Frontal Fibrosing Alopecia and Sunscreen Use: A Cross-sectional Study of Actinic Damage.

Patients with frontal fibrosing alopecia report higher rates of sunscreen use than control subjects. However, it is not known whether the higher use of sunscreens is a cause or a consequence of the alopecia. A greater use of sunscreens should be associated with a lower incidence of signs of actinic damage. The aim of this study is to assess the presence of actinic damage in patients with frontal fibrosing alopecia. A cross-sectional study was carried out on 101 patients with frontal fibrosing alopecia and 40 control subjects. The presence of actinic damage, in the form of solar lentigines, actinic keratoses, and basal and squamous cell carcinomas, was recorded in both groups, together with sunscreen use. Trichoscopy and skin biopsy were performed on patients. Actinic damage was present more frequently in patients with frontal fibrosing alopecia (69.3%) than in control subjects (50%) (p = 0.031). Patients used sunscreens more frequently than did control subjects (83.2% vs 62.5%, p = 0.008). However, the prevalence of trichoscopic inflammatory signs, peripheral alopecia, and inflammatory infiltrate and sebaceous gland involvement in skin biopsy, were similar in patients who used sunscreens and those who did not use them. In conclusion, patients with frontal fibrosing alopecia had greater actinic damage than did control subjects, and this is hypothesized as a reason for the higher use of sunscreens among patients. Thus, use of sunscreens may not be the trigger for frontal fibrosing alopecia that dermatologists have proposed.

Colour Doppler ultrasound study in patients with frontal fibrosing alopecia.

BACKGROUND: The sonographic characteristics of frontal fibrosing alopecia have been scarcely studied. The aim of this study was to perform a colour Doppler ultrasound evaluation in frontal fibrosing alopecia. MATERIALS AND METHODS: A cross-sectional study including 99 women with frontal fibrosing alopecia and 40 control subjects was performed using ultrasound equipment with a lineal 18 MHz probe. Three areas were evaluated per patient: the alopecic area (a), the hairline implantation area (b) and healthy scalp (c). The diameter (cm) and flow (m/s) of the two most significant vessels were recorded. RESULTS: With regard to the hairline implantation area, patients presented higher vessel diameter (0.127 cm vs 0.103 cm, P = .03) and vessel flow (8.183 m/s vs 7.670 m/s, P = .05) than the control group. Vessel diameter was higher in the healthy scalp area in patients than in the control group (0.088 cm vs 0.078 cm, P = .03). CONCLUSION: Patients presented higher vessel diameter and flow in the hairline implantation area compared to the control group.

A Cross-sectional Study of Rosacea and Risk Factors in Women with Frontal Fibrosing Alopecia.

Frontal fibrosing alopecia has been related to some autoimmune diseases, but the association with rosacea is not clear. The objective of this study was to analyse the prevalence of rosacea in a group of patients with frontal fibrosing alopecia. A cross-sectional study, including 99 women with frontal fibrosing alopecia and 40 controls, was performed, in which clinical, dermoscopic and hormonal data were analysed. Women with frontal fibrosing alopecia presented a higher prevalence of rosacea than the controls did (61.6% vs. 30%, p = 0.001), especially those with severe grades of alopecia (77.8% in grade V vs. 33.3% in grade I, p = 0.02). Binary logistic multivariate analysis showed that perifollicular erythema (odds ratio (OR) 8.5; 95% confidence interval (95% CI) 1.73-42.30), higher body mass index (OR 1.16; 95% CI 1.01-1.34) and lower progesterone levels (OR 0.15; 95% CI 0.028-0.89) were associated with a higher risk of rosacea in patients with frontal fibrosing alopecia. In conclusion, patients with frontal fibrosing alopecia presented a higher prevalence of rosacea than did controls. Perifollicular erythema, higher body mass index and lower progesterone levels were associated with a higher risk of rosacea in the group with frontal fibrosing alopecia.

Familial frontal fibrosing alopecia: A cross-sectional study of 20 cases from nine families.

BACKGROUND/OBJECTIVES: Frontal fibrosing alopecia (FFA) is a scarring alopecia whose prevalence is increasing. The pathogenesis of this disease is not well known. Genetic, environmental, hormonal and autoimmunity related factors have been considered; however, only a few cases of familial frontal fibrosing alopecia have been reported. MATERIAL AND METHODS: A cross-sectional study was performed at University Hospital in Granada (Spain). Twenty patients with frontal fibrosing alopecia belonging to nine different families were included, and clinical and dermoscopic features were analysed. RESULTS: Overall, 90% of the patients studied were women (mean age 61.4 years). About 50% of the patients had grade II frontal fibrosing alopecia at the time of diagnosis, whilst 35% had grades III or V. Mean recession was 2.83 cm in the frontal area and 1.99 cm in the temporo-parietal area. Daughters presented a shorter recession area and earlier debut of the disease than mothers. Androgenetic alopecia was found in only two patients (10%). The dermoscopic signs most commonly found were perifollicular erythema (85%), hyperkeratosis (85%), and absence of vellus hair in the hairline (78.9%). CONCLUSION: This study adds to the growing evidence that there is a genetic component to frontal fibrosing alopecia. The clinical pattern of frontal fibrosing alopecia was not different from that found in non-familial cases, but the debut of the disease in daughters of mothers with frontal fibrosing alopecia may be earlier.

Study of Human Leukocyte Antigen (HLA) in 13 cases of familial frontal fibrosing alopecia: CYP21A2 gene p.V281L mutation from congenital adrenal hyperplasia linked to HLA class I haplotype HLA-A*33:01; B*14:02; C*08:02 as a genetic marker.

BACKGROUND/OBJECTIVES: The aetiology of frontal fibrosing alopecia is unknown, and its genetic aspect remains uncharacterised. The aim of this report is to elucidate if major histocompatibility complex is associated with familial frontal fibrosing alopecia. METHODS: A case-control study was performed of 13 patients with frontal fibrosing alopecia belonging to six families. Their human leukocyte antigen profiles were compared to the data of 636 healthy controls without frontal fibrosing alopecia. Patients underwent high-resolution genomic typing for human leukocyte antigen class I and II loci by PCR-SSO for Luminex. In addition, CYP21A2 gene (major histocompatibility complex class III) mutations were detected by PCR-SSO on strips. RESULTS: 61.5% of patients shared CYP21A2 gene p.V281L linked to the F16A human leukocyte antigen class I haplotype (HLA-A*33:01; B*14:02; C*08:02; Pc < 0.000001). The patients F16A-negative shared other human leukocyte antigen class I haplotypes: Y16A (3/13) and S26 (2/13). CONCLUSION: CYP21A2 gene p.V281L mutation can be used as a genetic marker for susceptibility to familial frontal fibrosing alopecia. Both the linkage of the mutation to F16A and the fact that F16A-negative patients share other human leukocyte antigen class I haplotype, point to an antigen-driven mechanism in susceptible patients with these haplotypes.

Hobnail Hemangioma With an Unusual Clinical Presentation.

Hobnail hemangioma, also known as targetoid hemosiderotic hemangioma, is an uncommon vascular proliferation that clinically presents as a small solitary red to purple papule or macule, located on the limbs or trunk. Multiple lesions and atypical locations have been described. Histopathologically, it exhibits a biphasic pattern, with dilated vessels in the superficial dermis and angulated vessels in the deeper dermis, with endothelial cells that show a hobnail appearance. There is controversy about the histogenetic origin of hobnail hemangioma, although recent studies support that it is a lymphatic malformation. The investigators report the case of a 41-year-old man with an irregular lesion, red to purple in color, with a maximum diameter of 4 cm, on the scalp. The location and in particular the clinical appearance are uncommon. Immunohistochemical analysis showed negativity for WT1 and focal positivity for D2-40. Clinical-pathologic correlation acquires particular importance in the case of lesions with atypical clinical presentation.

Frontal Fibrosing Alopecia Quality of Life Index: A Validated Disease-Specific Questionnaire Involving Women.

Quality of life (QoL) can be affected in patients with alopecia. The few studies that evaluate QoL in FFA use unspecific questionnaires. The aim of this report was to design and validate a specific questionnaire to assess the impairment of QoL in FFA patients. A specific questionnaire, called the Frontal Fibrosing Alopecia Quality of Life Index (FFA-QLI), was designed and validated using the Dermatology Life Quality Index (DLQI). One-hundred and one women with FFA were included. Cronbach's alpha value was 0.865, and the intraclass correlation coefficient between all the items in the questionnaire was 0.870. The FFA-QLI correlated positively with the DLQI (r = 0.729, p < 0.001). Patients with severe FFA showed a higher FFA-QLI (19.72) score compared to those with a mild disease (14.11) (p = 0.002), and the area under the curve for identifying severe disease was greater in the FFA-QLI than in the DLQI. The cut-off points were used to select patients with mild, moderate, and severe impairment in QoL. A score < 21 in the FFA-QLI corresponded to a low impact on QoL; values > 35 matched with greater QoL impairment; and values ranging from 21 to 35 corresponded to moderate QoL alteration. To conclude, a validated disease-specific questionnaire to assess QoL in FFA patients is here presented, with a greater power to discriminate severe cases of FFA than the DLQI.

Frontal Fibrosing Alopecia: A Histopathological Comparison of the Frontal Hairline with Normal-Appearing Scalp.

Frontal fibrosing alopecia is characterized by the presence of a lymphocytic inflammatory infiltrate around the upper follicle and by perifollicular fibrosis, which results in the destruction of the hair follicle. Recent reports have also found the presence of those findings in clinically unaffected areas. The aim of this report is to perform a deeper analysis of the histopathological features of this apparently unaffected scalp. A cross-sectional study including 52 women with frontal fibrosing alopecia was performed. Two areas were biopsied: the frontal hairline and a normal-appearing scalp area. Sebaceous glands were reduced/absent in 80.8% of the frontal hairline samples compared to 42.3% of the "healthy scalp" samples (p = 0.001). Inflammatory infiltrate was observed in 92.3% of patients in the frontal hairline and in 86.5% of them in the "healthy scalp" area (p = 0.508), although the severity was higher in the former (p = 0.013). Follicular epithelium changes were seen in 70.6% of the frontal hairline biopsies compared to 48.1% of the "healthy scalp" biopsies (p = 0.012). Fibrous tissular changes were noted in 80.8% and 53.8% of the frontal hairline and "healthy scalp" biopsies, respectively (p = 0.003). In conclusion, the histopathological features of frontal fibrosing alopecia are shared by both affected and clinically unaffected areas.

Hemangiopericytoma-like dermatofibroma with mast cells.

We report an unusual case of hemangiopericytoma-like dermatofibroma in the right shoulder of an 82-year-old patient with a well-defined nodular growth located in the dermis. Microscopic study revealed a band of haphazardly arranged cells with a vascular component of gaping, simple, endothelial-lined vascular structures with intervening postcapillary venules and capillary-sized slit-like "staghorn" vascular channels filled with erythrocytes; abundant mast cells were also observed. The neoplasm cells were positive for CD68 and Factor XIIIA and negative for CD34. Few data have been published on the presence of abundant mast cells (tryptase and CD117 positive) in these neoplasm. The differential diagnosis of this entity should consider other spindle cell neoplasm, including hemangiopericytoma/solitary fibrous tumor, dermatofibrosarcoma protuberans, myopericytoma, angioleiomyoma, amelanotic melanoma, pecoma, and benign and malignant peripheral nerve tumors. We present an infrequent case of dermatofibroma with a vascular pattern resembling hemangiopericytoma and the presence of abundant mast cells, which may be responsible for this vascular component.

Trichoscopic Findings in Pressure Alopecia: Report of Two Cases and Review of the Literature.

Pressure alopecia (PA) is an uncommon type of hair loss due to ischemic changes of the scalp, as a result of prolonged immobilization. Clinically, it often appears within the 1st month of the trigger and tends to resolve spontaneously within 4 months. If the duration of the immobilization is longer, irreversible alopecia can be developed. Trichoscopy is usually nonspecific, being black dots, broken, and dystrophic hairs the most frequent findings. However, yellow dots and thin hairs have also been reported. We herein present two patients with PA, one with a recent development and another one with a long-lasting alopecia. Both of them showed keratotic follicular plugs and thin hairs as the main trichoscopic findings.

Frontal Fibrosing Alopecia: A Review.

Frontal fibrosing alopecia is a scarring alopecia, the prevalence of which is increasing worldwide since its first description in 1994. The reason for this emerging epidemic may be a higher exposure to an unknown trigger, although its aethiology and pathogenesis still remain enigmatic. Clinical, trichoscopic, sonographic, and histopathologic findings are allowing clinicians to understand more aspects about this type of cicatricial alopecia. Several treatments have been used in frontal fibrosing alopecia, although the 5-alpha reductase inhibitors seem to be the most promising. The aim of this report is to provide a compilation about the published data regarding frontal fibrosing alopecia in a narrative review.

Exophytic and ulcerated lesion on the leg.

A healthy, 34-year-old male presented with a 1-year history of an exophytic lesion on the leg during. On physical examination there was a 2 cm x 3 cm diameter nodule with pinkish, crusted ulceration on its surface; it was attached to skin by a pedicle. It had grown rapidly in the last month. After surgical excision the histological study confirmed the diagnosis of a polypoid dermatofibroma.

Homogeneous blue pattern: A rare presentation in an acral congenital melanocytic nevus.

Acral melanocytic nevi are relatively frequent in the palmoplantar location. In congenital nevi various characteristic dermoscopic patterns have been described, such as reticular and globular patterns, brown pigmented areas, and areas with peripilar depigmentation. However, there are few reports on the dermoscopic pattern of acral congenital nevi. The homogeneous blue pattern is typical of blue nevus and is not typical of acral localization and metastasis of cutaneous melanoma. However, this pattern should be considered characteristic of acral congenital nevus.

Atypical Kaposi's sarcoma in young inmunocompetent patient.

Kaposi´s sarcoma is a rare tumor associated with human herpes virus 8 (HHV-8) infection. Four main clinical subtypes have been described. This study reports on a form of KS in an HIV negative and immunocompetent middle-aged man. The only remarkable factor is that he has sex with other men. This form of Kaposi´s sarcoma is rare. It occurs more in younger patients than in the classic form, is limited to the skin, and is associated with a good prognosis. The means of transmission of the virus is through saliva in oroanal or orogenital sexual practices. Mechanisms of tumor development are still not well known. Given the possible increased number of this variant, it would be interesting to extend this study.

Dermatoscopy-guided therapy of pigmented basal cell carcinoma with imiquimod.

BACKGROUND:: Dermatoscopy is a non-invasive diagnostic tool used to examine skin lesions with an optical magnification. It has been suggested as a useful tool for monitoring therapeutic response in lentigo maligna patients treated with imiquimod. OBJECTIVE:: To examine the accuracy of dermatoscopy as a tool to monitor the therapeutic response of pigmented basal cell carcinoma treated with imiquimod. METHOD:: The authors designed a prospective study. Patients with pigmented basal cell carcinoma were included and data regarding the dermatoscopy features were collected following the Menzies criteria, prior to initiating the imiquimod treatment. Subsequent dermatoscopic evaluations were performed at weeks 4 and 8, following imiquimod discontinuation. RESULTS:: Twenty lesions were included. The most common pigmented dermatoscopy features were large blue-grey ovoid nests (80%), followed by blue-grey globules (50%) and leaf-like areas (30%). No spoke wheel areas were observed. In 17 out of 20 patients, a response was noted during the first evaluation at 4 weeks, while the clearance was noted at the second check-up after 8 weeks. In two patients, the clearance was found at the initial evaluation at 4 weeks, while in one patient, the response remained unchanged. Blue-grey globules were the fastest to exhibit clearance (50% at week 4), followed by leaf-like areas (15%) and large blue-grey ovoid nests (6.25%). CONCLUSION:: According to our results, dermatoscopic evaluation enhances the accuracy in the assessment of the clinical response to imiquimod in pigmented basal cell carcinoma.