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ABSTRACT: Background Most melanomas are first recognized by patients themselves or by their friends and family. Objectives To assess the ability of laypersons to identify melanomas using dermoscopy images. Methods This is an image-based study using laptop computers in the community. Seventeen laypersons were given a one-page educational brochure on the AC Rule for melanoma (asymmetry, colour variation). These laypersons and three expert dermoscopists completed two image sets, each containing a series of 100 pigmented skin lesions. Set 1 contained five melanomas, while set 2 contained 20 melanomas. Participants viewed a clinical image followed by a dermoscopy image for each lesion. For each image a score of 0-10 was assigned for asymmetry and colour, and then an overall assessment was made for suspicion of melanoma. Mean estimates have been calculated for sensitivity and specificity. Results Laypersons achieved a clinical sensitivity of 91·2% and a significantly higher dermoscopy sensitivity of 94·0%, P = 0·013. This improvement was not associated with a significant change in overall specificity, which for the clinical image was 64·2% and with dermoscopy was 62·0%, P = 0·97. Conclusions These results indicate that laypersons may be able to use dermoscopy to identify more melanomas than naked eye examination alone. Further study into the practice of dermoscopy by laypersons is warranted.
British Journal of Dermatology 07/2012; · 3.67 Impact Factor
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ABSTRACT: Several clinical and histopathologic variants of mycosis fungoides (MF) have been well described, including the often elusive interstitial MF. Differentiation from other inflammatory disorders, such as interstitial granuloma annulare (GA) and inflammatory morphea, may be extremely difficult. We report a case of MF and GA coexisting in a 54-year-old woman who initially presented to clinic in 2000 with slightly scaly patches on the trunk and extremities, histopathologically diagnostic of MF. A second biopsy taken a few months later revealed an interstitial infiltrate that was initially interpreted as interstitial MF. Over the following 10 years, additional biopsies revealed features of conventional MF. In 2009, a new biopsy showed unequivocal features of interstitial GA. Reevaluation of the original biopsy, diagnostic of "interstitial MF," revealed that this, too, could be better classified as interstitial GA than interstitial MF. Our case illustrates that MF and interstitial GA may coexist simultaneously, thus representing a pitfall in the histopathologic diagnosis of MF. Given the similarities in clinicopathologic presentation, dermatologists and dermatopathologists should be cautious not to inadvertently misinterpret GA as interstitial MF.
The American Journal of dermatopathology 01/2012; 34(2):198-202. · 1.30 Impact Factor
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ABSTRACT: Extranodal natural killer (NK)/T-cell lymphoma, nasal type, is a rare type of cytotoxic lymphoma involving mainly the upper aerodigestive tract and associated with Epstein-Barr virus. The disease has usually a poor prognosis related to several factors. The skin is the second most common affected organ, and cases may be localized to the skin only without any other extracutaneous manifestations. Although primary cutaneous cases may have a better prognosis, survival usually is still poor. We report a case of primary cutaneous extranodal NK/T-cell lymphoma, nasal type, in a 77-year-old woman with an indolent course for more than 22 years and still limited to the skin.
The American Journal of dermatopathology 12/2011; 34(2):194-7. · 1.30 Impact Factor
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Journal of the American Academy of Dermatology 12/2011; 65(6):1233-4. · 3.99 Impact Factor
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ABSTRACT: We report on a 69-year-old female patient with specific cutaneous manifestations of B-cell chronic lymphocytic leukemia that arose at the site of erythema chronicum migrans due to Borrelia burgdorferi infection. Histological examination revealed the presence of dense infiltrates of small hyperchromatic lymphocytes admixed with clusters of plasma cells. Immunohistology showed a CD5+/CD20+ phenotype of the lymphocytes and monoclonal expression of kappa immunoglobulin light chain by the plasma cells. Presence of Borrelia DNA was confirmed by polymerase chain reaction studies. The unusual histopathological and phenotypic findings described in this case of cutaneous manifestations of B-cell chronic lymphocytic leukemia associated with Borrelia burgdorferi infection may lead to the misdiagnosis of cutaneous marginal zone B-cell lymphoma.
The American Journal of dermatopathology 10/2011; 33(7):712-5. · 1.30 Impact Factor
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ABSTRACT: Data on early lesions of primary cutaneous follicle center lymphoma (PCFCL), diffuse type are very limited.
We sought to elucidate the early clinicopathologic features of PCFCL, diffuse type.
Clinical, histologic, immunohistologic, molecular, and fluorescence in situ hybridization data from 24 patients with early lesions of PCFCL, diffuse type (male:female = 19:5; median age: 57 years) were determined.
Lesions consisted mostly of solitary or clustered papules and small nodules located on the trunk (21 cases), arm (two cases), and scalp (one case). In 3 patients small papules were located at a distance from the main affected area. All biopsy specimens from early lesions showed aggregates of medium and large centrocytes admixed with small lymphocytes without formation of clear-cut lymph follicles. Staining for Bcl-2 was positive in only 7 cases, one revealing also a rearranged BCL2 signal by fluorescence in situ hybridization. Data on treatment and follow-up were available for 22 patients. At last examination 13 patients were in complete remission (median follow-up: 60 months), 6 were alive with skin disease alone (median follow-up: 60 months), two were alive with skin disease and bone-marrow or lymph node involvement, respectively, and one died of unrelated causes while in complete remission.
The retrospective study and the fact that patients were treated at different institutions are limitations.
Early lesions of PCFCL, diffuse type present with characteristic clinicopathologic features. Dermatologists should be alert particularly to the early clinical manifestations of this lymphoma and to the presence of small, inconspicuous lesions at a distance from the main affected area in order to plan treatment properly.
Journal of the American Academy of Dermatology 06/2011; 65(5):991-1000. · 3.99 Impact Factor
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Michaela Auer-Grumbach,
Martin Weger, Regina Fink-Puches,
Lea Papić,
Eleonore Fröhlich,
Piet Auer-Grumbach,
Laila El Shabrawi-Caelen,
Maria Schabhüttl,
Christian Windpassinger,
Jan Senderek,
Herbert Budka,
Slave Trajanoski,
Andreas R Janecke,
Anton Haas,
Dieter Metze,
Thomas R Pieber,
Christian Guelly
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ABSTRACT: To identify the disease-causing gene responsible for an autosomal dominantly inherited Charcot-Marie-Tooth neuropathy subtype in a family excluded for mutations in the common Charcot-Marie-Tooth genes, we used array-based sequence capture to simultaneously analyse the disease-linked protein coding exome at chromosome 14q32. A missense mutation in fibulin-5, encoding a widely expressed constituent of the extracellular matrix that has an essential role in elastic fibre assembly and has been shown to cause cutis laxa, was detected as the only novel non-synonymous sequence variant within the disease interval. Screening of 112 index probands with unclassified Charcot-Marie-Tooth neuropathies detected two further fibulin-5 missense mutations in two families with Charcot-Marie-Tooth disease and hyperextensible skin. Since fibulin-5 mutations have been described in patients with age-related macular degeneration, an additional 300 probands with exudative age-related macular degeneration were included in this study. Two further fibulin-5 missense mutations were identified in six patients. A mild to severe peripheral neuropathy was detected in the majority of patients with age-related macular degeneration carrying mutations in fibulin-5. This study identifies fibulin-5 as a gene involved in Charcot-Marie-Tooth neuropathies and reveals heterozygous fibulin-5 mutations in 2% of our patients with age-related macular degeneration. Furthermore, it adumbrates a new syndrome by linking concurrent pathologic alterations affecting peripheral nerves, eyes and skin to mutations in the fibulin-5 gene.
Brain 06/2011; 134(Pt 6):1839-52. · 9.46 Impact Factor
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ABSTRACT: Primary cutaneous follicle center lymphoma (PCFCL) presents usually with reddish nodules, plaques, and tumors on the head and neck area, particularly the scalp, and on the back.
We sought to describe a peculiar clinical variant of PCFCL.
We report a series of 18 patients (male:female = 7:11; median age 52 years; mean age 50.8 years; age range 27-75 years) with a clinical variant of PCFCL characterized clinically by multiple, miliary, or agminated papules predominantly on the head and neck.
All patients presented with multiple erythematous, firm papules arranged in a manner that resembled millet seeds or collected together in small clusters. Lesions were located on the entire face in one patient (5.6%), the forehead in 8 (44.4%), the cheeks in 3 (16.7%), the preauricular region in two (11.1%), and multiple regions on the head and neck area in 3 (16.7%). The last patient had miliary papules on the entire face, back, upper aspect of arms, and scattered on the front of the chest. The initial diagnosis was never cutaneous lymphoma, and all patients had been treated unsuccessfully for different skin conditions including mainly rosacea, lupus miliaris disseminatus faciei, and persistent arthropod bite reaction. Microscopic examination confirmed the diagnosis of PCFCL in all patients.
Small number of cases and retrospective study are limitations.
This peculiar clinical presentation of PCFCL is unusual and represents a pitfall in the clinical diagnosis. Dermatologists should be aware of this variant of PCFCL so as to treat patients timely and properly.
Journal of the American Academy of Dermatology 05/2011; 65(4):749-55. · 3.99 Impact Factor
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The Lancet 01/2011; 377(9760):178. · 38.28 Impact Factor
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ABSTRACT: A rare variant of mycosis fungoides (MF) characterized by prominent involvement of the eccrine glands with syringometaplasia has been reported in the past as "syringolymphoid hyperplasia with alopecia," "syringotropic cutaneous T-cell lymphoma," "adnexotropic T-cell lymphoma," or "syringotropic MF." The clinicopathologic features of this variant are not well understood, and only a few case reports or small series have been published to date. We reviewed the clinicopathologic features of 14 patients with syringotropic MF (male:female=10:4; median age, 59 years; mean age, 57.8; age range, 33 to 83 y). Six patients had variably large, solitary patches or plaques, located on the thigh (n=3), arm, trunk, or eyebrow (1 each). The other 8 patients had multiple, mostly generalized lesions. A history of MF was known in 4 of these 8 patients. With the exception of 1 biopsy specimen that was too superficial and did include the eccrine secretory coils but not the eccrine glands, all cases showed prominent involvement of the eccrine glands. Variable degrees of syringometaplasia ranging from small to large epithelial complexes were present in all specimens. The eccrine glands and syringometaplastic structures were surrounded by dense lymphoid infiltrates with prominent epitheliotropism. Concomitant involvement of the epidermis and of the hair follicles was observed in 13 and 8 biopsies, respectively. This is the largest series of syringotropic MF, showing that this is a rare variant of the disease with peculiar clinicopathologic features. Dermatologists and dermatopathologists should be aware of this rare variant of MF to avoid delayed diagnosis and treatment.
The American journal of surgical pathology 01/2011; 35(1):100-9. · 4.06 Impact Factor
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ABSTRACT: Lymphomatoid papulosis (LyP) is a recurrent, self-healing eruption belonging to the spectrum of cutaneous CD30+lymphoproliferative disorders. Three main histologic subtypes of LyP are recognized: type A (histiocytic), type B (mycosis fungoides-(MF)-like), and type C (anaplastic large cell lymphoma-like). We reviewed 26 biopsies from 9 patients (M:F=6:3, median age: 29; mean age 27,2; age range 10 to 38) who presented with clinical features typical of LyP but with histopathologic aspects that resembled primary cutaneous aggressive epidermotropic CD8+cytotoxic T-cell lymphoma. In all but 1 case atypical lymphoid cells showed expression of CD30, and in 8 of 9 cases a T-cell cytotoxic phenotype could be observed (betaF1+, CD3+, CD4-, CD8+). Expression of at least 1 cytotoxic marker (TIA-1, granzyme B) was observed in all cases. Polymerase chain reaction analysis of the T-cell receptor genes revealed a monoclonal rearrangement in 2 of 5 cases tested. Follow-up data available for 8 patients (mean follow-up time: 84 mo, median: 32.5 mo; range: 1 to 303 mo) revealed that none of them developed systemic involvement or signs of other cutaneous lymphomas. This cytotoxic variant of LyP may be histopathologically indistinguishable from primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma, and may be the source of pitfalls in the diagnosis and classification. We propose the term LyP type D for this unusual variant of the disease. Accurate clinicopathologic correlation is required in this setting, with crucial implications regarding prognosis and management of patients.
The American journal of surgical pathology 08/2010; 34(8):1168-75. · 4.06 Impact Factor
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ABSTRACT: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a neoplasm derived from precursors of plasmacytoid dendritic cells. Cutaneous involvement represents often the first manifestation of the disease. We studied 45 skin biopsies from 33 patients (M:F=7.25:1; median age: 71 y; age range: 30 to 89) with BPDCN to delineate histopathologic and immunophenotypic features of this disease. Patients presented with generalized (n=18), localized (n=6), or solitary (n=9) macules, plaques, and/or tumors. Staging investigations at presentation were negative in 20 patients. Unusual histologic features included a perivascular/periadnexal pattern (6 biopsies from 4 patients) and the presence of pleomorphism of neoplastic cells with blastoid cells admixed with elongated, twisted, or hyperchromatic cells (observed in 24 specimens). Negativity of 1 among the 4 markers CD4, CD56, CD123, and TCL-1 was seen in 11 biopsies, and of 2 markers in 4 biopsies. Staining for CD68 revealed positivity of the majority of cells in 1 and of scattered cells in 24/37 stained cases. Terminal deoxynucleotidyl transferase was observed in 22/37 stained cases. Staining for Bcl-6, MUM-1 and FOX-P1 revealed positivity of a variable proportion of neoplastic cells in 16/30, 19/29, and 21/23 cases, respectively. Our study shows that cutaneous lesions of BPDCN display a greater variability of morphologic and phenotypic features than recognized earlier. Discrete perivascular infiltrates, pleomorphic morphology of neoplastic cells, and unusual phenotypic profiles may be the source of diagnostic pitfalls. These atypical variants should be recognized to make an early diagnosis and to manage properly patients with this aggressive hematological disorder.
The American journal of surgical pathology 12/2009; 34(1):75-87. · 4.06 Impact Factor
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ABSTRACT: BACKGROUND: A possible association between lichen aureus (LA) and mycosis fungoides (MF) has been suggested in the past. We evaluated the clinicopathologic features of LA and its relationship to MF. Data from 23 patients with a clinicopathologic diagnosis of LA were reviewed. OBSERVATIONS: Lesions were asymmetrically localized on 1 area of the body (mostly 1 extremity) and were characterized histologically by dense, bandlike lymphocytic infiltrates. A monoclonal T-cell population was detected in half of the cases. After a mean follow-up of 102.1 months, 14 patients had no sign of skin disease, 7 patients had unmodified skin lesions, and 2 other patients with unmodified skin lesions had died of unrelated conditions. Treatment modalities did not affect the outcome. There was no relationship between the presence or absence of monoclonality and patient status at follow-up assessments. Conclusion Patients with classic lesions of LA do not show progression to MF.
Archives of dermatology 10/2008; 144(9):1169-73. · 4.76 Impact Factor
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Journal of Investigative Dermatology 09/2008; 129(1):238-40. · 6.31 Impact Factor
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Iris Zalaudek,
Harald Kittler,
Ashfaq A Marghoob,
Anna Balato,
Andreas Blum,
Stéphane Dalle,
Gerardo Ferrara, Regina Fink-Puches,
Caterina M Giorgio,
Rainer Hofmann-Wellenhof,
Josep Malvehy,
Elvira Moscarella,
Susana Puig,
Massimiliano Scalvenzi,
Luc Thomas,
Giuseppe Argenziano
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ABSTRACT: To determine the time required to perform a complete skin examination (CSE) as a means of opportunistic screening for skin cancer both without and with dermoscopy.
Randomized, prospective multicenter study.
Eight referral pigmented lesion clinics. Patients From June 2006 to January 2007, 1359 patients with at least 1 melanocytic or nonmelanocytic skin lesion were randomly selected to receive a CSE without dermoscopy or CSE with dermoscopy. For each patient, the total number of lesions and the duration of the CSE were recorded. A total of 1328 patients were eligible for analysis (31 were excluded because of missing data).
The median time (measured in seconds) needed for CSE with and without dermoscopy and according to total cutaneous lesion count.
The median time needed for CSE without dermoscopy was 70 seconds and with dermoscopy was 142 seconds, a significant difference of 72 seconds (P < .001). The use of dermoscopy increased the duration of CSE, and this increase was in direct proportion to the patient's total lesion count. In contrast, the time required to perform a CSE without dermoscopy remained the same irrespective of whether the patients had few or many lesions.
A CSE aided by dermoscopy takes significantly longer than a CSE without dermoscopy. However, a thorough CSE, with or without dermoscopy, requires less than 3 minutes, which is a reasonable amount of added time to potentially prevent the morbidity and mortality associated with skin cancer.
Archives of dermatology 04/2008; 144(4):509-13. · 4.76 Impact Factor
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ABSTRACT: Halo nevi (HN) are benign melanocytic nevi surrounded by a depigmented area (halo). This study aims to evaluate the dermoscopic features of HN and their changes during digital dermoscopic follow-up and to investigate the frequency of the halo phenomenon in a series of melanomas.
In a retrospective study, digital dermoscopic images of HN from patients who attended the Pigmented Skin Lesions Clinic of the Department of Dermatology, Medical University of Graz, between October 1, 1997, and March 31, 2004, were reviewed and classified by dermoscopic morphologic criteria. For HN that were followed up with digital dermoscopy, the percentages of changes in the size of the nevus and halo components were calculated. In addition, digital dermoscopic images of histopathologically confirmed melanomas obtained from the same database were reviewed for the presence of an encircling halolike depigmentation. We classified 138 HN in 87 patients (mean age, 22.4 years). The most common dermoscopic structures were the globular and/or homogeneous patterns in more than 80% of HN. Follow-up of 33 HN revealed considerable size reduction of the nevus component, but this was not associated with significant structural changes. Of a total of 475 melanomas, only 2 revealed an encircling halo, but both displayed clear-cut melanoma-specific patterns according to dermoscopy.
Halo nevi exhibit the characteristic dermoscopic features of benign melanocytic nevi, represented by globular and/or homogeneous patterns that are typically observed in children and young adults. Halo nevi reveal considerable changes of area over time during digital dermoscopic follow-up, albeit their structural patterns remain unchanged. For this reason and because melanoma with halolike depigmentation, despite being rare, additionally exhibits melanoma-specific dermoscopic criteria, the role of digital dermoscopic follow-up in the diagnosis of HN is insignificant.
Archives of Dermatology 01/2007; 142(12):1627-32. · 3.89 Impact Factor
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ABSTRACT: Subcutaneous T-cell lymphoma (STCL) represents a controversial entity and a confused concept in the field of cutaneous T-cell lymphomas (CTCLs). Recently, alpha/beta+/CD8+ STCL has been recognized by the new World Health Organization (WHO)-European Organization for Research and Treatment of Cancer (EORTC) classification of primary cutaneous lymphomas as a distinct entity in the group of CTCLs.
We reviewed a series of 53 biopsies from 26 patients (F : M = 19:7; median age: 48; range 18-87) of cutaneous B- and T-cell lymphomas characterized by prominent involvement of the subcutaneous tissue. We could classify our cases according to the following seven categories--(i) STCL: n = 16; (ii) extranodal NK/T-cell lymphoma, nasal type: n = 2; (iii) cutaneous gamma/delta T-cell lymphoma: n = 2; (iv) anaplastic CD30+ large T-cell lymphoma: n = 1; (v) diffuse large B-cell lymphoma, secondary cutaneous: n = 3; (vi) lymphoplasmacytic lymphoma, secondary cutaneous: n = 1; (vii) specific cutaneous manifestations of myelogenous leukemia: n = 1.
We demonstrated the protean nature of lymphomas with prominent involvement of the subcutaneous fat tissues. The term STCL should be restricted to a homogeneous group of cases characterized morphologically by an exclusive involvement of subcutaneous tissues, immunohistochemically by a T-cytotoxic alpha/beta phenotype, and biologically by a relatively good prognosis.
Journal of Cutaneous Pathology 07/2006; 33(6):418-25. · 1.56 Impact Factor
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Journal der Deutschen Dermatologischen Gesellschaft 10/2005; 3(9):710-20; quiz 721. · 1.47 Impact Factor
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ABSTRACT: Rituximab is a genetically engineered antibody directed against the CD20 antigen. Intravenous administration of rituximab has been used for the treatment of patients with low-, intermediate-, and high-grade B-cell non-Hodgkin's lymphomas and is a registered treatment modality for this indication. Treatment of primary cutaneous B-cell lymphoma (CBCL) with intralesionally or systemically administered rituximab has been described only in a few cases.
Our purpose was to assess the efficacy of rituximab in the treatment of CBCL.
We performed a retrospective study on 9 patients with CBCL who were treated with intralesional or systemic administration of rituximab.
Two patients treated with systemic rituximab achieved complete remission. Complete remission could be observed in 6 of 7 patients after 1 to 8 cycles of intralesional treatment with rituximab. In one patient one of two lesions showed a partial remission after 4 cycles of treatment, whereas the second showed complete remission. A local recurrence was observed in one patient after 27 months of follow-up and in two patients recurrences developed at other body sites after 12 and 14 months of follow-up. No severe side effect occurred except for slight pain during intralesional injection.
Rituximab therapy is a well-tolerated and effective treatment for primary CBCL. In comparison to intravenous administration, intralesional application of the drug allows the use of lower dosages. Intralesional therapy with rituximab deserves further investigation and comparison to systemic administration of the drug in controlled multicenter studies.
Journal of the American Academy of Dermatology 06/2005; 52(5):847-53. · 3.99 Impact Factor
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ABSTRACT: Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma. In early stages of the disease many different clinicopathologic variants have been observed.
We report 6 patients with early manifestations of MF characterized by the sole presence of papules which, unlike the papules of lymphomatoid papulosis, did not show a tendency for spontaneous resolution. Histologic examination confirmed the diagnosis of MF in all cases. Immunohistochemical staining for CD30 was negative in all cases. Follow-up data showed the nonaggressive behavior of the disease, confirming that the lesions were not manifestations of advanced MF.
Papular MF is a new variant of early MF characterized by the presence of papules in the absence of more conventional early lesions (patches) of the disease. This variant should be added to the long list of clinicopathologic subtypes of MF.
Journal of the American Academy of Dermatology 05/2005; 52(4):694-8. · 3.99 Impact Factor
