Harlequin Ichthyosis in a Filipino newborn: Management pearls in a resource-limited setting

Introduction@#Harlequin ichthyosis (HI) is a rare type of autosomal recessive congenital ichthyosis. There are approximately 200 documented cases worldwide, with less than five published reports in the Philippines. Despite its rarity, current literature suggests a better prognosis for these patients. @*Case description@#We describe a preterm male newborn who presented at birth enclosed in a thick hyperkeratotic armor-like scale plates with areas of fissures, with associated ectropion, conjunctiva dehiscence, and eclabium. The thickened encasement also covered the hands and feet, causing severe contractures. A diagnosis of harlequin ichthyosis was given based on the clinical features. The patient was managed through a multidisciplinary approach, including referral to the tele-ichthyosis platform of a US-based foundation for patients with ichthyosis. Thermoregulation, nutrition, and hydration were carefully managed. Bland emollients were applied generously following normal saline soaks to improve barrier protection. Acitretin was administered on day 2 of life to facilitate the desquamation of the thickened encasement. A marked decrease in erythema and the thickness of the hyperkeratotic skin, and reduced conjunctival dehiscence were noted after one week of therapy. However, the constrictions on the hands and feet showed bluish discoloration and signs of necrosis. Linear band excision was performed to release the constrictors. Despite aggressive management, the patient succumbed to sepsis on day 12 of life. @*Conclusion@#Improved prognosis amongst HI patients is correlated with optimal quality of care regardless of resource limitations. A multidisciplinary approach and early administration of retinoids cannot be overemphasized. Linear band excision within the first week of life is suggested for constrictions on the extremities that do not improve with retinoids to avoid necrosis and autoamputation.

Acupuncture as an adjunct to standard therapy for Pruritus in patients with Atopic Dermatitis: A patient- and assessor-blinded, randomized, placebo-controlled trial

Background@#Pruritus can impair quality of life in patients with atopic dermatitis. There is evidence that acupuncture reduces pruritus and disease severity, and improves quality of life. @*Objectives@#This study aimed to determine the efficacy of acupuncture in reducing pruritus intensity, disease severity, and medication use, and improving quality of life. @*Methods@#This was a patient- and assessor-blinded, randomized, placebo-controlled trial. Patients diagnosed with atopic dermatitis underwent twice-weekly acupuncture for 12 weeks, with an 8-week follow-up period. Baseline and weekly assessment were done using standard data collection forms and validated assessment tools. @*Results@#Thirty patients were randomized and 28 patients were eligible for the efficacy analysis. There were no significant differences in the baseline demographic and clinical characteristics between the True Acupuncture group (TA) (n=16) and Sham Acupuncture group (SA) (n=12). Both groups showed reduction in mean itch intensity (visual analogue scale, VAS) (p=0.024) but TA showed greater reduction (p=0.009) that was sustained after end of treatment. There was also a reduction in medication use in both groups. The comparable efficacy of SA to TA is attributed to similar peripheral receptive fields and stimulation of cutaneous C-fibers which depletes the neurotransmitters mediating pruritus and results in tachyphylaxis. Mild adverse events, such as petechiae and erythema, were noted in both groups and resolved spontaneously. @*Conclusion@#Acupuncture is a promising adjunct treatment in atopic dermatitis with significant reduction in pruritus, disease severity and medication use and a trend towards improved quality of life. Studies with larger sample size and comparison to acupuncture points farther from the true acupuncture points are recommended. @*Trial Registration@#Food and Drug Administration Philippine Health Research Registry ID PHRR171012-001696

Immunohistochemical expression of CK19, AR, PHLDA1, CD10 and Ki67 in the differentiation between Trichoepithelioma and Basal Cell Carcinoma: A systematic review

Background@#Basal cell carcinoma (BCC) and trichoepithelioma (TE) are follicular adnexal neoplasms that arise from the follicular germ but with divergent biological behavior. The gold standard in the differentiation is through histopathological examination using hematoxylin and eosin (H and E) stain. There are cases, however, when the distinction is not straightforward. @*Objective@#To assess the association and diagnostic accuracy of the immunohistochemical (IHC) expressions of CD10, Ki67, CK19, androgen receptor (AR), and PHLDA1 in distinguishing between basal cell carcinoma and trichoepithelioma. @*Methods@#We conducted a comprehensive search on cross-sectional studies on human tissue from 2000 to 2020 in MEDLINE (PubMed), CENTRAL and EMBASE for comparative studies and reference lists. The data were summarized and analyzed using Microsoft Excel and RevMan. We used Chi-square test for independence, summary receiver operator curves (sROC), and diagnostic odds ratio (OR). @*Results@#We included 15 articles containing 686 BCC and 367 TE in the systematic review. The pooled staining of biomarkers showed a significant difference in the staining of CK19 (p<0.05) and AR (p<0.0001), and PHLDA1 (p<0.0001). Diagnostic odds ratio was used to confirm these associations. AR was found to have the highest odds in the diagnosis of BCC (OR 27.92, 95% CI 10.69, 72.86). The pattern of staining of CD10 is significant (p<0.001) with staining of both tumor and stroma (OR 8.09, 95% CI 4.57, 13.53) and staining of tumor alone (OR 8.15, 95% CI 4.56, 14.35) (p<0.001) in the diagnosis of BCC. CD10 stromal staining, on the other hand, is significantly associated with the diagnosis of TE (OR 7.26, 95% CI 5.06, 10.44) (p<0.0001). There is no significant association between Ki67 staining (OR 1.22, 95% CI 0.48, 3.09) (p=0.67) and the diagnosis of BCC. The forest plot and sROC showed that AR had high specificity across all included studies in the diagnosis of basal cell carcinoma, while PHLDA1 demonstrated high specificity and high sensitivity in diagnosing trichoepithelioma. @*Conclusion@#The biomarkers AR and PHLDA1 are useful as an initial panel to distinguish between BCC and TE, given that both showed high sensitivity as well as significant association with BCC and TE respectively. CD10 and CK19 may also be used with AR and PHLDA1 for further confirmation.

Low-dose systemic retinoids in preventing subsequent non-melanoma skin cancers (NMSC) in patients with history of NMSC: A systematic review

@#<p style="text-align: justify;"><strong>BACKGROUND:</strong> Non-melanoma skin cancers (NMSC) consists of  basal-cell carcinomas (BCC) and squamous-cell carcinomas (SCC).Certain populations are predisposed to develop  NMSC,  including  patients  with  previous  history  of  NMSC.Systemic  retinoids  have  shown  promising  results  in chemoprevention of recurrence of NMSC in other high-risk populations (xeroderma pigmentosum and renal-transplant patients).We  assessed  the  efficacy  and  safety  of  low-dose  systemic  retinoids  compared  with  placebo,  as  a  chemopreventive agent for NMSC in patients with previous NMSC.<br /><strong>METHODOLOGY:</strong> Electronic  databases  were  systematically searched for this study. Participants in the studies selected must have had a biopsy-proven NMSC, over 18 years of age, with  no  exclusion  of  other  demographic  characteristics. All  types  of  systemic  retinoids  were  included  with  no restriction on dosage. Two authors independently performed standardized  eligibility  assessment  and  data-extraction.Differences in opinion were resolved by consensus with the third author. Statistical analysis was done using the Review Manager 5 software.<br /><strong>RESULTS:</strong> Eleven full-text studies were assessed for eligibility out of 178 studies found. Five studies were excluded because of the different population, while the two articles used topical retinoids. Four articles were included. The interventions were 10.0 mg isotretinoin, 25,000IU retinol and 25.0 mg acitretin,compared with placebo. Meta-analysis produced RR of 0.94 (95% CI, 0.89-1.00), with moderate heterogeneity (34%) due to the difference in interventions used. There are significantly more  adverse  events  in  the  retinoids  group,  especially  in the  incidence  of  mucocutaneous  adverse  events,  and deranged lipid profile and liver enzymes.<br /><strong>CONCLUSION:</strong> There is insufficient evidence to support the use of low-dose systemic retinoids as chemoprevention for patients with previous NMSC. Furthermore, adverse events may limit their use. Topical preparations with less side-effects may be investigated.</p>

Low-dose systemic retinoids in preventing subsequent non-melanoma skin cancers (NMSC) in patients with history of NMSC: A systematic review

BACKGROUND: Non-melanoma skin cancers (NMSC) consists of  basal-cell carcinomas (BCC) and squamous-cell carcinomas (SCC).Certain populations are predisposed to develop  NMSC,  including  patients  with  previous  history  of  NMSC.Systemic  retinoids  have  shown  promising  results  in chemoprevention of recurrence of NMSC in other high-risk populations (xeroderma pigmentosum and renal-transplant patients).We  assessed  the  efficacy  and  safety  of  low-dose  systemic  retinoids  compared  with  placebo,  as  a  chemopreventive agent for NMSC in patients with previous NMSC.METHODOLOGY: Electronic  databases  were  systematically searched for this study. Participants in the studies selected must have had a biopsy-proven NMSC, over 18 years of age, with  no  exclusion  of  other  demographic  characteristics. All  types  of  systemic  retinoids  were  included  with  no restriction on dosage. Two authors independently performed standardized  eligibility  assessment  and  data-extraction.Differences in opinion were resolved by consensus with the third author. Statistical analysis was done using the Review Manager 5 software.RESULTS: Eleven full-text studies were assessed for eligibility out of 178 studies found. Five studies were excluded because of the different population, while the two articles used topical retinoids. Four articles were included. The interventions were 10.0 mg isotretinoin, 25,000IU retinol and 25.0 mg acitretin,compared with placebo. Meta-analysis produced RR of 0.94 (95% CI, 0.89-1.00), with moderate heterogeneity (34%) due to the difference in interventions used. There are significantly more  adverse  events  in  the  retinoids  group,  especially  in the  incidence  of  mucocutaneous  adverse  events,  and deranged lipid profile and liver enzymes.CONCLUSION: There is insufficient evidence to support the use of low-dose systemic retinoids as chemoprevention for patients with previous NMSC. Furthermore, adverse events may limit their use. Topical preparations with less side-effects may be investigated.