Chronic Progressive Pink-Yellow Papules and Nodules in a Middle-Aged Man.

A 38-year-old man presented with numerous pink-yellow firm papules and nodules on the bilateral elbows for 10 years spreading to the hands and knees in the past year. What is your diagnosis?

IDH2-mutated near ETP-ALL with aggressive leukemia cutis and brisk response to venetoclax and decitabine.

Near early T-cell precursor acute lymphoblastic leukemia (ETP-ALL) is a rare hematologic malignancy, for which second line therapeutic options are limited. T-cell leukemias are also rarely associated with leukemia cutis, which is more often seen in leukemias of myeloid origin. We present the case of an adult male diagnosed with near ETP-ALL, with IDH2 and DNMT3A mutations, suggestive of a myeloid origin, and leukemia cutis. After the patient progressed on hyper-CVAD and nelarabine, we treated him with the BCL-2 inhibitor venetoclax and the hypomethylating agent decitabine. The regimen induced a rapid bone marrow response and resolution of the leukemia cutis.

The Relationship Between Epidermal Mitotic Density, Atypical Mitotic Figure Density, Breslow Depth, Ulceration, and Dermal Mitotic Rate in Cutaneous Melanoma: A Retrospective Cohort Study.

Prognostic factors for melanoma include Breslow depth (BD), ulceration, and dermal mitotic rate (DMR). No studies have queried the effect of epidermal mitotic density (EMD) or atypical mitotic figure density (AMD) in an outcome-based assessment. Our objective was to determine if there is a relationship between EMD, AMD, BD, DMR, and ulceration and patient outcomes. This was a retrospective cohort study of 185 cases of thick and thin melanomas. Univariate and multivariate cause-specific regression analysis was performed. There was a positive correlation between EMD and BD (P = .0001). The difference between AMD in thick and thin melanomas was statistically significant. For every unit increase in EMD, patients had a 2.8-fold increase in the risk of distant metastasis; however, statistical significance was lost in the multivariate analysis. In adjusted analyses, ulceration, DMR, and BD were associated with outcomes. There were no statistically significant correlations between AMD and outcomes. This study is limited by its small sample size, diminution of the epidermis in some thick melanomas preventing EMD estimates, and reproducibility of mitotic figure counting. EMD and AMD do not seem to have any independent value in multivariate analyses for melanoma. Ulceration, BD, and DMR were significantly associated with outcomes and further solidify these known predictors of prognosis.

Cutaneous intralymphatic anaplastic lymphoma kinase-negative anaplastic large-cell lymphoma arising in a patient with multiple rounds of breast implants.

Primary cutaneous anaplastic large-cell lymphoma and breast implant-associated ALCL (BIA-ALCL) are rare subtypes of anaplastic lymphoma kinase (ALK)-negative ALCLs originating from skin and breast implants, respectively. Herein, we report a unique case of cutaneous ALK-negative ALCL occurring in the skin of left medial breast from a patient with multiple rounds of bilateral breast implants and a history of breast carcinoma. The lymphoma cells are entirely confined to the lymphatic channels in the dermis, and the patient has no other areas of skin abnormality, no lymphadenopathy, peri-implant fluid accumulation, or masses from the bilateral capsules of implants. The differential diagnosis and its relationship with breast implants are further explored.

An Unusual Case of Morphea in the Setting of Aplastic Anemia.

Cutaneous sclerosis occurs in association with a variety of systemic diseases, including hematologic malignancy, plasma cell dyscrasias, solid organ tumors, and other systemic autoimmune conditions. Herein, we present a unique case of morphea/lichen sclerosus overlap arising in association with aplastic anemia. To expand upon this rare case, we also review the literature surrounding paraneoplastic sclerosing skin disorders. A 53-year-old man presented with a 13-month history of progressive and generalized skin changes. Exam revealed irregular, hypopigmented indurated plaques with focal areas of scale on the bilateral axillae and hips, as well as hyperpigmented brown papules and plaques on the back. Laboratory evaluation revealed pancytopenia and positive anti-nuclear antibody (1:160). Bone marrow biopsy demonstrated hypocellular marrow consistent with aplastic anemia. Furthermore, skin biopsies revealed lichen sclerosus overlying superficial morphea, consistent with a paraneoplastic sclerodermoid-like eruption. While preparations for hematologic-directed therapies were made, skin-directed therapy with a combination topical steroids and topical calcineurin inhibitors was initiated. Eosinophilic fasciitis and scleroderma have been linked to aplastic anemia, and herein, we expand upon this phenomenon by presenting our case of generalized plaque morphea/lichen sclerosus overlap arising in the setting of aplastic anemia. Dermatologists must be aware of this rare association in order to identify precocious hematologic disease.

Morphologic Forms and Classification of Dermal Mitotic Figure Density in Primary Cutaneous Melanoma: A Retrospective Study.

New American Joint Committee on Cancer eighth edition staging parameters have removed mitotic rate as a stage T1 category criterion, but it remains embedded in the synopsis of primary cutaneous melanoma (CM). A paucity of data is available, characterizing atypical mitotic forms in CM. In this study, we classify the various morphologic forms of atypical mitoses, characterize mitotic figure density, and examine the correlation between atypical mitotic figures and Breslow depth. We performed a retrospective study of 185 thick (>0.8 mm) and thin (<0.8 mm) CM specimens. Metaphase mitotic figures represented the highest percentage of total mitotic figures in cases of thick melanoma (40%) and were the second most common in thin melanoma (18%). The average Breslow depth for melanoma harboring starburst mitoses was 2.85 mm, compared with the average Breslow depth of all thick melanoma cases, 1.88 mm. The average thickness of melanoma cases containing tripolar mitoses was 2.28 mm. Breslow depth correlated with the number of atypical mitotic figures in both thick and thin melanomas (the Pearson correlation test, r = +0.18, P < 0.01). Metaphase and prophase mitoses are a common finding in both thick and thin melanomas. Although atypical mitoses were indiscriminate, starburst and tripolar (ie, multipolar) mitoses were only inherent to cases of thick melanoma (stage T3). In sum, our study reveals a parallel relationship between the density of atypical mitotic figures and Breslow depth.

Genomic analysis reveals low tumor mutation burden which may be associated with GNAQ/11 alteration in a series of primary leptomeningeal melanomas.

Primary central nervous system melanoma is rare and characterized by a variable prognosis, and no current treatment guidelines exist. We describe the clinical course of a 70-year-old female patient diagnosed with primary leptomeningeal melanoma (LMN) whose case represents the diagnostic and management challenges of this tumor. Targeted genomic sequencing of 315 genes from this tumor revealed GNAQ Q209L mutation and low (4 mutations/Megabase) tumor mutation burden (TMB). Wild-type NRAS, KIT, and BRAF were also observed. A cohort of 4,787 melanomas was subsequently analyzed to identify additional primary central nervous system melanomas, of which 10 additional tumors met pathologic criteria (0.21% of total melanoma cohort). These tumors were genomically assessed according to the same targeted sequencing panel, and 6 of the tumors were also found to harbor a GNAQ mutation. All 10 tumors had low (less than or equal to 2 mutations/Megabase) TMB indicating a potential trend between G-protein-coupled receptor (GPCR) alterations and low TMB in LMNs. GPCR alterations were found to significantly correlate with TMB across the cohort of 4,787 melanomas, supporting this potential finding in the limited LMN subset.

A Precocious Presentation of B-cell Acute Lymphoblastic Leukemia as Infiltrated Plaques on the Face.

Leukemia cutis, or infiltration of leukemic cells into the skin, occurs rarely in B-cell acute lymphocytic leukemia (ALL). Herein, we have described a rare, precocious presentation of B-cell ALL presenting as indurated facial plaques in a 69-year-old man. Biopsy of the facial plaques revealed precursor B-cell leukemia/lymphoma in the skin and prompted urgent hematologic-oncologic evaluation. Bone marrow biopsy yielded a final diagnosis of B-cell ALL. The patient underwent induction therapy, and at the last available follow-up, a matched unrelated donor transplant was planned.

Histiocyte-rich Discoid Lupus Erythematosus: A Peculiar Perifollicular Distribution Histologically Mimicking an Acneiform Disorder.

The histologic profile of discoid lupus erythematosus typically involves a vacuolar interface reaction with an associated superficial and deep perivascular infiltrate composed of lymphoplasmacytes. We present a unique case of discoid lupus erythematosus in which cluster of differentiation 68 (CD68) immunochemistry identified widely dispersed histiocytes. Few reports of histiocyte-rich cutaneous lupus erythematosus exist in the literature, and these lymphohistiocytic infiltrates, when present on the H-zone of the face, could be misconstrued as acne/rosacea. Our case demonstrates that cutaneous lupus erythematosus can present with a predominantly histiocytic infiltrate, a pattern dermatopathologists should be aware of to avoid non-recognition or misdiagnosis.

Scabies presenting as cutaneous nodules or malar erythema: reports of patients with scabies surrepticius masquerading as prurigo nodularis or systemic lupus erythematosus.

Scabies surrepticius is a unifying term that represents non-classical presentations of scabies mite infestation. A patient with scabies surrepticius is described: a man with scabies masquerading as prurigo nodularis. The 91-year-old man had metastatic prostate cancer and presented with diffuse pruritic nodules. Prurigo nodularis was suspected; however, the biopsy revealed scabies mites in the stratum corneum. He was successfully treated with topical permethrin 5% cream and oral ivermectin. In addition, the features of a woman with scabies mimicking systemic lupus erythematosus are summarized. The 47-year-old woman had idiopathic thrombocytopenic purpura and presented with malar erythema and a positive antinuclear antibody (titer 1:320). A diagnosis of systemic lupus erythematous was entertained until skin scraping and mineral oil preparation revealed scabies mites; she was successfully treated with oral ivermectin. In conclusion, Sarcoptes scabiei infestation can present with atypical clinical morphology and an absence of classical lesions such as burrows conventionally distributed in the interdigital web spaces, volar wrists, periumbilical area, or genitalia. Scabies surrepticius is a term that has been designated to describe these unusual presentations. Prurigo nodularis and systemic lupus erythematosus can be added to the litany of conditions masquerading as scabies and are included amongst the guises of scabies surrepticius.

Leukemia Cutis Associated with Secondary Plasma Cell Leukemia.

Plasma cell leukemia is an uncommon, aggressive variant of leukemia that may occur de novo or in association with multiple myeloma. Leukemia cutis is the cutaneous manifestation of leukemia, and indicates an infiltration of the skin by malignant leukocytes or their precursors. Plasma cell leukemia cutis is a rare clinical presentation of leukemia. We present a man who developed plasma cell leukemia cutis in association with multiple myeloma. Cutaneous nodules developed on his arms and legs 50 days following an autologous stem cell transplant. Histopathologic examination showed CD138-positive nodular aggregates of atypical plasma cells with kappa light chain restriction, similar to the phenotype of his myeloma. In spite of systemic treatment of his underlying disease, he died 25 days after the presentation of leukemia cutis. Pub-Med was searched for the following terms: cutaneous plasmacytomas, leukemia cutis, plasma cell leukemia nodules, plasma cell leukemia cutis, and secondary cutaneous plasmacytoma. Papers were reviewed and appropriate references evaluated. Leukemia cutis in plasma cell leukemia patients is an infrequent occurrence. New skin lesions in patients with plasma cell leukemia should be biopsied for pathology and for tissue cultures to evaluate for cancer or infection, respectively. The diagnosis plasma cell leukemia cutis is associated with a very poor prognosis.

Acquired Elastotic Hemangioma: Case Series and Comprehensive Literature Review.

Background Acquired elastotic hemangioma is a benign vascular proliferation that typically presents as an asymptomatic red plaque on a sun-exposed site of an adult. Material and Methods The PubMed database was used to search the following words: acquired, angioma, arm, basal, carcinoma, cell, elastosis, elastotic, exposed, forearm, hemangioma, solar, sun, and vascular. The relevant papers and reference cited generated by the search were reviewed. The features from a case series of 11 patients with acquired elastotic hemangioma are presented. In addition, a comprehensive review of the characteristics of this unique hemangioma-not only in our 11 patients but also in the previously reported 34 individuals with this lesion-is provided. Results Acquired elastotic hemangioma, reported in 45 patients (24 women and 21 men), typically appeared as an asymptomatic solitary red plaque in sun-exposed areas-most commonly the forearm--of adults aged 50 years or older. The pathology shows a proliferation of vascular channels-surrounded and intertwined by intense solar elastosis--in the upper dermis, located parallel to the overlying epidermis, and separated from it by a zone of normal-appearing superficial papillary dermis. There was extensive solar elastosis surrounding and between the new blood vessels; some of the endothelial cells protrude (in a hob-nail pattern) into the vessel lumen. The clinical differential diagnosis includes basal cell carcinoma and the pathologic differential diagnosis includes other benign, malignant, and reactive vascular lesions. Ultraviolet radiation may contribute to the pathogenesis of this hemangioma since it occurs on sun-exposed sites. There was no recurrence of the lesion following either excision or observation. Conclusions The possibility of acquired elastotic hemangioma should be considered by clinicians when they encounter an older individual with a new red plaque on a sun-exposed site that clinically appears to be a superficial basal cell carcinoma.

Erythrodermic Psoriasis in a Man with Monoclonal B-cell Lymphocytosis.

Erythroderma is characterized by erythema involving greater than 90% of the body surface area and may be caused by several etiologies, including erythrodermic psoriasis. Psoriasis is an autoimmune skin and systemic condition characterized by erythematous and scaly plaques. Monoclonal B-cell lymphocytosis is an asymptomatic hematological disorder diagnosed by elevated, small, clonal B-cell counts in the peripheral blood. The characteristics of a 71-year-old man with new onset of erythrodermic psoriasis and concurrent monoclonal B-cell lymphocytosis are presented. The simultaneous development of these two conditions raises the possibility that they may share a related pathogenesis.

Crohn disease-associated neutrophilic urticarial dermatosis: report and literature review of neutrophilic urticarial dermatosis.

Neutrophilic urticarial dermatosis (NUD) is a useful diagnostic term for urticarial lesions that are less pruritic and more painful than conventional urticaria. The histopathologic features include neutrophilic infiltrates in the interstitial dermis with a higher density than idiopathic urticaria. NUD has been associated with several systemic conditions, which are predominantly autoimmune and autoinflammatory in nature. A woman with Crohn disease who developed NUD is described. Literature reports of other conditions in which neutrophilic urticarial dermatosis have been observed are also reviewed and summarized. NUD has not only been described in the setting of inflammatory bowel disease, but also in patients who have systemic lupus erythematosus, adult-onset Still disease, and IgA gammopathy. NUD is usually associated with an underlying disease. Therapeutic agents that target neutrophils (such as dapsone and colchicine) and antagonists to interleukin-1 receptor (such as anakinra) may be effective modalities for affected patients. NUD can be added to the list of dermatologic manifestations associated with systemic inflammation, particularly inflammatory bowel disease.

Cutaneous sclerosis: a previously undescribed manifestation of sclerosing mesenteritis.

BACKGROUND: Sclerosing mesenteritis is a rare disease of unknown etiology that is characterized by self-limited, nonspecific inflammation and fibrosis of the mesenteric adipose tissue. Histologic classification characterizes 3 main stages in the evolution of the fibroinflammatory process: mesenteric lipodystrophy (ML), mesenteric panniculitis (MP), and sclerosing (retractile) mesenteritis (SM). OBSERVATIONS: A 68-year-old woman with biopsy-proven MP presented with multiple asymptomatic, indurated subcutaneous nodules on both arms, as well as 2 indurated plaques on her abdomen. The cutaneous changes preceded the diagnosis of SM by roughly 3 years. The arm lesions were centrally depressed with a prominent groove and a peau d'orange appearance. Biopsy findings revealed a subcutaneous process with almost total replacement of adipocytes by zones of woody sclerosis and fat necrosis identical to that observed in the mesentery. To our knowledge, this manifestation of sclerosing mesenteritis has not been reported previously. CONCLUSIONS: Sclerosing mesenteritis has rarely been associated with extra-abdominal idiopathic fibrosclerotic disorders, but a cutaneous component of SM has never been characterized. The fact that the cutaneous lesions were histopathologically identical to the mesenteric changes and their presence prior to the recognition of intra-abdominal disease suggests that future patients with such lesions might be evaluated for this disorder leading to earlier recognition.