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Atypical fibroxanthoma (AFX) with osteoclast-like giant cells is a rare entity. We present the case of an elderly woman who presented with a pink-purple dome-shaped nodule with central hyperkeratotic crust. Biopsy revealed a cellular, dermal-based tumor comprised of spindle, oval, and osteoclast-like giant cells with pleomorphism. The immunohistochemistry profile supported a diagnosis of AFX with osteoclast-like giant cells. We performed a literature review through PubMed and Google Scholar for AFX with osteoclast-like giant cell formation and found 16 previously reported cases. We aim to provide a review and discuss features of these cases. We also discuss the pathogenesis of these osteoclast-like cells as well as potential pitfalls in diagnosis.

Amyloid elastosis is an exceedingly rare form of amyloidosis characterized by amyloid material deposited on dermal elastic fibers. Most reported cases have been associated with systemic amyloid light-chain amyloidosis. A single previously reported case of amyloid elastosis showed evidence that the amyloid material was derived from light-chain proteins and was associated with a monoclonal plasma cell infiltrate but failed to demonstrate systemic involvement. As a result, the case was felt to represent localized cutaneous amyloid elastosis. We present a case of localized cutaneous amyloid elastosis that is not associated with a definitive monotypic plasma cell population or with systemic amyloidosis. We also review the clinical and histopathologic features of reported cases of amyloid elastosis and discuss possible etiologic considerations. Because amyloid elastosis can be either localized to the skin or associated with systemic involvement, additional workup to exclude an underlying plasma cell dyscrasia or hematologic malignancy is warranted.

Autoimmunity Related Neutrophilic Dermatoses (ARND) is a recently described entity characterized by urticarial papules and plaques with histology showing a neutrophilic perivascular and interstitial infiltrates with leukocytoclasis, along with variable vacuolar interface dermatitis. We report a 38 year old Caucasian female with a ten year history of lupus who presented with pruritic urticarial papules and plaques on the face, trunk, upper extremities and thighs. The onset occurred 10 days after she started a calcium channel blocker (CCB), diltiazem. Histopathology revealed scattered dyskeratotic keratinocytes, vacuolar interface dermatitis, and a sparse perivascular and interstitial mixed infiltrate of neutrophils within the dermis. There was focal leukocytoclasis, dermal edema, and rare eosinophils. The patient initially improved with prednisone taper, but flared again upon starting a different CCB, verapamil. Exposure to certain medications such as CCB in the setting of autoimmune connective tissue disorder may be the inciting trigger for a neutrophilic dermatoses. Future studies may provide further information on the pathogenesis of ARND and cutaneous drug eruption in the setting of SLE.

Kikuchi-Fujimoto disease is a rare, self-limited benign necrotizing lymphadenitis that typically manifests as fever and lymphadenopathy with or without extension to other organs including the skin, eyes, and bone marrow.1, 2 The rash may be variable in appearance, although most commonly presents with erythematous macules, papules, and plaques involving the face, upper extremities, and trunk.3 Because the laboratory findings and imaging are nonspecific, the diagnosis depends on histologic examination. Characteristic histopathologic features of Kikuchi-Fujimoto disease include abundant nuclear debris surrounded by a brisk histiocytic infiltrate with varying degrees of lymphocytes and immunoblasts. Neutrophils are typically absent.2 The etiology of Kikuchi-Fujimoto disease is unknown, although various infections including Epstein-Barr virus (EBV), parvovirus B-19, HIV, and human herpesvirus 6 have been implicated. An association with systemic lupus erythematosus (SLE) has also been reported, although the nature of the relationship is unclear.3 We report a case of Kikuchi-Fujimoto disease in the setting of EBV infection diagnosed following correlation between skin and lymph node biopsies.

Vismodegib demonstrated 60% response rates in the ERIVANCE trial. Basal cell carcinoma has various histopathologies. Their effect on response is unclear.The purpose of this study was to determine whether basal cell carcinoma histopathology affected vismodegib response.This phase 2b, single-center, prospective case series study compared the efficacy of vismodegib in infiltrative, nodular, and superficial basal cell carcinomas treated for 12 or 24 weeks in 27 patients. Patients had 1 target lesion and up to 3 nontarget lesions.Twenty-seven patients were enrolled, with 65 tumors (27 target lesions/38 nontarget lesions). At 24 weeks, most basal cell carcinomas achieved histologic clearance, with positive biopsy results in 10.5% of target lesions, 30.4% of nontarget lesions, and 21.4% overall. No statistical differences were observed between histopathologic subtypes. One hundred percent of patients experienced an adverse event, 94% grade 1 or 2. The most common adverse events were dysgeusia/loss of taste (86%), muscle spasms (82%), and alopecia (71%). Clinically progressive disease during treatment was low (1.5%). Two patients had recurrence within 1 year of treatment.Limitations included sample size of basal cell carcinoma histopathologic subtypes, sampling punch biopsies, and short follow-up.Basal cell histopathologic subtype did not significantly affect response to vismodegib. Each subtype was observed to completely respond at 12 weeks of therapy, 24 weeks, or both.

Background Studies on the precise cause of increased melanization in pigmented basal cell carcinomas (BCC) are limited. We aimed to determine whether the cause of melanization is from increased number of melanocytes or increased melanin pigment, and if there is a difference in the number of melanocytes on different sun-exposed locations. Methods A retrospective review of 45 skin biopsies from January 2011 to February 2011 was performed; 30 were diagnosed as pigmented BCC and 15 as non-pigmented basal cell carcinoma. Immunohistochemistry for MART-1 (melanoma-associated antigen recognized by T-cell 1)/Melan-A (clone M2-7610 + M2-9E3; Leica) from Biocare Medical (Concord, CA) was performed on all biopsies. Associations between histopathologic features, number of melanocytes, location, and specific diagnoses were analyzed by Mann Whitney U test. Results The mean melanocyte count per high powered field in pigmented BCCs from sun-exposed skin was 101.9 and from intermittently sun-exposed skin was 122.5, as compared to the controls (nodular non-pigmented BCC) of 27.4 (p = 0.002) and 34.9 (p = 0.002), respectively. Conclusions Pigmented BCCs have a higher mean melanocyte count as compared to non-pigmented BCCs irrespective of location. Therefore, the pigment is not only due to increased melanin, but also due to increased melanocytes.

Cutaneous mantle cell lymphoma (MCL) is exceedingly rare, almost always occurring as a dissemination of systemic MCL. To date, only 32 cases have been described. We report a series of 10 cases of MCL in the skin, and provide a comprehensive clinicopathologic review with clinical follow-up. Our cases occurred in older individuals (mean age = 70) and were more frequently in men (90%). Half of them presented in the head and neck region as a mass/nodule, and the remainder in the trunk and extremities as nodules. All patients have stage IV disease. In two of the 10 cases (20%) the cutaneous lesions preceded the diagnosis of disseminated disease. In two of the seven cases with available clinical follow-up information (33%) the skin was immediately involved after the diagnosis of MCL, and in three of the seven the skin was the first site of recurrence (mean interval = 57 months). The mean time to recurrence of the disease was 45.4 months and the overall survival was 66.3 months. Histologically six out of 10 cases (60%) had either pleomorphic or blastoid morphology (four out of 10 and two out of 10, respectively). The mean number of mitoses per 10 high-power fields was 18.44. While nine out of 10 cases expressed cyclin-D1, one case was not positive for cyclin-D1 but did label with SOX-11. Limited cytogenetic data showed trisomy 14 in one case, in addition to the t(11;14) translocation.

BACKGROUND Mantle cell lymphoma (MCL) is associated with the worst prognosis among low-grade B-cell lymphomas. While cutaneous involvement by nodal or systemic MCL is uncommon, its differentiation from primary cutaneous B-cell lymphoma (CBCL) or cutaneous involvement by other extra-cutaneous BCL is challenging as neither histomorphology nor immunophenotype can be absolutely specific. We analyzed the diagnostic utility of SOX11 immunohistochemistry in differentiating secondary cutaneous MCL from other low-grade CBCL. METHODS Immunohistochemical staining with anti-SOX11 antibody was performed on 8 cases of secondary cutaneous MCL, 16 secondary cutaneous CLL, 20 primary cutaneous MZL, 12 cutaneous FCL (6 primary, 6 secondary), 7 primary cutaneous DLBCL, leg type, 5 systemic DLBCL and 3 B-ALL. SOX11 and cyclin D1 staining were compared in secondary cutaneous MCL. RESULTS Nuclear SOX11 staining was seen in seven of eight cases (88%) of secondary cutaneous MCL, including a case with minimal cyclin D1 expression. All other CBCL lacked detectable nuclear SOX11 expression. The sensitivity and specificity for SOX11 in MCL were 87.5 and 100%, respectively. Both the sensitivity and specificity for combined SOX11 and cyclin D1 immunohistochemistry were 100%. CONCLUSION SOX11 immunohistochemistry could be a useful adjunct in distinguishing secondary cutaneous MCL from other CBCL.

Muir-Torre syndrome (MTS) is a rare inherited syndrome, with an increased risk of sebaceous and visceral malignancy. Prior reports suggest screening for mismatch repair (MMR) deficiency may be warranted in patients50 years and when sebaceous neoplasms are located on a non-head and neck location. Previously, appropriate use criteria (AUC) were developed for clinical scenarios in patients60 years concerning the use of MMR protein immunohistochemistry (MMRP-IHC). This analysis explores the appropriateness of testing in patients ≤60 years.Panel raters from the AUC Task Force rated the use of MMRP-IHC testing for MTS for previously rated scenarios with the only difference being age.Results verify the previously developed AUC for the use of MMRP-IHC in neoplasms associated with MTS in patients60 years. Results also show that in patients ≤60 years with a single sebaceous tumor on a non-head and neck site, MMRP-IHC testing should be considered. Testing can also be considered with a 2-antibody panel on periocular sebaceous carcinoma in younger patients.Our findings align with known evidence supporting the need to incorporate clinical parameters in identifying patients at risk for MTS, with age being a factor when considering MMRP-IHC testing.

While the vast majority of skin cancers can be managed by a single practitioner in the outpatient setting, there is a subset of complex skin cancers that benefit from multidisciplinary care. At our institution, we have built strong collegial relationships with allied specialties such as surgical oncology, otolaryngology-head and neck surgery, plastic surgery, facial plastic surgery, and oculoplastic surgery. It is common for patients with high-risk or complex tumors to be seen pre-operatively by several allied specialties. This allows for care coordination and more seamless care in addition to fostering skin cancer expertise within our collaborators in allied specialties.

We conducted a retrospective study of patients with cutaneous myeloid sarcoma, from 2 tertiary care institutions. Eighty-three patients presented, with a mean age of 52 years. Diagnosis of myeloid sarcoma in the skin was difficult due to the low frequency of myeloperoxidase and/or CD34+ cases (56% and 19% of tested cases, respectively). Seventy-one of the 83 patients (86%) had ≥ 1 bone marrow biopsy. Twenty-eight (39%) had acute myeloid leukemia with monocytic differentiation. Twenty-three had other de novo acute myeloid leukemia subtypes. Thirteen patients had other myeloid neoplasms, of which 4 ultimately progressed to an acute myeloid leukemia. Seven had no bone marrow malignancy. Ninety-eight percent of the patients received chemotherapy, and approximately 89% died of causes related to their disease. Cutaneous myeloid sarcoma in most cases represents an aggressive manifestation of acute myeloid leukemia. Diagnosis can be challenging due to lack of myeloblast-associated antigen expression in many cases, and difficulty in distinguishing monocyte-lineage blasts from neoplastic and non-neoplastic mature monocytes.

Idelalisib is a selective inhibitor of the delta isoform of phosphatidylinositol 3-kinase which was approved by the United States Federal Drug Administration in 2014 for the treatment of relapsed chronic lymphocytic leukemia and indolent non-Hodgkin lymphoma. Drug-induced injury of the gastrointestinal tract is a relatively frequent but usually under-recognized disease entity. We report the case of a 56-year-old male with a history of relapsed follicular lymphoma status post allogenic bone marrow transplant who developed severe diarrhea with a skin eruption mimicking graft-versus-host disease (GVHD) 6 months after starting idelalisib. He underwent a colonoscopy demonstrating a grossly normal-appearing colon and terminal ileum. Biopsies taken during the procedure revealed mild active ileitis, colitis, and proctitis with frequent epithelial apoptosis, and focal intra-epithelial lymphocytosis. Skin biopsies revealed sub-acute spongiotic dermatitis suggestive of either contact dermatitis or an eczematous drug reaction. Symptoms were attributed to idelalisib given their resolution with withdrawal of the drug in conjunction with the skin and colonic biopsies. High clinical suspicion and awareness of the histological features of idelalisib-associated colitis is important to distinguish it from potential mimickers such as GVHD and infectious colitis.

Cutaneous mantle cell lymphoma (MCL) is exceedingly rare, almost always occurring as a dissemination of systemic MCL. To date, only 32 cases have been described. We report a series of 10 cases of MCL in the skin, and provide a comprehensive clinicopathologic review with clinical follow-up. Our cases occurred in older individuals (mean age = 70) and were more frequently in men (90%). Half of them presented in the head and neck region as a mass/nodule, and the remainder in the trunk and extremities as nodules. All patients have stage IV disease. In two of the 10 cases (20%) the cutaneous lesions preceded the diagnosis of disseminated disease. In two of the seven cases with available clinical follow-up information (33%) the skin was immediately involved after the diagnosis of MCL, and in three of the seven the skin was the first site of recurrence (mean interval = 57 months). The mean time to recurrence of the disease was 45.4 months and the overall survival was 66.3 months. Histologically six out of 10 cases (60%) had either pleomorphic or blastoid morphology (four out of 10 and two out of 10, respectively). The mean number of mitoses per 10 high-power fields was 18.44. While nine out of 10 cases expressed cyclin-D1, one case was not positive for cyclin-D1 but did label with SOX-11. Limited cytogenetic data showed trisomy 14 in one case, in addition to the t(11;14) translocation.

Cutaneous and systemic plasmacytosis is a rare condition primarily affecting middle-aged individuals of Japanese descent and has principally been reported in cases originating from Asia. Its relationship, if any, to Castleman disease, is controversial. The authors report a case of cutaneous and systemic plasmacytosis involving a 36-year-old man of Chinese ancestry born in North America. The biopsy of an involved lymph node revealed changes resembling Castleman disease. Significantly, this case to our knowledge represents the first case of cutaneous and systemic plasmacytosis presenting in a patient of Chinese ancestry born on the North American mainland. Shadel BN, Frater JL, Gapp JDG, Hurley MY. Cutaneous and systemic plasmacytosis in an Asian male born in the North American continent: A controversial entity potentially related to multicentric Castleman disease.

Primary cutaneous lymphomas are non-Hodgkin lymphomas, which are broadly divided into cutaneous T-cell lymphomas and cutaneous B-cell lymphomas. These classifications include numerous distinct entities, all with varying clinical presentations and disease courses. Herein, we will review the cutaneous T-cell lymphomas, including Mycosis Fungoides, Sézary syndrome, CD30+ lymphoproliferative disorders, as well as other less common entities. Cutaneous B-cell lymphomas will also be discussed, including primary cutaneous marginal zoned lymphoma, cutaneous follicle-center lymphoma, diffuse large B-cell lymphoma, leg type, as well as other less common entities. Accurate and early diagnosis is key, as the treatment and prognosis varies significantly between conditions.

Studies on the precise cause of increased melanization in pigmented basal cell carcinomas (BCC) are limited. We aimed to determine whether the cause of melanization is from increased number of melanocytes or increased melanin pigment, and if there is a difference in the number of melanocytes on different sun-exposed locations.A retrospective review of 45 skin biopsies from January 2011 to February 2011 was performed; 30 were diagnosed as pigmented BCC and 15 as non-pigmented BCC. Immunohistochemistry for MART-1 (melanoma-associated antigen recognized by T-cell 1)/Melan-A (clone M2-7610 + M2-9E3; Leica Microsystems Inc. Buffalo Grove, IL, USA) from Biocare Medical (Concord, CA, USA) was performed on all biopsies. Associations between histopathologic features, number of melanocytes, location, and specific diagnoses were analyzed by Mann-Whitney U test.The mean melanocyte count per high powered field in pigmented BCCs from sun-exposed skin was 101.9 and from intermittently sun-exposed skin was 122.5, as compared to the controls (nodular non-pigmented BCC) of 27.4 (p = 0.002) and 34.9 (p = 0.002), respectively.Pigmented BCCs have a higher mean melanocyte count as compared to non-pigmented BCCs irrespective of location. Therefore, the pigment is not only due to increased melanin, but also due to increased melanocytes.

Mantle cell lymphoma (MCL) is associated with the worst prognosis among low-grade B-cell lymphomas. While cutaneous involvement by nodal or systemic MCL is uncommon, its differentiation from primary cutaneous B-cell lymphoma (CBCL) or cutaneous involvement by other extra-cutaneous BCL is challenging as neither histomorphology nor immunophenotype can be absolutely specific. We analyzed the diagnostic utility of SOX11 immunohistochemistry in differentiating secondary cutaneous MCL from other low-grade CBCL.Immunohistochemical staining with anti-SOX11 antibody was performed on 8 cases of secondary cutaneous MCL, 16 secondary cutaneous CLL, 20 primary cutaneous MZL, 12 cutaneous FCL (6 primary, 6 secondary), 7 primary cutaneous DLBCL, leg type, 5 systemic DLBCL and 3 B-ALL. SOX11 and cyclin D1 staining were compared in secondary cutaneous MCL.Nuclear SOX11 staining was seen in seven of eight cases (88%) of secondary cutaneous MCL, including a case with minimal cyclin D1 expression. All other CBCL lacked detectable nuclear SOX11 expression. The sensitivity and specificity for SOX11 in MCL were 87.5 and 100%, respectively. Both the sensitivity and specificity for combined SOX11 and cyclin D1 immunohistochemistry were 100%.SOX11 immunohistochemistry could be a useful adjunct in distinguishing secondary cutaneous MCL from other CBCL.

Background: The latest comprehensive review of primary cutaneous Rosai-Dorfman disease was published as part of an exhaustive survey of sinus histiocytosis with massive lymphadenopathy in 1990. Since then, much progress has been made in the understanding of malignant lymphoma and benign disorders of lymphoid and histiocytic origin. Objective: We reviewed cases of primary cutaneous Rosai-Dorfman disease published since 1990 and discuss their clinical and pathologic features, comparing them with cases of systemic Rosai-Dorfman disease. Methods: We conducted a search of the National Library of Medicine PubMed database for cases of cutaneous Rosai-Dorfman disease reported in the English-language medical literature since February 1990. Results: We identified 72 patients with cutaneous Rosai-Dorfman (female to male ratio 1:0.5). The gross appearance and number or distribution of lesions were highly variable. Abnormal laboratory data included peripheral blood cytopenias (10 patients) and increased gammaglobulin fraction (10 patients). The response to treatment was variable. Conclusion: Purely cutaneous disease without the characteristic lymphadenopathy is rare but has been increasingly reported in the literature. Compared with patients with systemic Rosai-Dorfman disease, patients with primary cutaneous Rosai-Dorfman disease are older, women are more commonly affected, and whites are more likely than blacks to be afflicted.

A 56-year-old Caucasian female presented with a 2-month history of alopecia. On examination, she had diffuse hair loss of her scalp with some discrete patches of nonscarring alopecia. Histopathology revealed an inflammatory nonscarring alopecia with prominent follicular mucinosis and findings suggestive of alopecia areata. The patient's alopecia completely resolved with oral prednisone. The histopathologic findings and clinical presentation are most consistent with a diagnosis of alopecia areata with follicular mucinosis, although the differential diagnosis is broad. As follicular mucinosis may be associated with both benign and malignant conditions, it is important to be cautious regarding the clinical diagnosis when this reaction pattern is observed histopathologically.

Incidents of new-onset vitiligo attributed to infliximab therapy for rheumatoid arthritis and ulcerative colitis have been reported. Reported cases share a common theme in that symptoms manifested in close proximity to the initiation or significant dose increase of the medication. This case describes the presentation of infliximab-induced vitiligo in a patient using it for long-term treatment of stable pityriasis rubra pilaris. The patient was initiated and titrated to a stable dose of infliximab totaling 27 months' duration. He was able to achieve near-complete resolution of symptoms before developing depigmented patches consistent with vitiligo. Infliximab was discontinued. Tacrolimus 0.1% ointment and narrow-band ultraviolet B light successfully repigmented the patches. The association of discontinuing infliximab and resolution of vitiligo suggests infliximab had a role in this case. Though the mechanism of involvement is undetermined, infliximab may have induced an autoimmune process by paradoxically activating lymphocytes. Alternatively, infliximab antibodies may have led to the process by disrupting the normal balance of cytokines.

9565Background: Gene expression profiling (GEP) is becoming an integral part of treatment decision making in cancer. A GEP test (DecisionDx-Melanoma) has been in commercial use based on data from m...

Acitretin, a metabolite of the aromatic retinoid etretinate, has been utilized successfully in the treatment of psoriasis since the late 1980s. Of the oral retinoids available, etretinate and acitretin are the most likely agents to induce various dose-dependent hair changes, but to our knowledge this is the first reported case of acitretin-induced poliosis. Additional cutaneous findings included skin atrophy and stickiness. Here we report a case of full body acitretin-induced poliosis with concurrent alopecia in a patient with psoriasis. A proposed mechanism for the poliosis is also presented here. Closer examination of retinoid-induced hair changes is needed in order to help physicians better counsel their patients regarding the adverse effects of acitretin and to expand the current knowledge on hair follicle biology.

A 23-year-old Chinese man presented with a 3-year history of a pruritic eruption. On examination, pink urticarial papules associated with hyperpigmented reticulated patches were noted on his neck, back, and upper chest. Histopathology revealed vacuolar interface dermatitis and numerous gram-negative rods within a dilated hair follicle. The organisms were reactive with anti-Helicobacter pylori immunohistochemisty. The histologic findings and clinical presentation support the diagnosis of prurigo pigmentosa. Additional testing demonstrated a positive urease breath test and serum H. pylori IgG antibodies. The patient was referred to gastroenterology and treated with appropriate antibiotics. After treatment, esophagogastroduodenoscopy revealed chronic gastritis without evidence of H. pylori infection and his skin showed reticulated hyperpigmented patches without evidence of active inflammatory papules. Although previous reports have associated prurigo pigmentosa to H. Pylori gastritis, this is the first report of H. pylori organisms identified in a skin biopsy of prurigo pigmentosa.

Basal cell nevus syndrome (BCNS) is an autosomal dominant genodermatosis that is characterized by early onset basal cell carcinomas (BCCs) that define the disease and often lead to diagnosis of the underlying syndrome. The objective of this study was to investigate the anatomic location, subtypes, and impact of BCCs on a group of 61 individuals affected with BCNS attending a research colloquium. Fifty individuals had at least one prior BCC with 22 participants having over 100 lesions. The median age of syndrome diagnosis was 11 years and median age of first BCC was 16 years. Males had more lesions on the upper back, upper extremities, and M-zone of the face, while females had more lesions on the scalp, back, and lower extremities. Pigmented BCCs were concentrated on the neck, upper trunk, and extremities. Subjects with >100 lesions showed wider anatomic distribution. The number of BCCs did not correlate with any of the other major features of the syndrome. Eighty percent of affected individuals reported great concern related to BCCs, citing the limitations and morbidity of available treatments. Vigilant surveillance, which was found to be inconsistent for participants in this study, is warranted. Future work should include development of a consensus guideline on skin examinations and strategies to optimize therapy of BCCs in this syndrome.

Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy is a rare disease. A third of patients with this disease have extranodal involvement affecting the skin. Of these individuals, only around 3% will have purely cutaneous Rosai-Dorfman disease, which is limited to skin manifestations without systemic involvement. Cutaneous (localized) scleroderma or morphea, on the other hand, is a more common disease that most often affects women of all ages. Both conditions have unknown etiologies. Presented here is a case of a 60-year-old white woman with cutaneous Rosai-Dorfman disease and coexisting morphea. Representative biopsies from both areas were performed: one showing a dermal S-100+ histiocytic infiltrate with emperipolesis and the other showing a deep perivascular and interstitial plasma cell infiltrate with dermal sclerosis and loss of perieccrine fat. A laboratory and radiologic workup revealed no evidence of systemic involvement by either entity. The diagnosis of coexisting cutaneous Rosai-Dorfman disease and morphea was established. To our knowledge, this is the first report of these 2 entities found simultaneously in 1 patient.