An Updated Review of the Therapeutic Management of Keratoacanthomas.

Objective: Keratoacanthomas are fast-growing cutaneous neoplasms that can be difficult to distinguish from squamous cell carcinoma, both clinically and histologically. The uncertain behavior of these neoplasms creates a challenge in management, and treatment choice often varies significantly between cases. The objective of this review is to discuss the most common and up-to-date treatment modalities used in the management of keratoacanthomas. Methods: A literature search was performed using PubMed to access and review relevant keratoacanthoma treatment modalities published within the last 40 years. Keywords searched included "keratoacanthoma," "Grzybowski syndrome," "Ferguson-Smith syndrome," "Witten-Zac syndrome," and "Muir-Torre" syndrome. Results: Our search resulted in 3,408 articles, of which 67 articles were ultimately included in this review. Conclusion: Although surgical removal with excision or Mohs micrographic surgery remains the standard of therapy, there are many alternative therapeutic modalities that can be utilized.

Superficial solitary fibrous tumor masquerading as a dermoid cyst: a case report.

Background: Solitary fibrous tumors (SFTs) are relatively rare spindle cell neoplasms uncommonly seen in dermatology practice. Initially discovered as a pleural tumor, SFTs have also been found in extra-pleural sites including the skin and soft tissues. When arising within the dermis or subcutis they are termed superficial SFTs, where they often present as solitary, unilateral, slow growing superficial masses. Histologically, they are composed of spindle cells arranged in a "patternless" pattern with hemangiopericytoma-like vessels dispersed throughout. Historically, CD34, CD99 and Bcl-2 immunohistochemical (IHC) stains were used to differentiate SFTs from other spindle cell neoplasms, however these markers are not entirely specific. Recent discovery of a disease defining NGFI-A binding protein 2 (NAB2)-signal transducer and activator of transcription 6 (STAT6) fusion gene has led to the use of STAT6 IHC staining to help verify the diagnosis of SFTs, particularly in unexpected sites. Case Description: We report a case of a 23-year-old woman with a slowly growing lateral supra-orbital mass, clinically concerning for a dermoid cyst, which was subsequently discovered to be a SFT on pathologic examination, with the diagnosis being verified by STAT6 immunostaining. Conclusions: SFTs are rarely encountered in dermatologic practice, however, must be kept on the differential of subcutaneous nodules, including those occurring in young adults. Due to the rarity of these tumors in clinical practice, a proposed algorithm for the approach to management of SFTs is included, guided by a validated, histology-driven, metastatic risk assessment tool, to help guide other clinicians confronted by these tumors.

Impaired calcium signalling and neuropsychiatric disorders in Darier disease: An exploratory review.

Darier (Darier-White) disease (DD) is an autosomal dominant skin disorder caused by pathogenic mutations in the ATP2A2 gene which encodes a calcium ATPase in the sarco-endoplasmic reticulum (SERCA2). Defects in the SERCA2 protein lead to an impairment of cellular calcium homeostasis, which in turn, triggers cell death pathways. There is a high prevalence of neuropsychiatric disorders in patients affected by this condition, namely intellectual disability, bipolar disorder, schizophrenia, and suicidality. Though these associations have been well-documented over the years, little has been discussed or investigated regarding the pathophysiological mechanisms. The goal of this article is to review the literature related to the most commonly associated neuropsychiatric disorders found in patients with DD, highlight the pathophysiological mechanisms underlying each condition, and examine potential interventions that may be of interest for future development. A literature search was performed using PubMed to access and review relevant articles published in the last 40 years. Keywords searched included Darier disease neuropsychiatric, Darier disease pathophysiology, SERCA2 central nervous system, SERCA 2 skin, ATP2A2 central nervous system, ATP2A2 skin, sphingosine-1-phosphate signalling skin, sphingosine-1-phosphate signalling central nervous system, P2X7 receptor skin, and P2X7 receptor central nervous system. Our search resulted in 2692 articles, of which 61 articles were ultimately included in this review.

Multiple Indolent Asymptomatic Yellow-Orange Patches and Plaques.

An 83-year-old Caucasian male presented with a history of asymptomatic yellow-orange macules and plaques concentrated on his trunk and proximal extremities that have been slowly progressing for the past three years. A punch biopsy revealed the presence of eosinophilic amorphous and fissured material within the superficial and interstitial dermis consistent with nodular amyloidosis. With the lack of concurrent systemic symptoms and negative systemic laboratory workup, the patient was diagnosed with disseminated primary localized cutaneous nodular amyloidosis (PLCNA). Due to the possibility of developing systemic progression, serial monitoring was recommended. This case highlights an under-reported and unusual presentation of a widely distributed form of PLCNA compared to the more common localized nodular and plaque variants.

Facial Edema Associated with Dasatinib: Case Report with Successful Treatment.

INTRODUCTION: Tyrosine kinase inhibitors (TKI) are an effective treatment option for chronic myeloid leukemia (CML). The most common associated adverse effects of TKI include thrombocytopenia, neutropenia, nausea, vomiting, and diarrhea. Facial edema is a rare adverse reaction that may cause significant psychological burden. Treatment is life-long in many cases therefore it is vital to have options available to manage these adverse effects. CASE REPORT: We present a 70-year-old female with a medical history of CML, diabetes, hypertension, and hypercholesterolemia who presented to our dermatology clinic for chief complaint of worsening edematous facial rash beginning after initiation of dasatinib. We were able to achieve significant improvement with a regimen that allowed her to remain on dasatinib. MANAGEMENT AND OUTCOME: We treated the patient with a novel, unreported regimen of topical metronidazole 1% gel to be applied every morning and topical tacrolimus 0.1% ointment to be applied twice daily. She had significant improvement with the treatment and was continued on this topical regimen indefinitely. DISCUSSION: Previous reports of treatment options available for TKI-associated facial edema include topical and systemic corticosteroids, which can cause long-term side effects int the context of long-term TKI use. Our patient achieved an acceptable reduction in facial edema and rash with our combination regimen of metronidazole gel and tacrolimus ointment. We present the only such case of successful treatment of facial edema associated with a tyrosine kinase inhibitor. We encourage future studies on the efficacy and safety of this regimen to treat this adverse effect.

Drug-induced hypersensitivity syndrome following temozolimide for glioblastoma multiforme and the role of desensitization therapy.

INTRODUCTION: Temozolomide is an oral alkylating agent used as first line treatment of glioblastoma multiforme (GBM). It has also been used in the treatment of certain solid tumors such as metastatic melanoma. Commonly reported adverse effects include nausea and vomiting, constipation, headache, fatigue and myelosuppression. Cutaneous hypersensitivity reactions are rare and include an urticarial hypersensitivity reaction, alopecia, and Stevens-Johnson syndrome. To our knowledge, there are minimal reports of temozolomide-induced DRESS syndrome. CASE REPORT: We present a 54-year-old man with glioblastoma multiforme who presented with a fever, diarrhea and progressively worsening rash 6 weeks after starting temozolomide. MANAGEMENT & OUTCOME: The patient was diagnosed recurrent DRESS syndrome and restarted on a gradual prednisone taper with resolution over the following weeks. Unfortunately, the patient was unable to be followed long-term due to relocation to a different state. DISCUSSION: To our knowledge, there are minimal reports of temozolomide-induced DRESS syndrome. The diagnosis can be life-threatening, which makes management of patients with GBM and no alternative treatment option challenging. The use of de-sensitization therapy to temozolomide has been proposed for the management of severe adverse cutaneous reactions.

An Updated Review of the Therapeutic Management of Keratoacanthomas.

Objective: Keratoacanthomas are fast-growing cutaneous neoplasms that can be difficult to distinguish from squamous cell carcinoma, both clinically and histologically. The uncertain behavior of these neoplasms creates a challenge in management, and treatment choice often varies significantly between cases. The objective of this review is to discuss the most common and up-to-date treatment modalities used in the management of keratoacanthomas. Methods: A literature search was performed using PubMed to access and review relevant keratoacanthoma treatment modalities published within the last 40 years. Keywords searched included "keratoacanthoma," "Grzybowski syndrome," "Ferguson-Smith syndrome," "Witten-Zac syndrome," and "Muir-Torre" syndrome. Results: Our search resulted in 3,408 articles, of which 67 articles were ultimately included in this review. Conclusion: Although surgical removal with excision or Mohs micrographic surgery remains the standard of therapy, there are many alternative therapeutic modalities that can be utilized.

Recurrent Erythema Multiforme Induced by the Combination of Pembrolizumab and Radiation.

Erythema multiforme is a self-limited condition that is characterized by the rapid onset of symmetric targetoid lesions. This hypersensitivity reaction is commonly triggered by herpes simplex virus and Mycoplasma pneumoniae infections. Pembrolizumab is an anti-programmed cell death protein 1 (PD-1) inhibitor that is associated with drug-induced erythema multiforme. Radiotherapy is a rarely reported trigger of erythema multiforme. We report a case of recurrent pembrolizumab and radiotherapy-induced erythema multiforme in a patient with stage IV non-small cell lung cancer. This study aimed to highlight a unique cause of erythema multiforme and to review the common radiation-induced cutaneous reactions.

Recurrent Drug-Induced Hypersensitivity Syndrome Following Bortezomib for Multiple Myeloma.

Drug-induced hypersensitivity syndrome is a rare, severe, and delayed hypersensitivity reaction that may occur with exposure to a number of medications. Typical implicated medications include aromatic anticonvulsants, sulfonamides, minocycline, dapsone, and allopurinol. Bortezomib is a proteasome inhibitor and has rarely been associated with cutaneous hypersensitivity reactions. We report a case of recurrent drug-induced hypersensitivity syndrome secondary to bortezomib in a patient with multiple myeloma. The aim of this article is to highlight a unique mediation that may cause drug-induced hypersensitivity syndrome and to emphasize the challenge of managing these patients long-term to prevent relapse of the syndrome.

Scabies Associated with Granulomatous Dermatitis.

Scabies is a relatively common parasitic infection, which has sometimes been associated with the formation of granuloma annulare. We report the case of an 86-year-old female who developed a non-necrotizing granulomatous dermatitis after a scabies infestation. This case highlights the importance of considering scabies in the differential diagnosis when granulomatous dermatitis is noted histologically that cannot be explained by other causes.

Pseudoxanthoma elasticum-like changes in nonuremic calciphylaxis: Case series and brief review of a helpful diagnostic clue.

Calciphylaxis is a rare syndrome of vascular calcification with thrombosis that occurs most often in patients with end-stage renal disease, and it frequently portends a guarded prognosis. Rarely, nonuremic calciphylaxis (NUC) may occur; in this context, a strongly supportive histology is crucial in establishing the diagnosis. Herein, we describe 2 cases of NUC associated with pseudoxanthoma elasticum-like changes, identified in both initial nondiagnostic and subsequent diagnostic biopsy specimens. This unusual but helpful histologic finding may support the early diagnosis and treatment of a potentially life-threatening disease in the context of subtle histopathologic vascular changes or in the absence of classic clinical or laboratory findings.