Search

ImportanceTrimethoprim-sulfamethoxazole (TMP-SMX) hypersensitivity reaction, ranging from circulatory shock to aseptic meningitis and respiratory failure, is a potentially life-threatening condition with dermatologic relevance.ObjectiveTo describe the mucocutaneous findings and clinical features of TMP-SMX hypersensitivity reaction.Design, Setting, and ParticipantsThis was a retrospective case series study of 7 patients who developed a characteristic rash, hemodynamic changes, and end-organ dysfunction after treatment with TMP-SMX at a large university hospital system during January 2013 to March 2022.ExposuresTreatment with TMP-SMX within 2 weeks of the reaction.Main Outcome and MeasuresDescriptions of the condition, including the demographic information of the affected population, the reaction timeline, and mucocutaneous and clinical features.ResultsThe cohort comprised 7 patients (median [range] age, 20 [15-66] years; 4 female and 3 male). The most common mucocutaneous findings were generalized sunburn-like erythema without scale, conjunctivitis, and mild facial and acral edema. Three patients had previous exposure to TMP-SMX and developed symptoms in 1 day or less, while those without prior exposure presented from 4 to 11 days after drug initiation. Among the 7 patients, 6 had fever, 7 had hypotension, and 7 had tachycardia. All patients had lymphopenia and evidence of end-organ dysfunction with either kidney or liver involvement. Median (range) time to resolution was 72 (48-96) hours.Conclusions and RelevanceThis retrospective case series indicates that SCoRCH (sudden conjunctivitis, lymphopenia, and rash combined with hemodynamic changes) should be considered in the differential diagnosis of patients presenting with acute generalized sunburn-like erythema, conjunctivitis, systemic symptoms, and hemodynamic changes in the setting of recent TMP-SMX use.

ImportanceScoring systems for Stevens-Johnson syndrome and epidermal necrolysis (EN) only estimate patient prognosis and are weighted toward comorbidities and systemic features; morphologic terminology for EN lesions is inconsistent.ObjectivesTo establish consensus among expert dermatologists on EN terminology, morphologic progression, and most-affected sites, and to build a framework for developing a skin-directed scoring system for EN.Evidence ReviewA Delphi consensus using the RAND/UCLA appropriateness criteria was initiated with a core group from the Society of Dermatology Hospitalists to establish agreement on the optimal design for an EN cutaneous scoring instrument, terminology, morphologic traits, and sites of involvement.FindingsIn round 1, the 54 participating dermatology hospitalists reached consensus on all 49 statements (30 appropriate, 3 inappropriate, 16 uncertain). In round 2, they agreed on another 15 statements (8 appropriate, 7 uncertain). There was consistent agreement on the need for a skin-specific instrument; on the most-often affected skin sites (head and neck, chest, upper back, ocular mucosa, oral mucosa); and that blanching erythema, dusky erythema, targetoid erythema, vesicles/bullae, desquamation, and erosions comprise the morphologic traits of EN and can be consistently differentiated.Conclusions and RelevanceThis consensus exercise confirmed the need for an EN skin-directed scoring system, nomenclature, and differentiation of specific morphologic traits, and identified the sites most affected. It also established a baseline consensus for a standardized EN instrument with consistent terminology.

Background Plasma cell vulvitis (PCV) is an inflammatory vulvar dermatosis that is not well characterized. Diagnosis is often delayed, and the condition can be refractory to treatment. To date, there are no systematic reviews on this topic. Objective This study aimed to provide a systematic review of PCV, including epidemiologic, clinical, and histopathologic findings, as well as associated comorbidities and treatment options. Methods A primary literature search was conducted using the PubMed, Ovid Medline, Cochrane, and CINAHL databases. Results Fifty-three publications with 196 patients (mean age: 55.3 ± 14.5 years) were included. The majority of studies were case reports and case series. Common symptoms included burning/stinging (52%), dyspareunia (44%), and pruritus (41%). Common findings included erythema (84%), glistening/shiny appearance (29%), well-demarcated lesions (25%), and erosions (22%). Common anatomic sites were the labia minora (45%), introitus (31%), and periurethral (19%). Fifty-three percent of patients had a solitary lesion. Common histologic findings were a predominant plasma cell infiltrate (88%), presence of other inflammatory cells (55%), hemosiderin/siderophages (46%), and epidermal atrophy (43%). Topical corticosteroids (64%) and tacrolimus ointment (13%) were the most frequent treatment modalities. In most reports, previous treatments were tried, and there was a diagnostic delay. Conclusion PCV is likely underrecognized and should be considered in patients with erythema of the mucous and modified mucous membranes, symptoms of burning or stinging, and a predominant plasma cell infiltrate on histopathology. First-line therapy should begin with high-potency topical corticosteroids, with the most evidence for clobetasol 0.05% or tacrolimus 0.1% ointment. Prospective studies are needed to further characterize this condition and to develop treatment guidelines.

The prevalence of acute vulvovaginal involvement in toxic epidermal necrolysis can be as high as 70%; up to 28% of female patients will also develop chronic vulvovaginal sequelae. There is little consensus regarding prevention and treatment of the gynecologic sequelae of both Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN). We review acute and chronic sequelae, including erosions, scar formation, chronic skin changes, urethral complications, adenosis, malignant transformation, vulvodynia, and dyspareunia. We provide comprehensive recommendations for acute and long-term vulvovaginal care in adult and pediatric SJS/TEN patients. Treatment should include an ultrapotent topical steroid, followed by a nonirritating barrier cream applied to vulvar and perineal lesions. A steroid should be used intravaginally along with vaginal dilation in all adults (but should be avoided in prepubertal adolescents) with vaginal involvement. Menstrual suppression should be considered in all reproductive age patients until vulvovaginal lesions have healed. Last, referrals for pelvic floor physical therapy and to surgical subspecialties should be offered on a case-by-case basis. This guide summarizes the current available literature combined with expert opinion of both dermatologists and gynecologists who treat a high volume of SJS/TEN patients.

Background Patient outcomes are improved when dermatologists provide inpatient consultations. Inpatient access to dermatologists is limited, illustrating an opportunity to use teledermatology. Little is known about the ability of dermatologists to accurately diagnose disease and manage inpatients with teledermatology, particularly when using nondermatologist-generated clinical data. Methods This prospective study assessed the ability of teledermatology to diagnose disease and manage 41 dermatology consultations from a large urban tertiary care center, using internal medicine referral documentation and photographs. Twenty-seven dermatology hospitalists were surveyed. Interrater agreement was assessed by the κ statistic. Results There was substantial agreement between in-person and teledermatology assessment of the diagnosis with differential diagnosis (median κ = 0.83), substantial agreement in laboratory evaluation decisions (median κ = 0.67), almost perfect agreement in imaging decisions (median κ = 1.0), and moderate agreement in biopsy decisions (median κ = 0.43). There was almost perfect agreement in treatment (median κ = 1.0), but no agreement in follow-up planning (median κ = 0.0). There was no association between raw photograph quality and the primary plus differential diagnosis or primary diagnosis alone. Limitations Selection bias and single-center nature. Conclusions Teledermatology may be effective in the inpatient setting, with concordant diagnosis, evaluation, and management decisions.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening conditions with high morbidity and mortality. Supportive care management of SJS/TEN is highly variable. A systematic review of the literature was performed by dermatologists, ophthalmologists, intensivists, and gynecologists with expertise in SJS/TEN to generate statements for supportive care guideline development. Members of the Society of Dermatology Hospitalists with expertise in SJS/TEN were invited to participate in a modified, online Delphi-consensus. Participants were administered 9-point Likert scale questionnaires regarding 135 statements. The RAND/UCLA Appropriateness Method was used to evaluate and select proposed statements for guideline inclusion; statements with median ratings of 6.5 to 9 and a disagreement index of ≤1 were included in the guideline. For the final round, the guidelines were appraised by all of the participants. Included are an evidence-based discussion and recommendations for hospital setting and care team, wound care, ocular care, oral care, urogenital care, pain management, infection surveillance, fluid and electrolyte management, nutrition and stress ulcer prophylaxis, airway management, and anticoagulation in adult patients with SJS/TEN.

Patients with vulvar dermatoses often delay seeking medical treatment because of anxiety and embarrassment. Moreover, women frequently self-treat with various home remedies and see multiple clinicians before presenting to a dermatologist. Despite serving as the primary providers for patients with vulvovaginal symptoms, gynecologists typically receive limited training in the causes and management of these conditions. Dermatologists are experts in the evaluation and management of cutaneous disease and should be the caretakers of all skin, including the genitalia. Vulvar disorders are underrecognized by dermatologists for numerous reasons: inadequate training, lack of comfort with both interview and examination techniques, and unfamiliarity with normal anatomic variations. The first article in this continuing medical education series on vulvar dermatoses reviews the fundamentals, approach, and techniques that can be used to ensure a successful visit for both patient and provider.

The most problematic vulvovaginal conditions are familiar to dermatologists but may exhibit distinct clinical features or medication management because of the anatomic location. The second article in this continuing medical education series focuses on management pearls for treating vulvar diseases. We highlight key conditions, such as lichen sclerosus, erosive lichen planus, and vulvodynia. In addition, we review conditions that dermatologists may be less familiar with, such as plasma cell vulvitis, desquamative inflammatory vaginitis, vulvar aphthae, and low estrogen states. Nearly 1 in 6 women experience undiagnosed and untreated vulvovaginal discomfort at some point in their lives. Physicians who treat vulvar disorders will improve the quality of life of countless women.

Vulvar lichen sclerosus (vLS) is an inflammatory skin condition that predominantly affects the vulvar and perianal regions. Approximately 50% of cases present prior to menopause; however, there is a paucity of data on vLS in women of reproductive age as well as during their pregnancies. A retrospective review was performed at two tertiary referral centers to better describe cases of vLS in women of reproductive age. Thirty-three patients with a mean age of 40 years met inclusion criteria. In this group, vulvar pruritus was the most common presenting symptom (52%); 61% had biopsy-proven vLS, 42% had at least one autoimmune condition, 21% had comorbid depression or anxiety, 33% were given an incorrect diagnosis prior to vLS, and 42% had documented nonadherence to topical steroids. Among the eight patients who became pregnant, four had cesarean deliveries and 63% were symptomatic during pregnancy. When treating a woman who presents with vulvar pruritus or skin changes, vLS should be considered.

Background: Maintenance of Certification (MOC) is a recertification program required by the American Board of Dermatology (ABD). The program was created because continuing medical education (CME) was viewed as inadequate in keeping physicians’ knowledge up to date. In July 2016, UTSW incorporated MOC content into Grand Rounds. Objective: To identify whether dermatologists at UTSW find MOC Grand Rounds to be useful and whether this process could be utilized in other institutions. Methods: A five question survey was piloted by a few dermatologists at UTSW. The survey was emailed to dermatologists who attended the MOC Grand Rounds. Three questions were scored on a scale of 1-6 (1= strongly disagree, 2=moderately disagree, 3= neutral, 4= mildly agree, 5= moderately agree, 6=strongly agree) with 2 free response questions. Results: 13/20 dermatologists responded to the survey (65% response rate). 11/13 dermatologists (84.6%) reported that MOC at Grand Rounds is valuable to them. 11/13 dermatologists (84.6%) reported that other dermatology programs should incorporate this method of obtaining MOC credit. 9/13 dermatologists (69.2%) reported audience response sessions improve the Grand Rounds experience. Conclusion: Dermatologists found the MOC content to be useful when incorporated into Grand Rounds.

Basal cell carcinoma (BCC) is characterized by slow but locally invasive growth. Although there is low metastatic potential, if not treated early, these skin cancers can lead to significant morbidity and mortality. In this case report, we present a man with a neglected BCC that developed into what is termed a giant BCC or one that is greater than 5 cm. This tumor was discovered only upon workup of orthostatic lightheadedness and iron deficiency anemia. Although rare, basal cell carcinoma must be included on the differential of a large cutaneous lesion and may be a source of significant blood loss.

Vulvar squamous cell carcinoma (VSCC) pathogenesis is traditionally defined by the presence or absence of human papillomavirus (HPV), but the definition of these groups and their molecular characteristics remains ambiguous across studies. Here, we present a retrospective cohort analysis of 36 patients with invasive VSCC where HPV status was determined using RNA in situ hybridization (ISH) and polymerase chain reaction (PCR). Clinical annotation, p16 immunohistochemistry (IHC), programmed death ligand-1 (PD-L1) IHC, HPV16 circular E7 RNA (circE7) detection, and RNA-sequencing (RNA-seq) of the cases was performed. A combination of ISH and PCR identified 20 cases (55.6%) as HPV-positive. HPV-status did not impact overall survival (HR: 1.36, 95% CI: 0.307 to 6.037, p=0.6857) or progression-free survival (HR: 1.12, 95% CI: 0.388 to 3.22, p=0.8367), and no significant clinical differences were found between the groups. PD-L1 expression did not correlate with HPV status, but increased expression of PD-L1 correlated with worse overall survival. Transcriptomic analyses (n=23) revealed distinct groups, defined by HPV status, with multiple differentially expressed genes previously implicated in HPV-induced cancers. HPV-positive tumors showed higher global expression of endogenous circular RNAs (circRNAs), including several circRNAs that have previously been implicated in the pathogenesis of other cancers.

Dermatologists are frequently called to evaluate patients with urticaria in the emergency room, urgent care clinics, and hospitals. Many acute urticarial eruptions will resolve without long-term sequelae; however, there are red flags that clinicians must be aware of. First-line treatment for acute urticaria is regular dose H1 antagonists; however, the dose can be increased up to fourfold for refractory disease. Short courses of corticosteroids should be avoided as rebound urticaria is common upon discontinuation. Urticarial vasculitis presents with persistent, atypical urticaria, burning, and residual lesions. The most common extra-cutaneous manifestation of urticarial vasculitis is musculoskeletal involvement. Schnitzler syndrome is a rare, severe condition but new evidence provides promise for use of biologic therapies. Acute spontaneous urticarial eruptions are commonly encountered in hospitalized patients. This review provides readers with the tools needed to delineate benign eruptions from more concerning conditions.

Lichen planus is an inflammatory mucocutaneous condition with a myriad of clinical manifestations. There are 3 forms of lichen planus that effect the vulva: papulosquamous, hypertrophic, and erosive. Erosive lichen planus can progress to vulvar scaring, vaginal stenosis, and squamous cell carcinoma; these long-term sequelae cause sexual distress, depression, and decreased quality of life for patients. Diagnosis is often delayed because of patient embarrassment or clinician misdiagnosis. Early recognition and treatment is essential to decreasing the morbidity of this condition. Multimodal treatment, along with a multidisciplinary approach, will improve outcomes and further clinical advances in studying this condition.

We present the case of a 28-year-old male with a history of HIV with a 1-month history of a steadily enlarging, firm painful lesion on the right posterior shoulder. The patient was initially treated for cellulitis given his clinical picture. Histopathologic examination revealed an angiocentric and dermal proliferation of markedly atypical lymphoid cells with numerous mitoses and apoptotic bodies along with broad zones of necrosis. Biopsy revealed the presentation to be consistent with NK/T-cell lymphoma. The cutaneous lesions from NK/T-cell lymphoma can often be initially mistaken for cellulitis, therefore this malignancy should be included on the differential in a patient HIV/AIDS.

The most problematic vulvovaginal conditions are familiar to dermatologists but may exhibit distinct clinical features or medication management because of the anatomic location. The second article in this continuing medical education series focuses on management pearls for treating vulvar diseases. We highlight key conditions, such as lichen sclerosus, erosive lichen planus, and vulvodynia. In addition, we review conditions that dermatologists may be less familiar with, such as plasma cell vulvitis, desquamative inflammatory vaginitis, vulvar aphthae, and low estrogen states. Nearly 1 in 6 women experience undiagnosed and untreated vulvovaginal discomfort at some point in their lives. Physicians who treat vulvar disorders will improve the quality of life of countless women.

Patients with vulvar dermatoses often delay seeking medical treatment because of anxiety and embarrassment. Moreover, women frequently self-treat with various home remedies and see multiple clinicians before presenting to a dermatologist. Despite serving as the primary providers for patients with vulvovaginal symptoms, gynecologists typically receive limited training in the causes and management of these conditions. Dermatologists are experts in the evaluation and management of cutaneous disease and should be the caretakers of all skin, including the genitalia. Vulvar disorders are underrecognized by dermatologists for numerous reasons: inadequate training, lack of comfort with both interview and examination techniques, and unfamiliarity with normal anatomic variations. The first article in this continuing medical education series on vulvar dermatoses reviews the fundamentals, approach, and techniques that can be used to ensure a successful visit for both patient and provider.

A 56-year-old man presented with a 2-day history of a painful rash. Examination revealed necrotic retiform purpura over the trunk and extremities (figure 1). Laboratory testing was notable for leucopenia (3.2 cells/µL) and positive peripheral antineutrophil cytoplasmic antibody (p-ANCA) with antimyeloperoxidase specificity (3.5 U, normal

Fixed drug eruptions (FDE) comprise 10 percent of alladverse cutaneous drug reactions and generalizedbullous fixed drug eruptions (GBFDE) are a raresubset of FDEs. We present a patient with severeGBFDE caused by ibuprofen successfully treated withcyclosporine. Further work is needed to determine ifcyclosporine can be an effective therapy for GBFDE.

Disseminated Gonococcal Infection A 20-year-old woman presented with erythematous pustules on her arms and legs and joint pain in both ankles. Blood cultures were positive for Neisseria gonorrhoeae.