Cutaneous collagenous vasculopathy (CCV) is an extremely rare acquired microangiopathy of unknown etiology. The authors describe a case of a 68-year-old man, a carrier of a heterozygous pathogenic variant of the glucocerebrosidase (GBA) gene, who was diagnosed with CCV, revealing uncommon fibrinogen positivity in direct immunofluorescence. The patient was subsequently diagnosed with multiple myeloma. Treatment of the myeloma with combined chemotherapy including bortezomib, followed by autologous stem cell transplantation, led to significant reduction of cutaneous lesions. To the best of the authors' knowledge, this is the first published case of CCV in a carrier of a pathogenic variant of the GBA gene, associated with multiple myeloma and with significant regression of CCV after myeloma treatment. Direct immunofluorescence examination revealed an unusual fibrinogen deposition. Hypothetical causative role of bortezomib treatment was proposed regarding significant regression of CCV.

• MeSH
• autologní transplantace MeSH
• bortezomib terapeutické užití   MeSH
• fibrinogen terapeutické užití   MeSH
• glukosylceramidasa terapeutické užití   MeSH
• kožní cévní nemoci * patologie   MeSH
• lidé MeSH
• mnohočetný myelom * komplikace   farmakoterapie   genetika   MeSH
• protokoly antitumorózní kombinované chemoterapie terapeutické užití   MeSH
• senioři MeSH
• teleangiektazie * patologie   MeSH
• transplantace hematopoetických kmenových buněk * MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• Publikační typ
• kazuistiky MeSH

Lichen aureus is a variant of pigmented purpuric dermatoses. The usual histopathology of lichen aureus is characterized by a subepidermal dense, band-like lymphocytic infiltrate, extravasated erythrocytes, and hemosiderin deposits. We report three patients with lichen aureus on the extremities with similar clinical, dermoscopic, and histopathological findings characterized by a dense band-like relatively deep dermal infiltrate accompanied by extravasation of erythrocytes and hemosiderin deposits occasioning a resemblance to a lymphoproliferative disorder.

• MeSH
• dermatoskopie metody   MeSH
• diferenciální diagnóza MeSH
• dospělí MeSH
• erytrocyty patologie   MeSH
• hemosiderin analýza   MeSH
• imunohistochemie metody   MeSH
• kožní nemoci patologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• lymfocyty patologie   MeSH
• lymfoproliferativní nemoci metabolismus   patologie   MeSH
• plazmatické buňky patologie   MeSH
• poruchy pigmentace patologie   MeSH
• pseudolymfom komplikace   patologie   MeSH
• purpura diagnóza   patologie   MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• kazuistiky MeSH

BACKGROUND: The plaque variant of trichoblastoma has been described as a solitary tumor with diffuse infiltration of the lower dermis and hypodermis, with poorly defined borders. Herein, we report a new variant of multiple centrofacial trichoblastoma. OBJECT: To describe clinical and pathological features of a new multiple kind of plaque variant of centrofacial trichoblastoma. METHODS: Case series of patients with a multiple-plaque variant of centrofacial trichoblastoma treated in our department between 2005 and 2017. We identified eight patients with the centrofacial plaque variant of trichoblastoma treated in our department from 2005 to 2017. RESULTS: The final study sample comprised 13 trichoblastomas from four patients. All patients also developed at least one basal cell carcinoma. Mohs surgery was the method of treatment in the majority of the cases of trichoblastoma and in all the cases of basal cell carcinoma. We needed between 2 and 6 stages to obtain free margins in our cases of facial plaque trichoblastomas treated by Mohs surgery. CONCLUSION: To the best of our knowledge, a multiple-plaque variant of trichoblastoma has not been described in the literature. We suggest a genetic origin of this variant of trichoblastoma and describe its remarkable infiltrative nature, with poorly defined surgical margins.

• MeSH
• lidé středního věku MeSH
• lidé MeSH
• Mohsova operace MeSH
• nádory kůže patologie   chirurgie   MeSH
• nemoci vlasů patologie   chirurgie   MeSH
• obličej patologie   MeSH
• senioři MeSH
• vlasový folikul patologie   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• kazuistiky MeSH

We present the case of a 35-year-old man who developed a follicular differentiated cutaneous carcinoma with an eosinophil-rich infiltrate and an aggressive clinical behavior. After an in-depth histopathological investigation the diagnosis of trichoblastic carcinoma was made. Over the course of the disease the patient developed a cutaneous in-transit metastasis as well as an axillary lymph node metastasis 18 months after the excision of the primary tumor on his back. Based on a literature review we discuss the different concepts behind the term "trichoblastic carcinoma" and we summarize the clinical and histological details of previously reported cases. Furthermore, we focus on the phenomenon of tumor-associated eosinophilia.

• MeSH
• dospělí MeSH
• karcinom patologie   MeSH
• lidé MeSH
• lokální recidiva nádoru patologie   MeSH
• nádory kůže patologie   MeSH
• nemoci vlasů patologie   MeSH
• vlasový folikul patologie   MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• kazuistiky MeSH

BACKGROUND: We present herein a series of 14 lesions showing overlapping features with the newly defined benign cutaneous mesenchymal neoplasm labeled as fibroblastic connective tissue nevus (FCTN). METHODS AND RESULTS: Total of 8 patients were male and 5 were female, ranging in age from 1 to 56 years. Lesions appeared as isolated nodules or plaques on the trunk (7 cases), the limbs (4 cases) and the neck (2 cases). Histologically, all cases were composed of bundles of bland spindle cells of fibroblastic/myofibroblastic lineage irregularly branching within the reticular dermis and along fibrous septa in the subcutis. Adnexal structures and dermal adipocytes were entrapped by the fascicles, the epidermis was often papillomatous and elastic fibers were decreased and fragmented. Expression of CD34 and ASMA was found in 8 and 7 cases, respectively. Follow-up was available for 7 patients (mean follow-up, 5 years; range, 1-10 years). None of the cases metastasized or recurred, even when incompletely excised. CONCLUSION: The differential diagnosis of FCTN is broad and includes hypertrophic scar, dermatofibroma, dermatomyofibroma, pilar leiomyoma, plaque-stage DFSP, CD34-positive plaque-like dermal fibroma, fibroblastic-predominant plexiform fibrohistiocytic tumor, lipofibromatosis, superficial desmoid fibromatosis and fibrous hamartoma of infancy, of which it represents probably the monophasic variant.

• MeSH
• antigeny CD34 metabolismus   MeSH
• dítě MeSH
• dospělí MeSH
• intradermální névus * metabolismus   patologie   MeSH
• keratinocyty metabolismus   patologie   MeSH
• kojenec MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• myofibroblasty * metabolismus   patologie   MeSH
• nádorové proteiny metabolismus   MeSH
• nádory kůže * metabolismus   patologie   MeSH
• předškolní dítě MeSH
• škára * metabolismus   patologie   MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• kojenec MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

Desmoplastic melanoma (DM) is histologically characterized by a proliferation of spindle melanocytes dispersed in a collagenous stroma that can be mistaken for a variety of neoplasms. The purpose of this study was to analyze 40 cases of DM with a comprehensive panel of immunohistochemical markers (KBA.62, p16, Ezrin, WT-1, MITF-1, SOX-10, CD117, SOX-2, nestin, PNL2, p75, MART-1, gp100 and S100p) to obtain a more complete understanding of the potential use of these antibodies in the diagnosis of DM. We found that all cases of DM expressed p16, WT-1, SOX-10, nestin and S100p and 95% of cases expressed p75. There was variable expression with Ezrin, SOX-2, KBA.62, MART-1 and HMB-45. Most DMs did not express MITF-1, PNL2 and CD117. Conditions that may enter in the histologic differential diagnosis of DM, including dermal scars, fibromatosis and dermatofibromas were also studied. Nearly all control cases also stained positive for p16 but were negative for WT1, SOX10, nestin, p75 and S-100p, as well as for most of the other markers tested. We conclude that a panel of S-100p, WT1, SOX10, p75 and nestin may constitute the optimal panel with the most sensitive and specific combination of immunostain available for the diagnosis of DM.

• MeSH
• barvení a značení metody   MeSH
• dospělí MeSH
• imunohistochemie MeSH
• lidé středního věku MeSH
• lidé MeSH
• melanom metabolismus   patologie   MeSH
• mladiství MeSH
• nádorové proteiny metabolismus   MeSH
• nádory kůže metabolismus   patologie   MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

We present six cases (five females, one male; aged 26-81 years) of dacryops, also known as lacrimal gland cyst, all of which occurred at the outer canthal area below the upper eyelid. All presented clinically as a painless cystic lesion that was white to blue in color. Microscopically, in addition to typical features of dacryops, which is characterized by a partially cystic proliferation that includes a double layer of columnar to cuboidal epithelial cells associated with lobules of lacrimal gland tissue, we identified evidence of apocrine secretion (i.e. apical snouts projecting into the lumen), either in the cystic component of the proliferation or in contiguous lacrimal duct, in all cases. One example was unusual. It manifested, in addition to typical cyst formation, with areas of ductal and probably acinar hyperplasia. We conclude that apocrine secretion in dacryops is a common and underrecognized phenomenon. Dacryops should be distinguished from apocrine hidrocystoma, a lesion commonly encountered in the periorbital area in the practice of dermatopathology.

• MeSH
• cysty patologie   sekrece   MeSH
• dospělí MeSH
• hlen sekrece   MeSH
• lidé středního věku MeSH
• lidé MeSH
• nemoci slzného ústrojí metabolismus   patologie   MeSH
• pohárkové buňky patologie   sekrece   MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• slzné ústrojí patologie   sekrece   MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

The authors report a case of Brooke-Spiegler syndrome (BSS) with a novel germline CYLD mutation and various somatic mutations identified in the lesional tissues. The patient was a 46-year-old man with multiple lesions on the face. The available histopathological material included 24 trichoepitheliomas, 2 large nodular basal cell carcinomas (BCCs), 2 spiradenomas, 1 spiradenocylindroma and 1 trichoblastoma composed of large and small nodules with prominent clear cell differentiation. Whereas one of the two BCCs manifested a conventional morphology, the second neoplasm additionally showed foci with high grade cytological features characterized by marked pleomorphism and numerous mitotic figures. There were also numerous signet ring cells and cells containing intracytoplasmic eosinophilic inclusions. The germline mutation was a substitution mutation c.1684 + 1G> A. Somatic mutations were investigated in eight tissue blocks from which high quality genomic DNA had been successfully extracted. Somatic mutations included loss of heterozygosity (LOH) in four lesions and a single sequence mutation, namely a single base deletion c. 2322delA causing a frameshift mutation E774DfsX2. LOH occurred in both BCCs, one trichoepithelioma and one spiradenoma. In the remaining three lesions, the somatic event remained undetected.

• MeSH
• adenoidně cystický karcinom genetika   patologie   MeSH
• adenom genetika   patologie   MeSH
• bazocelulární karcinom genetika   patologie   MeSH
• karcinom kožních adnex genetika   patologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• mutace MeSH
• nádorové supresorové proteiny genetika   MeSH
• nádory kůže genetika   patologie   MeSH
• nádory obličeje genetika   patologie   MeSH
• rodokmen MeSH
• syndrom MeSH
• ztráta heterozygozity MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• práce podpořená grantem MeSH

BACKGROUND: Nestin is one of the intermediate filaments that are expressed in proliferating neural progenitor cells during development of the central nervous system (CNS) and peripheral nervous system. Postnatal re-expression of the protein occurs mainly under pathological conditions, including injury and neoplasia. In this study, nestin expression was detected in both benign and malignant melanocytic skin lesions and its diagnostic relevance was then evaluated. METHODS: Altogether 139 bioptic tissue samples consisting of 42 nodular melanomas, 32 superficial spreading melanomas, 12 metastatic melanomas, 10 dysplastic nevi and 43 common melanocytic intradermal and dermoepidermal nevi were analysed using indirect immunohistochemical staining. RESULTS: We demonstrated that nestin immunostaining was significantly increased in melanomas where it correlated with more advanced stages of the disease. CONCLUSION: We conclude that expression of the intermediate filament protein nestin might be an indicator of tumor dedifferentiation and more aggressive behaviour. Furthermore, we suggest that nestin might be a relevant marker of tumorous and non-tumorous angiogenesis.

• MeSH
• financování organizované MeSH
• imunohistochemie MeSH
• lidé MeSH
• melanom krevní zásobení   metabolismus   MeSH
• nádorové biomarkery analýza   MeSH
• nádory kůže krevní zásobení   metabolismus   MeSH
• patologická angiogeneze metabolismus   MeSH
• pigmentový névus krevní zásobení   metabolismus   MeSH
• proteiny intermediálních filament biosyntéza   MeSH
• proteiny nervové tkáně biosyntéza   MeSH
• Check Tag
• lidé MeSH

BACKGROUND: Cutaneous lymphomas co-expressing CD56 and CD30 are very rare. They share a clinicopathological overlap with natural killer- (NK)/T-cell lymphomas and anaplastic large-cell lymphomas (ALCLs), two entities with widely disparate clinical behavior. METHODS: We present a case of an immunocompetent 57-year-old Caucasian woman with a rapidly growing, angiodestructive and neuroinvasive primary cutaneous ALCL (PCALCL). The neoplastic population of large anaplastic CD30+ and CD56+ T cells was masked by a massive admixture of histiocytes and neutrophils. The partially ulcerated and pus-secreting tumor involved the forehead and scalp and was assessed as clinical stage IAE. RESULTS: After chemotherapy (cyclophosphamide, doxorubicin, vincristine, and prednisone), the patient achieved a complete remission. Additionally, high-dose chemotherapy with autologous peripheral blood stem-cell transplantation was administered as a consolidation of complete remission, in which she has remained for 6 years. CONCLUSIONS: This is the first CD30+ and CD56+ primary skin lymphoma to be reported on the head. The presented case carries a remarkable combination of clinicopathological features of PCALCL and NK-/T-cell lymphoma.

• MeSH
• antigen Ki-1 analýza   MeSH
• antigeny CD56 analýza   MeSH
• čelo MeSH
• difúzní velkobuněčný B-lymfom farmakoterapie   imunologie   patologie   MeSH
• financování organizované MeSH
• histiocyty cytologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• nádory hlavy a krku farmakoterapie   imunologie   krevní zásobení   patologie   MeSH
• nádory kůže farmakoterapie   imunologie   krevní zásobení   patologie   MeSH
• neutrofily cytologie   MeSH
• skalp inervace   krevní zásobení   patologie   účinky léků   MeSH
• transplantace kmenových buněk MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• ženské pohlaví MeSH