Epidemiology, clinical characteristics, treatment, and outcomes of proliferating pilar tumors: A systematic review.

BACKGROUND: Proliferating pilar tumors (PPTs) are rare cutaneous neoplasms arising from hair follicles that have both malignant and metastatic potential. OBJECTIVE: To present a systematic review of the epidemiology, clinical characteristics, and treatment and outcome data on PPTs. METHODS: Using the OVID platform, MEDLINE and Embase were searched from inception until May 26, 2022. All studies that provided original data on PPTs in English were included. References of these studies were also cross-checked to identify any additional relevant articles. Oxford's Levels of Evidence-Based Medicine was used for quality assessment. RESULTS: A total of 114 articles, providing data on 361 cases of PPTs, were included in our synthesis. Every study included was either a case series or case report. The mean age at diagnosis was 61.7. Most patients in the synthesis were female (71%), and the majority of cases occurred on the scalp (73.1%). The presence or absence of cytological atypia was only reported in one-third of the cases; 36.8% of cases were classified as malignant and 7.5% metastasized. Although no lesions treated with Mohs micrographic surgery required adjuvant radiation and only one reported recurrence occurred after Mohs surgery, there is insufficient data to make conclusions on a superior treatment modality. LIMITATIONS: All studies in this review were either case reports or case series. CONCLUSIONS AND RELEVANCE: Our study supports the notion that PPTs occur most commonly on the scalp of elderly female patients. Moreover, our findings confirm that PPT is capable of demonstrating aggressive biology and metastasis. Given the lack of uniformity in histologic description, pathologists should be encouraged to comment on the presence and degree of cytological atypia when reporting cases of rare neoplasms such as the PPT. Greater consensus on diagnosis and classification as well as more robust data is needed regarding optimal management.

A Systematic Review of the Progression of Cutaneous Lupus to Systemic Lupus Erythematosus.

Lupus erythematosus is an autoimmune disease that may manifest in a variety of organs and tissues including the skin, kidney, brain, heart and lung. Many patients present with cutaneous lupus, where disease is often limited to the skin, but are at risk for developing systemic lupus. The objective of our present study is to perform a systematic review of studies that investigated patient cohorts and populations for the occurrence of cutaneous lupus progressing to systemic lupus. Inclusion criteria required that studies present longitudinal data of patients with limited cutaneous lupus erythematosus who were followed for development of systemic lupus erythematosus. Studies were excluded if patients had concurrent diagnosis of SLE, or if they failed to present longitudinal data. Medline and Embase were searched for English language studies using the Ovid platform. A total of 25 adult studies were identified, as well as 8 pediatric studies. The rate of cutaneous to systemic lupus progression ranged between 0% to 42% in the adult studies and 0% to 31% in the pediatric groups. The variability in these rates were due to differences in patient populations, study design, criteria used to diagnose systemic lupus, and follow-up time. Common risk factors associated with systemic lupus erythematosus development including having positive anti-nuclear antibodies, hematologic abnormalities, and higher number of lupus classification criteria at baseline. This study emphasizes the importance for providers to routinely monitor for systemic lupus in patients with cutaneous lupus.

Co-existence of PrPD types 1 and 2 in sporadic Creutzfeldt-Jakob disease of the VV subgroup: phenotypic and prion protein characteristics.

We report a detailed study of a cohort of sporadic Creutzfeldt-Jakob disease (sCJD) VV1-2 type-mixed cases (valine homozygosity at codon 129 of the prion protein, PrP, gene harboring disease-related PrP, PrPD, types 1 and 2). Overall, sCJDVV1-2 subjects showed mixed clinical and histopathological features, which often correlated with the relative amounts of the corresponding PrPD type. However, type-specific phenotypic characteristics were only detected when the amount of the corresponding PrPD type exceeded 20-25%. Overall, original features of types 1 (T1) and 2 (T2) in sCJDVV1 and -VV2, including rostrocaudal relative distribution and conformational indicators, were maintained in sCJDVV1-2 except for one of the two components of T1 identified by electrophoretic mobility as T121. The T121 conformational characteristics shifted in the presence of T2, inferring a conformational effect of PrPD T2 on T121. The prevalence of sCJDVV1-2 was 23% or 57% of all sCJDVV cases, depending on whether standard or highly sensitive type-detecting procedures were adopted. This study, together with previous data from sCJDMM1-2 (methionine homozygosity at PrP gene codon 129) establishes the type-mixed sCJD variants as an important component of sCJD, which cannot be identified with current non-tissue based diagnostic tests of prion disease.

Photolichenoid dermatitis: a presenting sign of human immunodeficiency virus.

Photolichenoid dermatitis is an uncommon eruptive dermatitis that often occurs in association with a photosensitizing drug. Photodermatitis, in general, is an uncommon clinical manifestation of human immunodeficiency virus (HIV), most often affecting patients of African and Native American descent. Photolichenoid dermatitis has infrequently been reported in patients with HIV who have not been exposed to a photosensitizing drug. We report a case of an African patient with a photodistributed depigmenting eruption without exposure to a photosensitizing drug. Histologic examination revealed a patchy perivascular and bandlike lymphocytic infiltrate with melanophages, interface changes, and dyskeratotic keratinocytes, consistent with photolichenoid dermatitis. Laboratory examination was significant for a positive HIV-2 antibody. Photolichenoid dermatitis may be a presenting sign of HIV infection and may not necessarily be associated with exposure to a photosensitizing drug. Testing for HIV should be done in patients who present with photodistributed depigmenting eruptions, even in the absence of exposure to a photosensitizing drug, and particularly in patients of African and Native American descent.

The readability, suitability, and content features of eczema action plans in the United States.

BACKGROUND/OBJECTIVES: Little is known about the reading grade level (readability), appropriateness of design (suitability), and content variability of written eczema action plans (EAPs), which can impact the effectiveness of these patient education tools. Here, we assess the readability, suitability, and content of EAPs currently used by pediatric dermatologists in the United States. METHODS: This was a cross-sectional study of EAPs submitted by members of the Society for Pediatric Dermatology (n = 26). Readability, suitability, and content of sampled plans were systematically assessed. RESULTS: Mean (SD) reading grade level was 9.0 (2.1); one in five was written at the recommended level of 6th grade or lower. While the majority of EAPs were found to be adequately suitable, one in five was unsuitable and only two superior. Documents scored most poorly in layout/design and learning stimulation. Plans scored best in the categories of content and literacy demand. EAPs focused on similar content themes, though specific recommendations and descriptors of atopic dermatitis (AD) disease states varied considerably. CONCLUSIONS: The health literacy burden of EAPs in the United States could be lowered by improving their readability, incorporating graphics, stimulating reader engagement, and developing standards for how AD flares are defined.

Mycoplasma pneumoniae, more than a lung disease.

Mycoplasma pneumoniae-induced rash and mucositis (MIRM) is a recently described clinical entity and should be considered in children who present with oral (94% of patients), ocular (82% of patients), and urogenital lesions (63% of patients). MIRM was first described as a distinct clinical entity from Stevens Johnson syndrome/Toxic epidermal necrolysis (SJS)/(TEN) in 2015 [1]. As a new, uncommon diagnosis it frequently poses a diagnostic and therapeutic challenge for pediatricians and dermatologists. We report a case of MIRM in a previously healthy 15-year-old boy.

A systematic review and meta-analysis of the effects of topical nitrates in the treatment of primary and secondary Raynaud's phenomenon.

BACKGROUND: Multiple placebo-controlled trials have assessed locally applied topical nitrate preparations in treating Raynaud's phenomenon (RP). OBJECTIVES: The objective of this meta-analysis was to assess the effects of local topical nitrates in primary and secondary RP with respect to a combined end point integrating parameters of digital blood flow and clinical severity. METHODS: A systematic review was performed using MEDLINE, Embase, and the Cochrane library. Only trials comparing locally applied topical nitrates with placebo comparators were included. Studies were appraised for bias by 2 independent reviewers. RESULTS: A total of 7 placebo-controlled trials including 346 patients were used in the meta-analysis; 4 trials used nitroglycerin ointments, 2 used the nitroglycerin gel vehicle MQX-503, and 1 used compounded nitrite. The meta-analysis results supported a moderate-to-large treatment effect in RP (standardized mean difference [SMD] = 0.70; 95% CI, 0.35-1.05; P < .0001). Subgroup analyses showed a large treatment effect in secondary RP (SMD = 0.95; 95% CI, 0.25-1.65; P = .008) and moderate effect in primary RP (SMD = 0.45; 95% CI, 0.05-0.85; P = .03). LIMITATIONS: Limitations include the inclusion of multiple topical nitrate preparations and integration of different outcomes assessments. CONCLUSION: Local topical nitrates have significant efficacy in the treatment of both primary and secondary RP.

An Update on Kaposi's Sarcoma: Epidemiology, Pathogenesis and Treatment.

Kaposi's sarcoma is an angioproliferative neoplasm which has undergone considerable epidemiologic change since the original description by Moritz Kaposi in the late 1800s. This opportunistic neoplasm gained widespread notoriety within the US during the height of the AIDS epidemic, where it was frequently found co-occurring with opportunistic infections. With the advent of modern antiretroviral therapies, as well as an increasing number of individuals on immunosuppression for autoimmune disease or organ transplantation, the landscape of the immunocompromised individual has changed. It is now important for clinicians to be mindful of Kaposi's sarcoma manifesting in a growing variety of clinical contexts.

Synthetic Aß peptides acquire prion-like properties in the brain.

In transmission studies with Alzheimer's disease (AD) animal models, the formation of Aß plaques is proposed to be initiated by seeding the inoculated amyloid ß (Aß) peptides in the brain. Like the misfolded scrapie prion protein (PrPSc) in prion diseases, Aß in AD shows a certain degree of resistance to protease digestion while the biochemical basis for protease resistance of Aß remains poorly understood. Using in vitro assays, histoblotting, and electron microscopy, we characterize the biochemical and morphological features of synthetic Aß peptides and Aß isolated from AD brain tissues. Consistent with previous observations, monomeric and oligomeric Aß species extracted from AD brains are insoluble in detergent buffers and resistant to digestions with proteinase K (PK). Histoblotting of AD brain tissue sections exhibits an increased Aß immunoreactivity after digestion with PK. In contrast, synthetic Aß40 and Aß42 are soluble in detergent buffers and fully digested by PK. Electron microscopy of Aß40 and Aß42 synthetic peptides shows that both species of Aß form mature fibrils. Those generated from Aß40 are longer but less numerous than those made of Aß42. When spiked into human brain homogenates, both Aß40 and Aß42 acquire insolubility in detergent and resistance to PK. Our study favors the hypothesis that the human brain may contain cofactor(s) that confers the synthetic Aß peptides PrPSc-like physicochemical properties.