Brian R Hinds

Dual MEK/AKT inhibition with trametinib and GSK2141795 does not yield clinical benefit in metastatic NRAS-mutant and wild-type melanoma

Aberrant MAPK and PI3K pathway signaling may drive the malignant phenotype in NRAS‐mutant and BRAFWT NRASWT metastatic melanoma. To target these pathways, NRAS‐mutant and BRAFWT NRASWT patients received oral trametinib at 1.5 mg daily and GSK2141795 at 50 mg daily in a two‐cohort Simon two‐stage design. Participants had adequate end‐organ function and no more than two prior treatment regimens. Imaging assessments were performed at 8‐week intervals. A total of 10 NRAS‐mutant and 10 BRAFWT NRASWT patients were enrolled. No objective responses were noted in either cohort. The median PFS and OS were 2.3 and 4.0 months in the NRAS‐mutant cohort and 2.8 and 3.5 months in the wild‐type cohort. Grade 3 and grade 4 adverse events, primarily rash, were observed in 25% of patients. We conclude that the combination of trametinib and GSK2141795 does not have significant clinical activity in NRAS‐mutant or BRAFWT NRASWT melanoma.

Congenital-type juvenile xanthogranuloma: A case series and literature review

Congenital juvenile xanthogranulomas are infrequently described in the medical literature. We report three previously unpublished cases and systematically review the literature to better characterize this variant.

An unusual case of metastatic breast carcinoma metastasizing to an antecedent rhytidectomy procedural scar

Breast carcinoma remains the most common cutaneous metastasis in women.1 Although breast carcinoma can arise within mastectomy scars as a sign of locoregional recurrence, distant metastasis in the skin is a less common event. We report the case of a 58-year-old white woman with a history of infiltrative ductal carcinoma who presented with multiple cutaneous papules and nodules on the scalp and retroauricular region. A single metastatic focus uniquely occurred in a prior rhytidectomy scar on the left postauricular sulcus. Only 6 cases of metastases homing to distant cutaneous scars have been reported2, 3, 4, 5, 6; to date, this exists as the first case in breast carcinoma (Appendix).

Livedo racemosa, reticulated ulcerations, panniculitis and violaceous plaques in a 46-year-old woman

Clinically amyopathic dermatomyositis (CADM) is a subset of dermatomyositis (DM) that has conventional cutaneous manifestations of DM, but paradoxically, little or no muscle involvement. In 2005, a novel antibody was described in association with CADM – anti-melanoma differentiation-associated gene 5 (anti-MDA5). Patients with this serologic marker have a characteristic mucocutaneous phenotype consisting of skin ulceration among other signs. We describe the case of a 46-year-old woman with CADM, elevated anti-MDA5 autoantibodies, and unusual clinical features (livedo racemosa, florid acral edema) among the classical phenotype of MDA5 DM (arthralgias, ulcerations, panniculitis) and classical DM lesions (Gottron papules, heliotrope rash). The patients did not develop interstitial lung disease or internal malignancies and experienced a rapid response to prednisolone and intravenous immunoglobulins. After 2 years, she has no relapse of her cutaneous disease and continues 5 mg prednisolone and 2 g/kg kilogram of intravenous immunoglobulin every 3 months for maintenance. Our case highlights the clinical heterogeneity of CADM and underscores the importance of a comprehensive approach to DM patients. It was previously postulated that anti-MDA5 antibody could target vascular cells and compromise vascular function, the presence of livedo racemosa lesions, and MDA5 antibodies in a patient with negative thrombophilia workup, reinforce this idea. This is the first case, to our knowledge, of CADM with acral panniculitis and livedo racemosa.

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.

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.

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.