Drug patch testing in Stevens-Johnson syndrome and toxic epidermal necrolysis: A systematic review.

BACKGROUND: The data on patch testing (PT) to identify culprit medications in Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) are limited to scattered case reports and small case series, without analysis of overall trends to inform clinicians of its utility, methodology, and safety. OBJECTIVE: To conduct a systematic review of the practice of PT in SJS/TEN, quantify the positivity rate of common drug classes, and assess safety during testing. METHODS: PubMed was searched from inception to 2021. Search terms included "patch testing" AND "SJS" OR "TEN" OR "Stevens-Johnson syndrome" OR "toxic epidermal necrolysis" OR "Lyell's syndrome." RESULTS: There were 58 articles that met the inclusion criteria. In total, 82 patients underwent patch testing for SJS/TEN, resulting in 104 positive reactions to 49 unique medications. Antiepileptic drugs were responsible for 48.1% of the positive reactions; antibiotics, 28.8%; and nonsteroidal anti-inflammatory drugs, 6.7%. The positivity rates of antiepileptics, antibiotics, and nonsteroidal anti-inflammatory drugs were 33.1%, 13.1%, and 21.9%, respectively. When accounting for suspected causality, these rates increased to 54.3%, 78.4%, and 54.5%, respectively. Three patients (3.7%), 2 of whom had human immunodeficiency virus infection and active tuberculosis, experienced systemic reactions during PT, which required only conservative treatment. CONCLUSION: Published reports suggest that PT in SJS/TEN is useful and safe. Antiepileptic drugs have been tested most frequently and found to have the highest positivity rate. There is a critical need for large-scale studies with standardized methodology to obtain reproducible data on PT in SJS/TEN.

Evaluation of a Case Series of Patients With Generalized Pustular Psoriasis in the United States.

IMPORTANCE: Generalized pustular psoriasis (GPP) is a chronic, orphan disease with limited epidemiological data. OBJECTIVE: To describe the clinical characteristics, treatments, longitudinal disease course, and disease-specific health care utilization among patients with GPP across the United States. DESIGN, SETTING, AND PARTICIPANTS: A retrospective longitudinal case series involving 95 adults who met the European Rare and Severe Psoriasis Expert Network consensus definition for GPP and were treated at 20 US academic dermatology practices between January 1, 2007, and December 31, 2018. MAIN OUTCOMES AND MEASURES: The primary outcome is to describe the patient characteristics, associated medical comorbidities, treatment patterns complications, and GPP-specific health care utilization. RESULTS: Sixty-seven of 95 patients (70.5%) were women (mean age, 50.3 years [SD, 16.1 years]). In the initial encounter, 35 patients (36.8%) were hospitalized and 64 (67.4%) were treated with systemic therapies. In total, more than 20 different systemic therapies were tried. During the follow-up period, 19 patients (35.8%) reported hospitalizations at a median rate of 0.5 hospitalizations per year (IQR, 0.4-1.6). Women had a decreased risk of an emergency department or hospital encounter (odds ratio, 0.19; 95% CI, 0.04-0.83). CONCLUSIONS AND RELEVANCE: Generalized pustular psoriasis is a rare, chronic disease without standard treatment and is associated with continued health care utilization over time.

Evaluation of a Case Series of Patients With Palmoplantar Pustulosis in the United States.

IMPORTANCE: Palmoplantar pustulosis (PPP) is a is a chronic, orphan disease with limited epidemiological data. OBJECTIVE: To describe the clinical characteristics, treatments, longitudinal disease course, and health care utilization in adults with PPP across the US. DESIGN, SETTING, AND PARTICIPANTS: This retrospective, longitudinal case series from 20 academic dermatology practices in the US included a consecutive sample of 197 adults who met the European Rare and Severe Psoriasis Expert Network consensus definition for PPP between January 1, 2007, and December 31, 2018. Data analysis was performed June 2020 to December 2020. MAIN OUTCOMES AND MEASURES: The primary outcome was to describe the patient characteristics, associated medical comorbidities, treatment patterns, complications, and PPP-specific health care utilization. RESULTS: Of 197 patients, 145 (73.6%) were female, and the mean (SD) age at presentation was 53.0 (12.6) years, with a mean (SD) follow-up time of 22.1 (28.0) months. On initial presentation, 95 (48.2%) patients reported skin pain, and 39 (19.8%) reported difficulty using hands and/or feet. Seventy patients (35.5%) were treated with systemic treatments, and use of more than 20 different systemic therapies was reported. In patients with at least 6 months of follow-up (n = 128), a median (IQR) of 3.7 (4-10) dermatology visits per year were reported; 24 (18.8%) patients had 5 or more visits during the study period. CONCLUSIONS AND RELEVANCE: In this case series, PPP was associated with persistent symptoms, continued health care utilization, and a lack of consensus regarding effective treatments, emphasizing the unmet medical need in this population. Additional research is necessary to understand treatment response in these patients.

Corrigendum: Thiotepa hyperpigmentation preceding epidermal necrosis: malignant intertrigo misdiagnosed as Stevens-Johnson syndrome-toxic epidermal necrolysis overlap.

The original article was published on February 15, 2020 and corrected on March 15, 2020.The revised version of the article corrects the contact information of the Corresponding Author. The changes appear in the revised online PDF copy of this article.

Thiotepa hyperpigmentation preceding epidermal necrosis: malignant intertrigo misdiagnosed as Stevens-Johnson syndrome-toxic epidermal necrolysis overlap.

Thiotepa is a common alkylating agent known to precipitate cutaneous reactions consistent with toxic erythema of chemotherapy, including erythema and hyperpigmentation. Herein, we describe an atypical case of malignant intertrigo involving preferential erythema and desquamation not only of skin folds but also of occluded areas after thiotepa-based conditioning. The diagnosis was complicated by concurrent stomatitis and oral petechiae in the setting of autologous stem cell transplant 11 days prior for diffuse large B-cell lymphoma. Histopathological examination from two cutaneous sites demonstrated epidermal dysmaturation and eccrine gland necrosis consistent with thiotepa-induced desquamation and not Stevens-Johnson syndrome or graft-versus-host-disease. Malignant intertrigo can present with extensive cutaneous involvement, as evidenced by our patient who had 25% body surface area affected. Mucosal involvement is common with most chemotherapeutic regimens and its presence should not deter the astute clinician from consideration of a diagnosis of toxic erythema of chemotherapy. No further interventions were needed and the patient healed spontaneously.

Squamous cell carcinoma of the perineum masquerading as necrotizing hidradenitis suppurativa.

Many cases of superinfected hidradenitis suppurativa (HS) involve multiple species of bacteria, but gas-producing infections are rare and can complicate the clinical picture. Additionally, recognizing squamous cell carcinoma (SCC) as a complication of longstanding HS is imperative. Herein, we present a unique case of a severe emphysematous HS that was initially mistaken for Fournier gangrene and eventually diagnosed as superinfected SCC.

Antiphospholipid syndrome in a patient with rheumatoid arthritis.

Antiphospholipid syndrome (APS) is an autoimmune condition characterized by a thrombotic event and/or pregnancy morbidity in the presence of persistently elevated antiphospholipid (aPL) antibody titers, which are most prevalent in patients with systemic lupus erythematosus but also have been associated with other autoimmune, malignant, and infectious diseases. In contrast to the clear correlation between high aPL antibody titers and thrombotic events in patients with systemic lupus erythematosus, the pathogenic role of these autoantibodies in association with other diseases, such as rheumatoid arthritis (RA), is not as well defined. We report a case of APS manifesting as cutaneous ulceration and necrosis in a patient with severe RA.

Fractional photothermolysis treatment of digital cutis laxa reverses hand disability.

In this case study, we present a safe and novel treatment for a patient with soft tissue hand disability caused by severe and chronic lupus and cutis laxa (CL). This patient was a woman in her 50s with a 20-year history of systemic lupus erythematous (SLE) and multiple sclerosis who developed hand disability because of the drastic loss of firmness in her soft tissue, extending from the dermis down to the ligaments. The likely cause was CL with SLE synovitis, exacerbated by corticosteroid tapering. Fractional photothermolysis (FP) LASER (Fraxel DUAL 1550/1927; Solta Medical) therapy profoundly alleviated her joint locking in addition to improving the firmness of the overlying skin to reverse her hand disability. This case illustrates a novel approach to CL hand treatment and the profound impact the treatment had on the patient's disabled hand. FP therapy is quick and safe, and its medical application to skin and joints should be further explored.

Viral infections in acute graft-versus-host disease: a review of diagnostic and therapeutic approaches.

BACKGROUND: While immunosuppressive therapy for acute graft-versus-host disease (aGVHD) advances, viral reactivation has been found to be an increasingly common complication in these patients. Dermatologists may often be consulted on inpatient services for evaluation. OBJECTIVE: We investigated the literature for the role of viral infections in aGVHD and review the current evidence regarding management. METHODS: Articles in the public domain regarding aGVHD, cytomegalovirus, Epstein-Barr virus, varicella zoster virus, hepatitis viruses, parvovirus B19, and respiratory viruses were included. RESULTS: Dermatologic findings vary between different viral antigens, and some infections may be a marker for the development of aGVHD or worsen prognosis. LIMITATIONS: The heterogeneous cohorts of the studies reviewed often preclude direct comparison between results. CONCLUSION: The relationship between viral reactivation and aGVHD may be bidirectional and is worthy of further exploration. Additional studies are needed to determine appropriate prophylaxis and treatment.