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.

Occipital alopecia in a young man.

Lipedematous alopecia is a rare, non-androgenic form of alopecia that is challenging to diagnose, often requiring clinical-pathological correlation. The condition has been reported predominantly in African-American females, but more recently has been described in a broader demographic [1,2]. We describe a rare case of a young Caucasian man with isolated lipedematous alopecia who presented with a boggy, erythematous plaque with alopecia of the occipital scalp and subcutaneous thickening with lymphocytic dermal infiltrate and decreased anagen hairs on histology.

Intralesional corticosteroid-induced hypopigmentation and atrophy.

Intralesional corticosteroids are associated with various, uncommon, local adverse events [1]. Atrophy and hypopigmentation most commonlyremain localized to sites of injection. However, outward radiation in a linear, streaky pattern has been reported and is termed "perilesional/perilymphatic hypopigmentation or atrophy [2]." We report a case of this rare adverse event.

Prevalence of Surfactant in the Contact Allergen Management Program.

BACKGROUND: Surfactants are common ingredients in topical products, which can cause both irritant and allergic contact dermatitis. OBJECTIVE: The aim of this study was to determine the prevalence of 12 common groups of surfactants and 12 common individual surfactants among products in each category in the American Contact Dermatitis Society Contact Allergen Management Program (CAMP). METHODS: The American Contact Dermatitis Society CAMP was queried for the 12 surfactant groups and the 12 individual surfactants. RESULTS: The laureth/pareth sulfate group was the most prevalent surfactant group in CAMP products (17.9%). Laureth/pareth sulfates were the most common surfactant group in all product categories, except household and eye care products. The betaine/sultaine group (13.5%) and glucosides (10.0%) were also found in a significant proportion of CAMP products. Oleamidopropyl dimethylamine has the highest positive reaction rate (3.5%) but was tied for the lowest prevalence (0.20%) of the 12 individual surfactants studied. In contrast, cocamidopropyl betaine has a lower positive reaction rate (1.6%) with a higher prevalence (10.4%). CONCLUSIONS: Surfactants were commonly found across all product types in CAMP. This study provides important information on allergen and irritant exposures in care products.