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Sixty-Second issue

June 12, 2024


Can multiple onychopapillomas be a sign of BRCA1-associated protein tumor predisposition syndrome?
JAMA Dermatology​​
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Time to start keeping an eye on those nails for more than just your next manicure… it could be a diagnostic clue!

BRCA1-associated protein tumor predisposition syndrome (BAP1 TPDS) is an autosomal dominant genodermatosis associated with an increased risk of cutaneous and internal malignancies. Onychopapillomas, benign nail bed, and distal matrix tumors that present with nail plate abnormalities are hypothesized to be a novel sign in BAP1 TPDS. This single-center cohort study set out to evaluate carriers of BAP1 germline variants for present or historic nail abnormalities.

What did they find?
  • 41 of 47 patients with BAP1 TPDS (87.2%) had at least one nail abnormality.
  • Onychopapilloma was the most frequently observed nail abnormality (83%).
  • Onychopapilloma exhibited a predominance for males (observed in 88.2% of males and 80.0% of females) and adult patients (observed in 87.5% of individuals over 30 years of age).
  • 38 of 39 patients (97.4%) with onychopapilloma experienced polydactylous nail changes, most frequently of the thumbnail (92.3%).

Main Takeaway: BAP1 TPDS is associated with a high rate of onychopapillomas, suggesting that onychopapillomas may serve as a novel diagnostic sign of the condition.

Can non-accidental trauma be misdiagnosed as dermatitis?
Journal of Pediatric Dermatology
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A Cautionary Tale: The itch may not always be an accident. 

Pediatric dermatitis and nonaccidental trauma (NAT) can present similarly, especially in the diaper area, leading to potential misdiagnosis. This systematic literature review examined cases of dermatitis and NAT that were originally misdiagnosed in the pediatric population with the goal of better understanding the frequency and features of misdiagnosis.

What did they find? 
  • 21 case reports or series involving 29 pediatric patients were included (mean age 3.62 years [range 0.09–13]; 14/27 [51.9%] females). 
  • Of the 29 patients, 26 were initially suspected of NAT. 65.4% (17) suspected to be due to burn, 15.4% (4) physical abuse, 15.4% (4) sexual abuse, and 3.8% (1) for other forms of abuse.
  • Final diagnoses for these 26 patients included irritant contact dermatitis (ICD) in 53.8% (14) of cases, phytophotodermatitis (PPD) in 30.8% (8), allergic contact dermatitis (ACD) in 7.7% (2), perianal infectious dermatitis 3.8% (1), and atopic dermatitis (AD) in 3.8% (1). 
  • 3 patients initially diagnosed with dermatitis were later found to be victims of NAT.
    ​
Main Takeaway: The study highlights the importance of comprehensive assessments, including detailed history-taking, thorough physical examinations, and consideration of differential diagnoses to prevent misdiagnosis and potential harm to pediatric patients suspected of dermatitis or NAT.

Treatment for Central Centrifugal Cicatricial alopecia - Delphi consensus recommendations
Journal of the American Academy of Dermatology
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Even Fred and George can’t do it.
​
Central centrifugal cicatricial alopecia (CCCA) is a type of scarring alopecia predominant among women of African descent. Currently, a wide range of treatment modalities, including topical steroids and oral antibiotics, are available to manage CCCA; however, there has yet to be an established standard of care. 

This study used a modified Delphi method to develop consensus recommendations for treating CCCA. Twenty-seven expert dermatologists collaborated to make recommendations in 5 treatment categories: topical, systemic, procedural, supplements, and behavioral. A strong consensus threshold was met if 75% of respondents agreed or disagreed with a statement. 

What did they find?
  • Overall, experts formed 20 consensus statements for treating CCCA in adults.
  • The use of topical (n= 20/21 agreed) and intralesional (n= 18/21 agreed) corticosteroids for CCCA met strong consensus agreement, while systemic corticosteroids (n= 14/16 disagreed) met strong consensus disagreement. 
  • Oral antibiotics (n= 16/20 agreed), topical and/or low-dose oral minoxidil (n= 15/16 agreed), and hair transplantation (n= 14/16 agreed) were validated as potential treatment options with strong consensus agreement. 
  • Limiting or discontinuing traction hairstyles in patients with CCCA met strong consensus agreement (n= 18/21 agreed).

Limitations: This study only surveyed US-based dermatologists and did not include non-pharmacological treatment options.

Main Takeaway: Using the Delphi method, experts in hair and scalp dermatology collaborated to create guidelines for the management of CCCA. Recommendations include but are not limited to, topical and intralesional corticosteroids, oral antibiotics, and minoxidil.

Skin barrier and skin immunity appear altered in post-menopausal women on hormone replacement therapy upon irritant challenge
British Journal of Dermatology
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Sticks and stones may break my bones, but HRT will never HuRT me.

Hormone replacement therapy (HRT) is often used to manage post-menopausal symptoms. HRT has shown positive effects on the skin barrier, but little is known about its impact on skin immunity. This prospective study utilized 1.25% topical sodium lauryl sulfate to induce skin irritation in two cohorts of post-menopausal women, either HRT+ or HRT-. The clinical and histological presentations of the irritated areas were compared to non-irritated areas and between cohorts.

What did they find?
  • HRT+ cohort showed increased trans-epidermal water loss (P<0.05) in the irritated skin.
  • HRT+ cohort saw increased filaggrin expression (P<0.01) and cytokeratin-10 expression (P<0.05) to a greater extent in irritated skin compared to HRT-.
  • Increased Langerhans’ cells (P<0.01) and dendritic cells (P<0.05) were seen in the dermis of HRT+ irritated skin.
  • Higher density of macrophages (P<0.01) was present in the dermis of HRT+ irritated skin.

Main Takeaway: In post-menopausal women, HRT appears to enhance the skin barrier and increase the number of immune cells present within the dermis upon skin irritation. These findings suggest both a heightened risk of inflammation and a heightened ability for tissue repair in post-menopausual women receiving HRT.

What effective stigma-reduction strategies can be implemented for HIV-associated malignancies?
Journal of the National Cancer Institute
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All the ingredients you need to build the perfect ~potion~ to combat stigma!

Kaposi’s sarcoma (KS) is a prevalent HIV-associated malignancy in sub-Saharan Africa. The intersectional stigma of HIV, cancer, and skin disease in KS patients leads to delays in diagnosis and treatment. This qualitative study investigates the effectiveness of a multicomponent navigation strategy in reducing stigma and improving chemotherapy initiation and completion among patients in Kenya with HIV-associated KS.

What did they find?
  • A six-part navigation strategy was implemented for 27 patients with newly diagnosed KS.
  • Strategies included physical navigation, video-based education, travel stipends, health insurance enrollment assistance, health insurance stipends, and peer mentorship.
  • 24 participants reported anticipated stigma, half (14/27) experienced internalized stigma, and the majority (20/27) experienced external stigma from their communities.
  • Participants unanimously reported increased knowledge regarding KS (27/27) following the intervention.
  • 21/27 reported reduced feelings of isolation, leading to improved confidence in disclosing cancer status and reduced fear of bewitchment.

Main Takeaways: Peer support, education, and physical navigation appear to foster a sense of belonging, hope, knowledge, encouragement, and social support among patients with HIV-associated KS, suggesting an effective support strategy for KS and other stigmatized medical conditions.

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Can superficial X-ray therapy be an effective alternative for non-melanotic skin cancers?
Journal of the American Academy of Dermatology​
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An X-ray a day keeps the non-melanotic skin cancer away.

Mohs micrographic surgery is the gold standard for treating non-melanotic skin cancers (NMSC), yet not all patients are candidates for this procedure. Superficial X-ray therapy (SXRT) has been an alternative option for these patients in treating NMSC but remains underused despite its efficacy. Administration of SXRT is often painless and occurs over multiple sessions with radiation dosages of 300-800 cGY. Additionally, treatment costs for SXRT tend to be lower than those for Mohs surgery. This retrospective study examined treatment outcomes of 2490 NMSCs treated with SXRT.

What did they find?
  • The rate of recurrence was 2.2% for all types of NMSC with a 95% confidence interval of (1.5%-2.9%) after 2 years, 6.0% (4.7%-7.4%) after 5 years, and 10.5% (7.8%-13.3%) after 10 years.
  • For basal cell carcinoma, the rate of recurrence was 2.8% (1.2%-4.4%) after 2 years, 6.9% (3.8%- 10.0%) after 5 years, and 12.4% (6.4%-18.5%) after 10 years.
  • For squamous cell carcinoma, the rate of recurrence was 2.0% (1.2%-2.7%) after 2 years, 5.8% (4.2%-7.3%) after 5 years, and 9.9% (6.8%-12.9%) after 10 years.
  • The average cost of treatment with SXRT was $734.67 per lesion, significantly lower than Mohs Micrographic surgery, which averages $1396.16 per lesion.

Main Takeaway: Superficial X-ray therapy is an economically favorable and effective alternative to Mohs micrographic surgery and should receive heightened consideration in those not able to undergo Mohs surgery. 

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Treated Primary Cutaneous Malignant Melanoma With Later Metastasis Found in Clinical Presentation of Left Axilla Lymphadenopathy
Malignant melanoma is the deadliest form of skin cancer. This case report higlights a 50-year-old woman who had a previous history of primary cutaneous malignant melanoma located on her left breast that was treated with wide local excision and closure, but recently presented with left axillary lymphadenopathy. 

Report of Case: 
  • The patient had not completed a screening mammogram in 4 years. 
  • H&E histology results revealed metastatic melanoma.
  • Flow cytometry revealed the tumor cells were positive for S100 and Sox10 and negative for GATA3, E-cadherin, estrogen and progesterone receptor, CD68, CD45, and broad-spectrum cytokeratin, strongly supporting the diagnosis of metastatic melanoma of the left axilla. 

Main takeaway: This case highlights the importance of continuous patient-specific oncology surveillance after the surgical treatment of cutaneous malignant melanoma due to potential metastatic spread. ​

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