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.

A Recalcitrant Case of Senear-Usher Syndrome Treated With Rituximab.

A 51-year-old uninsured, otherwise healthy male who works in the fishing industry presented with a two-month history of pruritic scaly plaques on his face, scalp, and trunk and mild photosensitivity. A biopsy of a scalp lesion revealed acantholysis consistent with pemphigus foliaceus. Laboratory testing demonstrated elevated anti-desmoglein 1, positive antinuclear antibodies (ANA and anti-dsDNA), and elevated Sjögren's anti-SS-A antibodies. The patient was diagnosed with pemphigus erythematosus. The patient was not optimally responsive and was unable to discontinue systemic corticosteroids despite a maximum dosage of mycophenolate mofetil of 3000 mg/day. Hence, rituximab was added as a rescue treatment with the rheumatoid arthritis protocol. Three months after starting rituximab, there was a marked improvement in symptoms with complete resolution of cutaneous lesions.

Localized Pemphigus Foliaceus: Diverse Presentations, Treatment, and Review of the Literature.

The clinical presentation of localized pemphigus foliaceus (PF) often involves photo exposed areas. We describe five cases of localized PF, two of which were rare locations for the disease in non-photo exposed areas, namely the genitalia and back. Patients were treated with topical corticosteroids and calcineurin inhibitors as well as systemic treatment with corticosteroids and dapsone. Each patient responded to treatment, with two achieving remission. No relapses occurred in any of these cases over a mean follow-up time of 3.7 years. A review of the English literature using MEDLINE® yielded 18 reported cases of localized PF. Most occurred in photo exposed areas such as the nose, cheeks, scalp, and other areas of the face. Two patients progressed to generalized involvement without treatment. Treatment regimens had much variation and included both topical and systemic agents. Localized PF is rare, and our findings suggest it may be controlled with topical therapy and systemic dapsone.

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.

Certolizumab-induced lichenoid eruption in a patient with rheumatoid arthritis.

Certolizumab is a monoclonal antibody against tumour necrosis factor-alpha (TNF-α) commonly used in rheumatologic conditions such as rheumatoid arthritis. Skin rashes are an uncommon side effect with few cases of lichenoid drug eruption reported in the literature. We describe a patient with rheumatoid arthritis who presented 6 weeks after initiating certolizumab pegol. Physical examination showed pink-to-violaceous papules on her upper and lower extremities. Biopsy confirmed a lichenoid drug eruption. The medication was discontinued and she was treated with topical steroids and a calcineurin inhibitor, with resolution of her lesions. Clinicians should be cognizant of such adverse reactions to TNF-α inhibitors and keep drug-induced lichenoid eruptions on the differential. Lichenoid eruptions induced by certolizumab pegol may affect the skin and/or mucous membranes. While most cases occur within weeks to months of starting therapy, eruptions may occur years after treatment initiation, underscoring the importance of a thorough review of medications.

Spironolactone-Induced Lichenoid Drug Reaction and Subsequent Diffuse Eruptive Squamous Cell Carcinomas Successfully Treated With Systemic Methotrexate.

Antihypertensive agents such as spironolactone have been reported to cause lichenoid drug eruptions. Eruptive keratoacanthomas (KA), considered to be well-differentiated squamous cell carcinoma (SCC), may develop in the setting of such lichenoid reactions. Thus, definitive treatment is imperative. This case report describes a patient on spironolactone who developed a lichenoid drug eruption followed by eruptive KAs and SCC. The treatment approach used systemic methotrexate. While most treatment regimens for widespread eruptive KA/SCC employ intralesional methotrexate, this case demonstrated the utility of its systemic counterpart. This may have also facilitated the resolution of the patient's lichenoid eruption. There are only three other reports in the literature describing a spironolactone-induced lichenoid drug eruption. Further investigations are needed to evaluate the adverse cutaneous effects of spironolactone as well as the efficacy of systemic methotrexate in treating patients with a significant number of SCCs arising from lichenoid drug eruptions.

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.

Rituximab as an Adjuvant Rescue Treatment for Ocular Cicatricial Pemphigoid.

PURPOSE: Ocular cicatricial pemphigoid (OCP) can lead to devastating ocular complications without prompt treatment. A number of immunomodulatory agents have been attempted with varying success. The objective of this study was to evaluate the use of rituximab as an adjuvant to immunomodulatory treatments (IMTs) in refractory OCP. METHODS: The clinical records of 14 patients with treatment refractory OCP treated with rituximab as monotherapy or in combination with IMTs were retrospectively reviewed, with a focus on demographics, treatments prerituximab and postrituximab, total number of rituximab infusions, response to treatment, and ocular outcomes including staging and best-corrected visual acuity. RESULTS: Thirteen patients (92.9%) achieved a complete response with rituximab over a mean period of 4.3 months. The average sustained complete response time in those without relapse was 29.7 months. Five patients relapsed over a mean period of 15 months, 2 of whom were able to regain control over a mean of 2.5 months with additional rituximab treatments. At the final evaluation, rituximab-based therapy improved ocular outcomes in OCP by stabilizing Foster staging and preventing the deterioration of best-corrected visual acuity in 72% of eyes. In total, 90% of eyes with Foster stages 3 or less did not progress. All patients were able to decrease IMT dosage and/or transition to less potent adjuvant treatments. CONCLUSIONS: The consideration of rituximab earlier in treatment of OCP as a rescue and/or maintenance therapy could result in an earlier arrest of disease progression, resulting in preservation of patients' vision, and enable tapering of adjuvant IMT.

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.

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.

Combination Therapy of Plasma Exchange and Rituximab to Treat Cicatricial Pemphigoid and Bullous Pemphigoid.

The pemphigoid group of subepidermal autoimmune blistering diseases can affect both cutaneous and mucosal tissues. Therapy of this group of diseases, including cicatricial pemphigoid (CP) and bullous pemphigoid (BP), consists of systemic steroids and immunomodulatory agents. Recalcitrant cases have typically been treated with plasmapheresis or rituximab individually. This report describes two patients with severe, rapidly progressive CP and BP refractory to high-dosage systemic steroids and immunomodulatory agents. Both patients were treated with a combination of plasmapheresis and rituximab. In addition to these cases, one retrospective study showed the effectiveness of other immunosuppressants in combination with plasmapheresis in 17 patients with pemphigus refractory to corticosteroids and immunosuppressants alone. No major adverse events occurred in the study. Similar studies employing immunoadsorption and rituximab with various combinations of intravenous immune globulin (IVIg), corticosteroids, and other conventional immunosuppressants have shown promising results in other autoimmune blistering diseases. The successful response in the patients described here, as well as those described in the literature who underwent similar management, provides a possible combination treatment option for patients with severe, recalcitrant pemphigoid. A further trial with a larger group of pemphigoid patients is warranted.

The Effect of Abobotulinum Toxin A on the Symptoms of Raynaud's Phenomenon: A Case Series.

Raynaud's phenomenon (RP) is a relatively common syndrome occurring alone or in combination with autoimmune and inflammatory diseases. It is characterized by pain and ulceration due to vasospasm in response to cold and stress, most often affecting the digits. Although pharmacologic treatment for this condition exists, it is not always efficacious. Our case series demonstrates the use of abobotulinum toxin A in the treatment of RP. We report the cases of four patients who received injections of abobotulinum toxin A to treat their mild to severe RP symptoms. They experienced clinical improvement for up to one year after treatment.

Efficacy of intralesional sodium thiosulfate for the treatment of dystrophic calcinosis cutis: A double-blind, placebo-controlled pilot study.

BACKGROUND: Intralesional injection of sodium thiosulfate has emerged as a promising therapy for calcinosis cutis, but to our knowledge there are no randomized controlled trials evaluating its efficacy as a treatment. OBJECTIVE: Conduct a prospective, double-blinded investigation of intralesional sodium thiosulfate versus normal saline in the treatment of dystrophic calcinosis cutis. METHODS: This prospective pilot study injected normal saline or sodium thiosulfate at 0.1 mL/cm2 into lesions at baseline and at 1- and 2-month follow-up. Subjects were followed for a total of 12 weeks. An in-person Physician Global Assessment score was assigned by the injecting physician at each visit and was repeated by an independent observer. RESULTS: Of 4 subjects who completed the study, only 1 experienced improvement in the size and Physician Global Assessment score of the lesion. By 3-month follow-up, there was no difference between the average size of the treatment and control lesions (P = .39). LIMITATIONS: This was a small single-center study with limited demographic diversity and a short follow-up period. Only dystrophic calcinosis cutis subjects were included, and subjects received only 3 monthly injections of sodium thiosulfate. CONCLUSIONS: With only 1 positive response, our results highlight the need for further study of sodium thiosulfate treatment for dystrophic calcinosis.