIONS: Cutaneous RDD appeared more frequently in Asian populations than in reports from Western countries. The incidence of multifocal involvement in this series is much lower than in other literature. Although treatment of disseminated cutaneous RDD is difficult, high-dose thalidomide (300 mg/d), which was effective in two patients in this series, may be helpful.

Annular pustules in Kawasaki disease: a further case indicating the association with psoriasis?

We report the case of a 4-year-old girl with Kawasaki disease (KD), or mucocutaneous lymph node syndrome, who presented with an annular pustular eruption. Targetlike erythematous and scaly patches were observed after resolution of the pustules. A biopsy of the skin was performed, and results showed spongy pustules not associated with the intraepidermal eccrine duct. Generalized pustular eruption, including pustular psoriatic lesions, has been described in KD. However, to our knowledge, this is the first report of annular pustular eruption mimicking annular pustular psoriasis in KD.

Urticarial vasculitis and dermatomyositis in a patient with nasopharyngeal carcinoma.

Urticarial vasculitis has rarely been described in association with polymyositis. We report the case of a 37-year-old man with dermatomyositis and nasopharyngeal carcinoma who presented initially with urticarial vasculitis. The lesions of urticarial vasculitis were initially photodistributed, indicating photosensitivity. The patient was treated with systemic steroids, chemotherapy (cisplatin and fluorouracil), and radiation therapy. The tumor and urticarial vasculitis completely resolved, and the myositis improved.