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Intravascular lymphoma (IL) is a rare variant of non-Hodgkin lymphoma with a predilection for skin. Most reported cases are large B cell lymphomas. Intravascular anaplastic large cell lymphoma (IALCL) is extremely rare. Retrospective analysis of a case of cutaneous IALCL was performed. Hematoxylin and eosin stained sections and immunohistochemical staining results were analyzed. The patient was a 47-year-old woman who had developed multiple erythematous patches and plaques on her back. The lesions responded well to CHOP (cyclophosphamide, hydroxydoxorubicin, oncovin, prednisone) chemotherapy, but relapsed shortly after therapy. The patient was surviving with the disease for eight years but was ultimately lost to follow up. Histopathologically, the neoplasm evolved from IL to extravascular lymphoma. This was showed in biopsies obtained at different stages of the disease. The lymphoma cells stained positively for CD30, CD45, CD3, CD4, CD5 and Ki67, and lacked expression of anaplastic lymphoma kinase (ALK), CD8, CD45RA, CD45RO, CD20, CD79, CD56, perforin and granzyme B. Our results suggest that IALCL represents a distinct subtype of IL and is histopathologically and biologically different from IL with B, NK or T cell phenotype. Wang L, Li C, Gao T. Cutaneous intravascular anaplastic large cell lymphoma.

Systemic cases of the CD30-positive T-cell neoplasm, anaplastic large cell lymphoma (ALCL), are typically anaplastic lymphoma kinase (ALK)-positive. The failure to express ALK protein has been shown to portend a worse prognosis. We describe a case of ALK-negative systemic ALCL that presented as a violaceous plaque on the scalp of a 79-year-old man. Interestingly, the neoplastic cells were confined largely within vascular spaces, a configuration that is exceedingly rare in the skin and is more typically seen with intravascular large B-cell lymphoma. In addition, bcl-2 immunohistochemical staining was strongly positive in this case, which may portend a more aggressive clinical course. To our knowledge, this report represents the first case of an ALK-negative ALCL to present intravascularly in the skin. Therefore, the recognition of systemic anaplastic T-cell lymphoma present within the intravascular spaces is important to avoid misdiagnosis. Rieger K, Polidore T, Warnke R, Kim J. ALK-negative systemic intravascular anaplastic large cell lymphoma presenting in the skin.

We describe a case of a 34-year-old, healthy, lactating female with a 2-month history of breast pain and an enlarging, tender mass on her right nipple. Her right breast was firm and mildly engorged without mass, warmth or erythema. A tender, yellow nodule was located on the superior aspect of the nipple, obstructing the flow of milk from this portion of the nipple. A biopsy showed epidermal erosion, sheets of cells with massively distended, foamy cytoplasm in the dermis, and a hypertrophied and occluded glandular duct, consistent with reactive squamous metaplasia. Immunostaining for CD68 confirmed the foamy cells were macrophages, and anti-human milk fat globulin-1 (HMFG1) labeled the substance within the macrophages consistent with human breast milk. Therefore, the lesion could be identified as a xanthogranulomatous reaction to a ruptured galactocele. Adams EG, Kemp JD, Holcomb KZ, Sperling LC. Xanthogranulomatous reaction to a ruptured galactocele.

Background: Atypical fibroxanthoma (AFX) is a pleomorphic spindle cell lesion of the skin; it is considered in the differential diagnosis with spindle cell malignant melanoma (MM) and sarcomatoid carcinoma/spindle cell squamous cell carcinoma (SCC). An optimum approach has yet to fully emerge with respect to the immunohistochemical discrimination of these lesions. Methods: Departmental archives from 1978 onwards were searched for clinicopathologically confirmed cases of AFX, MM and SCC. Immunostains for CD10, CD99 and p63 were performed in each case. Scored staining results were analyzed using Fisher's Exact Test. Results: Twenty-seven of 31 cases of AFX were positive for CD10, as compared with 3 of 22 SCCs and 0 of 20 MMs. CD10 positivity was preferentially associated with the diagnosis of AFX (p < 0.001). p63 reactivity was observed in 15/22 cases of SCCs, 5/31 AFXs and 1/20 MMs. CD99 reactivity was observed in 3/31 cases of AFX, 2/22 SCCs and 3/20 MMs. Conclusion: CD10 positivity is relatively specific in this context for the diagnosis of AFX. Its utility is enhanced when only strong, diffuse membranocytoplasmic staining is considered as a positive result. In contrast to prior reports, p63 was not found to be highly sensitive for SCC. Similarly, CD99 showed no preferential staining of any single diagnostic group of lesions. Kanner WA, Brill LB, Patterson JW, Wick MR. CD10, p63 and CD99 expression in the differential diagnosis of atypical fibroxanthoma, spindle cell squamous cell carcinoma and desmoplastic melanoma.

Background: Although histopathologic identification of regression of melanoma is usually straightforward, sometimes it can be difficult to distinguish it from scarring fibrosis. Therefore, this study investigates the elastic fiber pattern in melanomas associated with either regression or scars. Methods: We compared 33 invasive melanomas with the fibrosing stage of regression to 10 cases of invasive melanomas with scarring fibrosis. None of the regression cases had a prior surgical procedure. Elastic fiber patterns were evaluated with Verhoeff's elastic van Gieson stain (EVG) and elastin immunostain. Results: Elastin immunostain was superior to EVG in revealing the elastic fiber patterns. Both regression and scars had decreased to absent elastic fibers in the areas of fibrosis. However, areas of regression had a well-defined compressed layer of thin elastic fibers pushed down from the papillary dermis to the base of the fibrosis. In contrast, the base of scars lacked this compressed elastic layer and had instead an abrupt transition to the thick elastic fibers of the spared reticular dermis. Conclusions: We have identified distinct changes of the elastic tissue network, which more accurately define the presence of regression in melanoma and distinguish it from scarring fibrosis Kamino H, Tam S, Roses D, Toussaint S. Elastic fiber pattern in regressing melanoma: a histochemical and immunohistochemical study.

Background: ProExC is a new marker for identification of precursor lesions of cervical carcinoma. Its utility in noncervical squamous cell carcinoma in situ (SCCIS) such as Bowen's disease (BD) and actinic keratosis (AK) where human papillomavirus (HPV) plays a role has not been elucidated. Our aim was to ascertain the immunohistochemical features and clinical utility of ProExC in SCCIS of the skin. Methods: HPV presence was tested in SCCIS (38 BD and 7 AK) using GP5+/6+ and Short PCR fragment (SPF) primers and subsequently genotyped. Histopathologic sections were stained for ProExC and Ki67. A set of non-neoplastic skin proliferative lesions were included for immunohistochemical evaluation [14 psoriasis (PS) and 6 psoriasiform dermatitis (PSD)]. Results: HPV was detected in 18.9% BD. ProExC and Ki67 in the whole epidermis thickness was observed in 86.5 and 37.1% BD, respectively (p < 0.0001). ProExC and Ki67 were restricted to the lower third of the epidermis in PS and PSD. Conclusions: ProExC expression is not associated with HPV in SCCIS of the skin. Proliferating cells are better delineated in SCCIS by ProExC which may be useful to assess the extent of these lesions. Different immunohistochemical profiles seen in neoplasic and non-neoplastic skin lesions suggest diverse alteration of cell-cycle kinetics. Sánchez-Hernández M, Conesa-Zamora P, García-Solano J, Corbalán-Vélez R, Martínez-Barba E, Pérez-Guillermo M. Expression profiles of ProEx C and Ki67 in squamous cell carcinoma in situ of the skin and their relationship with human papillomavirus genotypes.

Background: Proper diagnosis of myeloid leukemia cutis (LC) is of great clinical importance but can be difficult because no single immunohistochemical marker is adequately sensitive or specific for definitive diagnosis. Thus, a broader panel of markers is often desirable. CD163 is highly specific for normal and neoplastic cells of the monocyte/histiocyte lineage. In this study, we examined the value of CD163 in the diagnosis of acute myeloid LC. Methods: A total of 34 cases, including 18 cases of myelomonocytic or monocytic LC, 10 cases of myeloid LC without monocytic component and 6 cases of acute lymphoblastic leukemia/lymphoma (ALL), were stained with CD163. Results: CD163 was expressed in 8 of 18 (44%) of myelomonocytic or monocytic LC and 1 of 10 (10%) of other myeloid LC, but in none of the ALL cases (0/6). CD163 was highly specific (90%) for myeloid LC with a monocytic component, but showed low sensitivity in the diagnosis of both myeloid LC in general (24%) and myeloid LC with a monocytic component (44%). Conclusions: Our results suggest that CD163 has utility as a specific marker for myeloid LC in conjunction with currently used immunohistochemical stains, but should not be used alone for diagnosis. Harms PW, Bandarchi B, Ma L. CD163 expression in leukemia cutis.

Microcystic adnexal carcinoma (MAC) is a rare, usually solitary, slowly growing, yet aggressive neoplasm with a tendency for local recurrences. Herein, we present two patients who had been histopathologically diagnosed as suffering from MAC on both cheeks since childhood, an unlikely scenario. Both from a clinical and from a histopathological point of view, our two cases showed some similarities with those previously described in patients with Nicolau-Balus syndrome, Rombo syndrome, and so-called eccrine-pilar hamartoma. Common to all these latter disorders are the round aggregations of elastic tissue in the papillary dermis, a histopathological feature which was also found in our patients. However, to our knowledge, the presence of a MAC-like ductal proliferation embedded in sclerotic stroma and extending to the deep dermis has not been previously described. Dermatologists and dermatopathologists should be aware of this disorder to avoid overdiagnosis of and inappropriate treatment for MAC. Schaller J, Rytina E, Rütten A, Hendricks C, Ha T, Requena L. Sweat duct proliferation associated with aggregates of elastic tissue and atrophodermia vermiculata: a simulator of microcystic adnexal carcinoma. Report of two cases.

CD10 is now commonly used to differentiate atypical fibroxanthoma (AFX) from melanoma, spindle cell and dedifferentiated variants of squamous cell carcinoma and leiomyosarcoma. However, we have encountered CD10-positive tumors that mimicked AFX but proved to be myxofibrosarcomas. The purpose of this study was to evaluate CD10 expression in a wide range of mesenchymal neoplasms that may involve the skin using tissue microarrays. Our results indicate that in addition to AFX, CD10 expression is common in myxofibrosarcomas, undifferentiated pleomorphic sarcomas, dermatofibromas and dermatofibrosarcoma protuberans. Myxofibrosarcomas commonly present in the skin and may be difficult to distinguish from AFX on small biopsies and CD10 positivity may confound the diagnostic difficulty. Clarke LE, Frauenhoffer E, Fox E, Neves R, Bruggeman RD, Helm KF. CD10-positive myxofibrosarcomas: a pitfall in the differential diagnosis of atypical fibroxanthoma.

Cutaneous angiosarcoma can sometimes mimic other benign and malignant lesions, thereby presenting a difficult differential diagnosis. In the two cases of cutaneous angiosarcoma presented herein, extensive foamy cell alteration of tumor cells resembled a reactive xanthogranulomatous process. Foamy cell angiosarcoma is an unusual and deceptively benign morphologic variant of cutaneous angiosarcoma. Critical features for diagnosis include the presence of a deep, permeative, sometimes 'scaffolding' growth pattern and subtle areas of vascular formation. Tatsas AD, Keedy VL, Florell SR, Simpson JF, Coffin CM, Kelley MC, Cates JMM. Foamy cell angiosarcoma: a rare and deceptively bland variant of cutaneous angiosarcoma.

Xanthogranulomas are benign lesions composed of macrophages in which some of the cells have vacuolated cytoplasm. They commonly occur in children, referred to as juvenile xanthogranulomata, and are histopathologically characterized by the presence of Touton giant cells. Xanthogranuloma can also occur in adults. A woman who developed a solitary xanthogranuloma within the field of radiotherapy following treatment of her breast cancer is reported. In addition to xanthogranuloma, other benign lesions of keratinocytes, mast cells, endothelial cells and lymphatic cells have been observed at the site of radiation treatment. Also, several malignancies, including hematopoietic cancers, sarcomas, metastatic cancers and common skin cancers, have been observed within radiation ports. A causal relationship in the development of our patient's xanthogranuloma is suggested by the temporal association between the treatment with radiotherapy and the subsequent appearance of a xanthogranuloma directly within the radiation field. Cohen PR, Prieto VG. Radiation port xanthogranuloma and solitary xanthogranuloma occurring within the irradiated skin of a breast cancer patient: report and review of cutaneous neoplasms developing at the site of radiotherapy.

The terms 'epidermotropism' and 'exocytosis' are commonly used to describe intraepithelial lymphocytes in the mycosis fungoides (MF) variant of cutaneous T cell lymphoma (CTCL). However, both terms have not been uniformly or consistently defined. In this study, definitions of both terms were requested from 34 experts. In definitions provided by 19 responders, 'epidermotropism' correlated with MF/CTCL, and 'exocytosis' correlated with benign conditions. Use of these terms often reflected the observer's interpretation, as they were not always used in purely descriptive fashion. Fung MA. 'Epidermotropism' vs. 'exocytosis' of lymphocytes 101: definition of terms.

The main histopathological features in the cutaneous lesions of Churg-Strauss syndrome (CSS) are dermal leukocytoclastic vasculitis with a variable eosinophilic infiltrate and non-vasculitic tissue eosinophilia with granuloma formation. This wide histopathological spectrum may account for the various skin manifestations of CSS. However, the unique histopathological combination of dermal eosinophilic vasculitis and subcutaneous granulomatous phlebitis accompanied by bulla formation has not been previously described. We report an unusual CSS case showing dermal necrotizing eosinophilic vasculitis and granulomatous phlebitis in purpuric lesions coupled with subepidermal blistering. The blisters showed dermal granulomatous dermatitis and eosinophilia without evidence of vasculitis. Dermal necrotizing eosinophilic vasculitis was characterized by fibrinoid alteration of the vessel wall, a prominent perivascular eosinophilic infiltrate, a few infiltrating histiocytes along the affected vessel wall, and the absence of neutrophilic infiltration. The underlying subcutaneous granulomatous phlebitis was characterized by an angiocentric histiocytic infiltrate surrounded by marked eosinophilic infiltrate. Deposition of cytotoxic proteins and radicals derived from eosinophils in the vessel walls and papillary dermis followed by a secondary granulomatous response may account for the unique clinical and histopathological features in this case. Ishibashi M, Kudo S, Yamamoto K, Shimai N, Chen K-R. Churg-Strauss syndrome with coexistence of eosinophilic vasculitis, granulomatous phlebitis and granulomatous dermatitis in bullous pemphigoid-like blisters.

Background: Non-mycosis fungoides (non-MF) primary cutaneous T-cell lymphomas (PCTCL) are heterogeneous and divided into subgroups by the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification of cutaneous lymphomas. We report the first North American series to examine the applicability of the classification, compare our findings with the predominant European literature and confirm the significance of separation into the indolent and aggressive groups. Methods: Forty-four non-MF PCTCL cases with available tissue for phenotyping, adequate clinical staging information and follow-up were reclassified according to the WHO-EORTC classification. Results: Non-MF PCTCL had a longer overall survival (OS) (13.8 years) compared with secondary cutaneous T-cell lymphoma (SC-TCL) (2.5 years). Primary cutaneous anaplastic large cell lymphoma (PC-ALCL) had the most favorable outcome (OS 14.1 years), whereas secondary and primary peripheral T-cell lymphoma, unspecified had the shortest OS (2.5 and 2.4 years, respectively). Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma (CTLCD4) appeared to have a favorable course. Conclusions: Most non-MF PCTCL can be classified according to the WHO-EORTC classification. The relative frequencies are similar to European experience. Non-MF PCTCL is a heterogeneous group with a favorable outcome compared to SC-TCL, especially PC-ALCL and CTLCD4. Separation of non-MF PCTCL into indolent and aggressive groups appears clinically significant and may provide direction for therapeutic decisions. Weaver J, Mahindra AK, Pohlman B, Jin T, His ED. Non-mycosis fungoides cutaneous T-cell lymphoma: reclassification according to the WHO-EORTC classification.

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. Kazakov DV, Schaller J, Vanecek T, Kacerovska D, Michal M. Brooke-Spiegler syndrome: report of a case with a novel mutation in the CYLD gene and different types of somatic mutations in benign and malignant tumors.