Wong-Type Dermatomyositis: Literature Review of a Rare Variant.

ABSTRACT: Wong-type dermatomyositis (WTDM) was first formally discussed in the literature in 1969 by Dr. K.O. Wong. This rare variant of dermatomyositis (DM) is characterized by overlapping features of both classic DM and the cutaneous features of pityriasis rubra pilaris. Since 1969, few cases of WTDM have been published in the literature likely due to the rarity of this condition or lack of recognition by clinicians. This narrative review presents the current published English literature on WTDM, analyzing its clinical presentation, diagnostic testing, and treatments along with a comparison to classic DM. Given the overlap of features of both diseases and patients experiencing a better response to classic DM treatments, our results suggest that WTDM is a rare subtype of DM rather than simply an overlap of pityriasis rubra pilaris and DM presenting in 1 patient. We suggest that clinicians evaluate WTDM patients with very thorough histories, physical examinations, histopathology, and appropriate serological studies and monitor closely for systemic symptoms and development of malignancy. WTDM should be treated using conventional treatments for classical DM. Further studies are needed to understand the pathogenesis of WTDM including more specific and distinguishing autoantibody profiles from classical DM, as well as long-term clinical course of WTDM for best management, including recently available biological treatments.

A Comparative Analysis of Local Anesthetics: Injection Associated Pain and Duration of Anesthesia.

BACKGROUND: Local anesthesia administration is frequently the most painful step of dermatologic surgery. Identification of an anesthetic that minimizes infiltration pain and toxicity while maximizing duration of action would improve both patient satisfaction and procedural safety. This study compared eight local anesthetic solutions to identify the composition that minimizes infiltration pain, maximizes duration of effect, and minimizes amount of local anesthetic needed. METHODS: In a double-blinded study, thirty subjects were injected with eight local anesthetic solutions of varied concentrations of lidocaine, epinephrine, benzyl alcohol, and sodium bicarbonate. Infiltration pain was rated by subjects using a visual analog scale and duration of anesthesia was assessed by needle prick sensation every 15 minutes. RESULTS: Solutions 2, 7, and 8, were significantly less painful (P<0.001), though not statistically different from each other. Two of the three solutions were buffered 10:1 with sodium bicarbonate. Additionally, two of the three contained notably decreased concentrations of lidocaine, 0.091% and 0.083%, than traditionally used in practice. The use of benzyl alcohol did not result in a reduction of reported pain. The duration of action was equal among the solutions regardless of anesthetic concentration. CONCLUSIONS: A solution of 0.091% lidocaine with epinephrine 1:1,100,000 and 0.82% benzyl alcohol reduces medication dose while ensuring maximum patient comfort and, theoretically, increases shelf life. While considered off-label, clinically effective dermal anesthesia may be obtained at a lower concentration of lidocaine and epinephrine than is commonly used, aiding conservative use of local anesthetic, particularly during times of national shortage. J Drugs Dermatol. 2023;22(4): doi:10.36849/JDD.5183 Citation: Moses A, Klager S, Weinstein A, et al. A comparative analysis of local anesthetics: Injection associated pain and duration of anesthesia. J Drugs Dermatol. 2023;22(4):364-368. doi:10.36849/JDD.5183.

Successful systemic treatment outcomes of lichen planus: A single-center retrospective review.

Lichen planus (LP) affects up to 4% of adults and can cause significant distress and morbidity, especially to those with persistent disease. As many as 20% of patients with LP may exhibit widespread or recalcitrant disease necessitating systemic treatment options. We sought to evaluate the effectiveness of systemic treatments for severe and recalcitrant LP not responsive to topical corticosteroids or calcineurin inhibitors. Over a 10-year period, 374 patients with cutaneous and mucosal LP were evaluated at a major regional tertiary medical center; 94 qualified for inclusion in the study. In all, 26 (28%) patients achieved remission, 52 (55%) experienced stable disease control, and 16 (17%) failed all attempted treatments. Among medications most trialed, intramuscular triamcinolone (IM TAC), hydroxychloroquine, and methotrexate were most successful with 79%, 61%, and 42% respective response rates. In contrast, oral corticosteroids and dapsone were less frequently successful at rates of 24% and 20%. IM TAC represented the highest level of treatment success and was statistically significant compared to other systemic treatments (P < .01). Among adjuvant therapies, intralesional triamcinolone (IL TAC) demonstrated higher success (71%) than oral corticosteroids (29%). Based on this multi-year evaluation, we recommend that clinicians consider IM TAC as a first-line systemic option for severe or refractory LP, with hydroxychloroquine as the steroid-sparing treatment of choice. For patients requiring adjuvant therapy, IL TAC should be considered to hasten response and symptom relief. Patients with severe or widespread disease may benefit from earlier initiation of systemic therapy to prevent significant morbidity and impact on daily function.

Pediatric epidermolysis bullosa acquisita: A review.

Epidermolysis bullosa acquisita (EBA) is an acquired autoimmune blistering skin disorder that is rare in adults and even rarer in childhood. This review aims to identify cases of pediatric EBA and report their clinical features and course. Our literature review was conducted in MEDLINE® using the search terms related to juvenile epidermolysis bullosa acquisita. We identified 40 cases of pediatric EBA. Mucosal tissues were affected in 29 out of 40 cases. Treatment mostly consisted of a systemic corticosteroid combined with dapsone. Prognosis is favorable with 17 of 40 cases achieving complete remission, 9 of 40 with complete control with therapy, 12 of 40 with partial control with therapy, 1 of 40 with no response to therapy, and 1 of 40 terminating treatment early. Though it is a rare condition, childhood EBA should still be included in the differential diagnosis of pediatric blistering diseases.

Cutaneous T-cell lymphoma mimicking palmoplantar pustular psoriasis: A rare variant.

The development of pustular cutaneous T-cell lymphoma (CTCL) on the palms and soles is rare. Without confirmatory biopsy and molecular studies, CTCL can be misdiagnosed as many benign inflammatory skin diseases. We present a case of cutaneous T-cell lymphoma (CTCL) that mimicked palmoplantar pustular psoriasis, a rarely reported manifestation of the disease. We stress the importance of biopsy to confirm diagnoses, especially when preliminary diagnoses do not respond to empiric treatment.

The use of rituximab in treatment of epidermolysis bullosa acquisita: Three new cases and a review of the literature.

Epidermolysis bullosa acquisita (EBA) is a rare, subepidermal blistering disease affecting the skin and mucous membranes that often remains refractory to standard immunosuppressive therapy. We present three original cases and a review of the literature of 20 cases of refractory EBA treated with rituximab as monotherapy or in combination with other agents. Complete control (with or without therapy) and remission were seen in 56% of patients treated with rituximab monotherapy and 75% of patients treated with rituximab and immunoadsorption (IA). We conclude EBA refractory to standard immunosuppressive therapy may show a more favorable long-term response to the addition of rituximab; and rituximab in combination with intravenous immunoglobulin or IA may provide utility in terminating acute disease. Additional data are needed to evaluate the safety and long-term outcomes of rituximab-based treatment.

Pediatric ocular lichen planus and lichen planopilaris: One new case and a review of the literature.

When lichen planus involves the scalp, it is known as lichen planopilaris, and when it involves the eye, it is known as ocular lichen planus; both are rare. Early detection and targeted therapy are crucial in preventing hair loss and scarring conjunctivitis. Little is known regarding appropriate treatment for lichen planopilaris. The objective of this case study is to present a new case of pediatric ocular lichen planus and lichen planopilaris and to identify all reported cases of pediatric lichen planopilaris, highlighting disease involvement, treatment, and response to treatment.

Perforating disorder secondary to leflunomide and review of the literature of medications associated with perforating disorder.

The perforating dermatoses are a group of disorders characterized by transepidermal elimination of a material from the upper dermis. The most widely accepted classification consists of four primary perforating disorders that are defined by the type of material eliminated and the type of epidermal disruption. Pathogenesis of the perforating dermatoses is poorly understood, but some appear to have a genetic component. There are also acquired forms, which have been associated with underlying systemic diseases and the use of certain drugs. In this report, we describe a perforating disorder that occurred secondary to leflunomide therapy. To our knowledge, this is the first case in which this has been reported. We also review the recent literature on medications associated with perforating disorders.

Successful treatment with IVIg therapy of diabetes-associated scleredema severe progressive case and review of the literature.

Scleredema is a deposition disorder which presents as diffuse, symmetric, non-pitting skin induration from dermal deposits of glycosaminoglycans ("mucins"). It classically affects the upper back and posterior neck, often causing skin tightness and decreased range of motion. In most patients the clinical course is chronic and treatment options are limited. We report a case of a patient diagnosed with scleredema associated with longstanding insulin dependent diabetes who was treated successfully with IVIg. Additionally, we have reviewed the literature reporting other patients with scleredema treated with IVIg.

Treatment of antiviral-resistant recurrent erythema multiforme with dapsone.

Recurrent erythema multiforme (REM) is a chronic disease characterized by frequent episodes of target cutaneous lesions in an acral distribution. Conventional treatment includes systemic corticosteroids and antiviral therapy. The aim of this study was to evaluate dapsone as a potential steroid sparing-agent for the treatment of REM after a failed trial of at least one antiviral therapy (acyclovir, famciclovir, or valacyclovir). A retrospective chart review was conducted on thirteen patients with a diagnosis of REM who underwent treatment with dapsone after failing at least one antiviral therapy. Out of 13 patients, 6 showed complete response (CR) and 5 showed partial response (PR). The underlying cause was identified in 5 patients with all showing at least PR. Adverse effects, observed in 4 patients, included fatigue, macrocytic anemia, anxiety, insomnia and involuntary movements, and drug-induced lupus erythematosus. A continuous course of dapsone, titrated up from 25 mg/day to a dose at which clinical improvement is seen with acceptable patient tolerance, is a viable steroid sparing-agent for REM treatment after a failed trial of antiviral therapy.

Woringer-Kolopp (Pagetoid Reticulosis) disease successful response to bexarotene gel.

Woringer-Kolopp (WK) is a rare subtype of cutaneous T-cell lymphoma (CTCL) with limited treatment options. Bexarotene gel is a topical retinoid used in the treatment of CTCL. This report describes three female patients (mean age 66 years) with WK disease who had an effective treatment response to bexarotene 1% gel. This treatment could provide a safe alternative to other current treatment modalities which have higher risks of potential adverse effects and lack of access to other conventional treatments such as light therapy.

Features of coexisting granulomatosis with polyangiitis in a patient with pemphigus vulgaris: a case report.

Pemphigus vulgaris (PV) and granulomatosis with polyangiitis (Wegener's or GPA) are two rare autoimmune disorders. Both can involve the upper airways, and diagnosis can be difficult in the absence of extra-airway symptoms. We report the case of a patient with well-controlled PV but persistent upper respiratory tract symptoms. Further evaluation revealed perforation of the nasal septum and elevated serologies consistent with GPA. The patient improved with rituximab treatments. This case demonstrates that alternative concomitant diagnosis should be considered in patients with symptoms suggestive of recalcitrant PV and/or GPA, since these patients may require more aggressive initial treatment.

Histiocytoid Sweet syndrome treated with azathioprine: a case report.

Histiocytoid Sweet syndrome (HSS) is a rare histologic variation of Sweet syndrome (SS) predominantly exhibiting mononuclear histiocytoid cells instead of neutrophils. We report a 22-year-old woman with HSS, who, after minimal improvement with colchicine and dapsone, had significant improvement of her cutaneous eruption and systemic symptoms following empiric treatment with azathioprine. Since azathioprine has historically been known to cause SS, this case highlights a previously unreported treatment response for the histiocytoid variant.

Mycophenolate mofetil therapy for pediatric bullous pemphigoid.

Bullous pemphigoid (BP) is a common autoimmune blistering disease in the adult population, but extremely rare in the pediatric population. Childhood BP usually has a favorable prognosis and responds well to topical and oral steroids. However, for patients that do not respond to corticosteroids, therapeutic alternatives are scarce. We report a case of a toddler with recalcitrant BP who was successfully treated with mycophenolate mofetil (MMF).

Rare Autoinflammatory Neutrophilic Dermatoses in Pregnancy: Literature Review.

Rare cases of autoinflammatory neutrophilic dermatoses (AINDs) have been reported in patients during pregnancy with associated adverse maternal and fetal outcomes. Due to the rarity and heterogeneous morphology of pregnancy-associated AINDs, clinical diagnosis is often overlooked, and treatment options are limited. In this review, we present the epidemiology, clinical characteristics, therapeutic interventions, maternal and fetal outcomes, and discuss the possible pathophysiology of various pregnancy associated AINDs. Risk factors for the onset and exacerbation of AINDs in pregnancy include older maternal age, disease duration, and specific gestational age. The varied disease courses and conflicting clinical outcomes in both mothers and fetuses demonstrate the importance of symptom recognition and the understanding of the role of pregnancy on AINDs.