Headache in Sturge-Weber syndrome: A systematic review.

BACKGROUND: Sturge-Weber syndrome (SWS) is a neurocutaneous disorder for which the neurological aspects, particularly headaches, remain poorly understood, despite significantly affecting morbidity. The present study aimed to elucidate the prevalence, characteristics and treatment strategies, as well as explore the pathogenesis of headaches, in SWS. METHODS: Using Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines, we systematically reviewed observational studies, case reports and series from eight databases (Cochrane Library, EBSCO, Embase, Medline, PubMed, Science Direct, Scopus and Web of Science), published from 1978 to 2023, to investigate the prevalence, characteristics, medication response and pathogenic theories of headaches in SWS. RESULTS: The review analyzed 48 studies, uncovering headache prevalence between 37% and 71%. Migraine-like headache affected up to 52% of individuals. Prophylactic and acute treatments included non-steroidal anti-inflammatory drugs, triptans and antiepileptic drugs, despite the lack of established guidelines. Life-threatening headaches in SWS are uncommon, typically accompanied by other neurological symptoms. The pathogenesis of headaches in SWS is considered to involve venous congestion and neuronal hyperexcitability linked to leptomeningeal angiomas. CONCLUSIONS: Headaches occur more frequently in individuals with SWS than in the general population. Despite symptoms meeting migraine criteria, these headaches should be considered secondary to vascular conditions. Implementing acute and prophylactic treatment is advised to reduce the impact on patients' lives.

Melanoma in children: A systematic review and individual patient meta-analysis.

The current evidence on paediatric melanoma is heterogeneous, especially regarding the prognosis of different histological subtypes. We sought to systematically review the evidence on paediatric melanoma, highlighting the major sources of heterogeneity and focusing on available data on single patients. A systematic search was performed from 1948 to 25 January 2021. Only studies reporting at least one case of cutaneous melanoma in patients aged ≤18 years were included. Unknown primary and uncertain malignant melanomas were excluded. Three couples of authors independently performed title/abstract screening and two different authors reviewed all the relevant full texts. The selected articles were manually cross-checked for overlapping data for qualitative synthesis. Subsequently data on single patients were extracted to perform a patient-level meta-analysis. PROSPERO registration number: CRD42021233248. The main outcomes were melanoma-specific survival (MSS) and progression-free survival (PFS) outcomes. Separate analyses were done of cases with complete information on histologic subtype, focusing on superficial spreading (SSM), nodular (NM) and spitzoid melanomas, as well as of those classified as de-novo (DNM) and acquired or congenital nevus-associated melanomas (NAM). The qualitative synthesis covered 266 studies; however, data on single patients were available from 213 studies including 1002 patients. Among histologic subtypes, NM had a lower MSS than both SSM and spitzoid melanoma, and a lower PFS than SSM. Spitzoid melanoma had a significantly higher progression risk than SSM and trended toward lower mortality. Focusing on nevus-associated status, DNM demonstrated better MSS after progression than congenital NAM, and no differences were highlighted in PFS. Our findings describe the existence of different biological patterns in paediatric melanoma. Specifically, spitzoid melanomas demonstrated intermediate behaviour between SSM and NM and showed a high risk of nodal progression but low mortality. This raises the question of whether spitzoid lesions are being over-diagnosed as melanoma in childhood.

Alopecia areata treated with topical and systemic brevilin A: A case series.

Alopecia areata (AA) is an autoimmune T CD8 cell mediated condition clinically characterized by hair loss from single or few small patches to complete hair loss. The management of AA is challenging and all available therapies does not ensure a long-term remission. To assess the safety and efficacy of both systemic and topical brevilin A, a natural compound, in AA patients not responding to other treatments. After obtaining informed consent, we administered off-label brevilin A to 13 adult patients affected by AA, for a period ranging from 6 to 18 months. Medical records for each patient and the severity of alopecia tool (SALT) score before and after brevilin A administration were recorded. The mean SALT score of our patients was 81.03 (SD 34.9) at baseline and 75.8 (SD 37.4) after brevilin A therapy, meaning no statistically significant improvement was observed (P = .2385 Paired t test). However, three multifocal AA (MAA) patients out of four attained an improvement (75%) suggesting that brevilin A may be represent an alternative therapy in this form of AA. Authors conclude that brevilin A could represent in the future a possible effective treatment in MAA forms but further studies are required.

A case of Jarisch-Herxheimer reaction in candidiasis treated with systemic fluconazole.

Jarisch-Herxheimer reaction (JHR) is an acute and self-limited condition, which commonly occurs after treatment for spirochetal infections. Probably, it corresponds to a transient immunological reaction to endotoxin-like products released from the microorganism during the therapy. For this reason, JHR may be associated with many other infectious diseases besides syphilis. Here, we report a case of a patient affected by extended cutaneous candidiasis diagnosed by culture examination; a JHR occurred after an accidental overdose of oral fluconazole.

Alopecia areata treated with dimethyl fumarate: A case series.

Alopecia areata is an autoimmune condition leading to non-scarring hair loss. Clinically, several presentations ranging from single or few small patches to complete hair loss are documented. The management of alopecia areata is challenging and all available treatments do not ensure a long-term remission to assess the safety and efficacy of systemic dimethyl fumarate in alopecia areata patients not responding to other systemic treatments. After obtaining informed consent, we administered off-label dimethyl fumarate to 10 adult patients with alopecia areata, for a period ranging from 4 to 37 weeks. Medical information for each patient and the severity of alopecia tool (SALT) score before and after dimethyl fumarate administration were recorded. During the treatment, 50% of patients (5 patients out of 10) had a slight improvement of hair regrowth; it was mainly as partial hair regrowth (ranging from 8% to 32%) and only one patient (10%) achieved > 50% terminal hair regrowth. Authors conclude that dimethyl fumarate is not advisable as a treatment of alopecia areata, also considering the risk of fumaric acid esters toxicity.

A "seasonal bromoderma" in a farmer.

Bromoderma is a rare skin disease caused by the ingestion, inhalation, or contact with products containing bromides. We report a case of bromoderma in a 36-year-old farmer, characterized by remission during the spring and exacerbation during the winter. The recognition of the use of products containing bromides during the winter helped us to understand the cause of the clinical condition.

The diagnosis of androgenetic alopecia in children: Considerations of pathophysiological plausibility.

Androgenetic alopecia (AGA), one of the most common causes of hair loss in men and women, is an infrequent cause of alopecia in children. In AGA, patients generally start noticing hair thinning after the onset of puberty due to progressive miniaturisation of the hair follicle which leads to vellus transformation of terminal hair. However, the occurrence of prepubertal AGA has rarely been reported in the literature. The pathophysiology of AGA is tightly linked to androgen hormones; prepubertal children do not usually produce significant amounts of adrenal or gonadal androgens. When it does occur, an underlying abnormality should be suspected. Secondary causes of AGA must be excluded when evaluating a patient before the appearance of puberty. Premature puberty, polycystic ovarian syndrome and other causes of hyperandrogenism can present with hair loss in an androgenetic pattern. This article reviews the normal physiology of androgen hormones and their role in the pathophysiology of childhood AGA.

The management of pseudomyogenic hemangioendothelioma of the foot: A case report and review of the literature.

Pseudomyogenic hemangioendothelioma (PMH) is a rare, mostly indolent, endothelial neoplasm of low-grade malignancy, often mimicking myoid and epithelioid tumors histologically. It is more frequent in young adult males and it usually presents with multiple cutaneous nodules, mostly localized at the extremities. It traverses several tissue planes simultaneously and can involve dermis, subcutis, skeletal muscle, and bone. Histologically, it is characterized by plump spindle cells with eosinophilic cytoplasm, often arranged in fascicles and epithelioid cells with "pseudomyogenic" morphology. Immunohistochemically, PMH is positive for Factor VIII, FLI-1, INI-1, vimentin, MDM2, CDK4, CD31, AE1/AE3, EMA, and P63. The efficacy of treatments is only partially known. Because of the frequent multifocal aspect of PMH, which contraindicates surgery, systemic treatments, such as gemcitabine, sirolimus, and everolimus are used. Based on our observation of multifocal PMH of the foot in a 17-year-old male patient, treated with gemcitabine with complete cutaneous response in a 2-year follow-up, we decided to discuss this rare tumor and underline its progression and therapeutic approaches. Thanks to a correct diagnosis, it is possible to avoid aggressive therapeutic approaches, which would be necessary for nonindolent diseases, such as sarcoma, which often needs amputation.

Pitted keratolysis, erythromycin, and hyperhidrosis.

Pitted keratolysis (PK) is a plantar skin disorder mainly caused by coryneform bacteria. A common treatment consists of the topical use of erythromycin. Hyperhidrosis is considered a predisposing factor for bacterial proliferation and, consequently, for the onset of PK. The aim of this study was to evaluate the relationship between PK erythromycin and hyperhidrosis. All patients with PK seen in Sant'Andrea Hospital, between January 2009 and December 2011, were collected. PK was clinically and microscopically diagnosed. All patients underwent only topical treatment with erythromycin 3% gel twice daily. At the beginning of the study and after 5 and 10 days of treatment, a clinical evaluation and a gravimetric measurement of plantar sweating were assessed. A total of 97 patients were diagnosed as PK and were included in the study. Gravimetric measurements showed that in 94 of 97 examined patients (96.90%) at the time of the diagnosis, there was a bilateral excessive sweating occurring specifically in the areas affected by PK. After 10 days of antibiotic therapy, hyperhidrosis regressed together with the clinical manifestations. According to these data, we hypothesize that hyperhidrosis is due to an eccrine sweat gland hyperfunction, probably secondary to bacterial infection.

Efficacy of a new combination of a microemulsion and oral supplementation for androgenetic alopecia: an interventional multicentric study.

BACKGROUND: The aim of this study was to study new therapeutic options for the treatment of female and male androgenetic alopecia (AGA) and to assess the efficacy of a possible new coadjuvant oral and topical therapy, containing a complex of natural substances. METHODS: Sixty individuals affected by mild-to-moderate female and male AGA were enrolled in the study and underwent the oral and topical treatment with a microemulsion formulation for 6 months. At baseline and at 3- and 6-month follow-up, global photographs were taken and three expert operators evaluated photographs using the 7-point scale. On a subgroup of our sample, non-invasive phototrichograms with TrichoScan® HD at baseline and follow-ups were performed to collect the trichological parameters of total number of hairs, hair density/cm2, vellus hair density/cm2, terminal hair density/cm2 and hair thickness. One-way ANOVA and Unpaired Student t-test were performed to analyze the data. RESULTS: Using the 7-point scale, a clinically visible improvement of hair loss was observed after three and six months of treatment. A statistically significant increase of all TrichoScan® trichological parameters was observed at both 3- and 6 month-follow-up. CONCLUSIONS: The complex of natural and active substances tested in this work showed good efficacy in improving both male and female hair loss. These new products could represent a valid alternative or coadjuvant therapy of AGA, increasing the efficacy of conventional treatments such as minoxidil or finasteride.

Clinical and Trichoscopic Graded Live Visual Scale for Androgenetic Alopecia.

Introduction: Currently, the mostly used classifications of androgenetic alopecia (AGA) only provide a macroscopic and subjective description of this disorder, without evaluating trichoscopic features. Objectives: The aim of this study is to elaborate a graded live visual AGA severity scale including macroscopic and microscopic (trichoscopic) pictures, and to determine the most frequent trichoscopic characteristics associated to each grade. Methods: A retrospective observational study was conducted on 122 patients (50 females and 72 males) affected by AGA. Macroscopic and trichoscopic photographs were taken at standardized scalp points. Results: Each picture was ranked from AGA stage I to VII, according to Hamilton scale for men and Sinclair scale for women, and the most representative images of each severity degree were collected to produce a graded live visual scale. In males, 2 live visual scales, 1 for the anterior and 1 for posterior region of the scalp were created. In females, only 1 scale of the anterior region was realized. For each stage of severity, the corresponding trichoscopic parameters were statistically analyzed. Conclusions: We realized new macroscopic and trichoscopic graded live visual scales for male and female patients affected by AGA, which could help physicians in giving an objective evaluation of the disease and in better managing it.

Recommendations on trichological treatments during COVID-19 pandemic.

COVID-19 is an infectious disease caused by the novel coronavirus SARS-CoV-2, which rapidly spreads via respiratory droplets and is the cause of the current pandemic. In this alarming situation, it is a delicate matter how to visit patients safely and how to manage their chronic treatments. The aim of this paper is to examine in detail the potential impact on SARS-CoV-2 infection of treatments routinely used in trichology and to provide a useful guide for the therapeutic management of trichological patients in this new COVID-19 era.

New-onset cutaneous kaposi's sarcoma following SARS-CoV-2 infection.

BACKGROUND: COVID-19 is associated with several cutaneous manifestations, including chilbain-like lesions, urticaria, erythema multiforme, and maculopapular lesions. Dermatoses may be directly linked to the viral infection or also represent a consequence of systemic therapies administrated for COVID-19. A potential role of SARS-CoV-2 in triggering the reactivation of other viruses, such as HHV-6, HHV-7 and Epstein-Barr virus has been hypothesized. OBJECTIVE: To better understand and hypothesize possible pathogenetic correlations of COVID-19 with other dermatological conditions. METHODS: We report the case of an 83-year-old woman hospitalized in a nursing home for several years. On November 2020, the patient had been diagnosed with SARS-CoV-2 infection, with repeated positive swabs until January 2021. After a month, new-onset asymptomatic cutaneous purplish macular lesions and violaceous patches occurred bilaterally on the feet. RESULTS: An incisional cutaneous biopsy and the histological examination of the plantar lesion revealed the diagnosis of Kaposi Sarcoma. CONCLUSION: We report a unique case of new-onset bilateral Kaposi's sarcoma following COVID-19, speculating on a possible role of SARS-CoV-2 in the reactivation of human herpes virus-8 (HHV-8) infection.

Are dissecting cellulitis and hidradenitis suppurativa different diseases?

Although dissecting cellulitis (DC) and hidradenitis suppurativa (HS) are classified separately, they share many clinical, dermatoscopic, pathogenetic, and histologic aspects, as well as therapeutic options. The association between DC, HS, and acne conglobata represents the follicular occlusion triad or follicular occlusion tetrad, which may include a pilonidal sinus. DC, also known as "folliculitis et perifolliculitis capitis abscendes et suffoidens," is classified as a secondary cicatricial and neutrophilic alopecia. It occurs with perifolliculitis of the scalp, dermal abscesses, sinus tract development, and secondary scarring alopecia. HS, sometimes known as acne inversa, is a chronic relapsing inflammatory disease afflicting apocrine gland-rich areas of the body with painful nodules and abscesses, sinus tracts, and scarring. Given the overlap between the clinical features and the pathogenesis of DC and HS, it would be more appropriate to consider these conditions as two different localizations of the same disease rather than two different pathologies, being a follicular occlusion disease occurring on the scalp and on the apocrine gland-rich areas of the body.

Telogen Effluvium after SARS-CoV-2 Infection: A Series of Cases and Possible Pathogenetic Mechanisms.

INTRODUCTION: Physicians have largely studied the cutaneous involvement of coronavirus disease 2019 (COVID-19), but only few reports have focused on telogen effluvium (TE) as a possible sequela of COVID-19. We assessed 14 cases of hair loss occurring after SARS-CoV-2 infection using trichoscopy and trichogram to investigate patterns related to COVID-19. Furthermore, we discussed possible mechanisms involved in COVID-19 TE. CASE PRESENTATION: Fourteen individuals were referred to our post-COVID-19 dermatology office complaining acute hair loss after SARS-CoV-2 infection. Clinical evaluation included pull test, trichoscopy, and trichogram. CO-VID-19 TE occurred after a median of 2 months (range 1-3 months) following SARS-CoV-2 infection. The median duration of hair loss was 5 months (range 1-6 months). Trichoscopy showed variable but typical TE patterns. Trichogram showed different telogen/anagen ratio depending on the interval between onset of hair loss and trichological visit. DISCUSSION/CONCLUSION: Our cases showed TE between 1 and 3 months after the onset of SARS-CoV-2 infection, thus earlier than classic TE. Trichoscopic features and trichogram showed no variations from classic TE. Different pathogenetic mechanisms including pro-inflammatory cytokines and direct viral damage on the hair follicle can be hypothesized; further studies on a larger sample are needed to better understand this condition.

Efficacy of Topical Finasteride 0.5% vs 17α-Estradiol 0.05% in the Treatment of Postmenopausal Female Pattern Hair Loss: A Retrospective, Single-Blind Study of 119 Patients.

BACKGROUND AND OBJECTIVES: Female pattern hair loss (FPHL) is a common form of scalp hair loss that occurs in 38% of females. Currently, minoxidil solution is the only therapy approved by the US Food and Drug Administration, but many other treatments are used, including cyproterone acetate, spironolactone, topical 17α-estradiol, and prostaglandin analogs. Systemic finasteride has been considered a treatment option in women even though its teratogenic effects tend to limit its prescription. Recently, topical finasteride has been evaluated to limit the side effect profile of the drug. The objective of the present study is to compare retrospectively the efficacy of topical 0.05% 17α-estradiol solution and a 0.5% finasteride lotion in the treatment of FPHL. PATIENTS AND METHODS: We enrolled 119 postmenopausal female patients. The first group comprised 69 women treated with finasteride 0.5% and minoxidil 2%. The second group included 50 women treated with 17α-estradiol 0.05% and minoxidil 2%. At baseline and at 6- and 12- to 18-month follow-up, global photographs were systematically taken. Three operators blind to the prescribed treatment evaluated photographs using a 7-point scale. One-way analysis of variance and unpaired Student t tests were performed to analyze 7-point scale scores. RESULTS: The improvement was statistically significant from 6 months to 12-18 months, both for finasteride (P < 0.005) and 17α-estradiol (P < 0.05). The efficacy of topical finasteride was significantly greater than that of 17α-estradiol solution, both at the 6-month (P < 0.05) and at the 12- to 18-month follow-up (P < 0.005). In general, the highest improvement was observed after 12-18 months of treatment with topical finasteride therapy. CONCLUSIONS: Topical finasteride 0.5% in combination with minoxidil 2% could represent a valid therapeutic option for the treatment of postmenopausal FPHL, showing higher efficacy than topical 17α-estradiol with minoxidil 2% both at 6-month and 12- to 18-month follow-up.

Italian Guidelines in diagnosis and treatment of alopecia areata.

Alopecia areata (AA) is an organ-specific autoimmune disorder that targets anagen phase hair follicles. The course is unpredictable and current available treatments have variable efficacy. Nowadays, there is relatively little evidence on treatment of AA from well-designed clinical trials. Moreover, none of the treatments or devices commonly used to treat AA are specifically approved by the Food and Drug Administration. The Italian Study Group for Cutaneous Annexial Disease of the Italian Society of dermatology proposes these Italian guidelines for diagnosis and treatment of Alopecia Areata deeming useful for the daily management of the disease. This article summarizes evidence-based treatment associated with expert-based recommendations.

Systemic mastocytosis presenting with gastrointestinal, bone and skin involvement.

Ultrasonography (US) is often the first imaging study performed in patient with abdominal pain or vague symptoms related to the gastrointestinal tract. To this end, it has been demonstrated that transabdominal sonography achieves good to excellent results in potential bowel disorders; especially, if ultrasonography findings are framed in the context of clinical information. Systemic mastocytosis is not a common disease and it usually involves skin, gastrointestinal tract and bone. It results from a clonal neoplastic proliferation of abnormal mast cells and clinically it can ranges from 'asymptomatic' with normal life expectancy to 'highly aggressive'. Symptoms are caused by the release of mast cells mediators, such as histamine, and by the increase bulk of mast cells in the tissue. We present herein a case of systemic mastocytosis presenting with abdominal symptomatology due to thickened colonic involvement showed by US associated with bone and skin involvement.