Sergio Vano-Galvan

Frontal fibrosing alopecia: A multicenter review of 355 patients

BACKGROUND To our knowledge, there are no large multicenter studies concerning frontal fibrosing alopecia (FFA) that could give clues about its pathogenesis and best treatment. OBJECTIVE We sought to describe the epidemiology, comorbidities, clinical presentation, diagnostic findings, and therapeutic choices in a large series of patients with FFA. METHODS This retrospective multicenter study included patients given the diagnosis of FFA. Clinical severity was classified based on the recession of the frontotemporal hairline. RESULTS In all, 355 patients (343 women [49 premenopausal] and 12 men) with a mean age of 61 years (range 23-86) were included. Early menopause was detected in 49 patients (14%), whereas 46 (13%) had undergone hysterectomy. Severe FFA was observed in 131 patients (37%). Independent factors associated with severe FFA after multivariate analysis were: eyelash loss, facial papules, and body hair involvement. Eyebrow loss as the initial clinical presentation was associated with mild forms. Antiandrogens such as finasteride and dutasteride were used in 111 patients (31%), with improvement in 52 (47%) and stabilization in 59 (53%). LIMITATIONS The retrospective design is a limitation. CONCLUSIONS Eyelash loss, facial papules, and body hair involvement were associated with severe FFA. Antiandrogens were the most useful treatment.

Dermoscopic Features of Skin Lesions in Patients With Mastocytosis

OBJECTIVES To evaluate dermoscopic features in a group of 127 patients with mastocytosis in the skin and to investigate the relationship between different dermoscopic patterns and other clinical and biological characteristics of the disease. DESIGN Clinical and laboratory data were compared among patients with mastocytosis grouped according to the different dermoscopic patterns. SETTING Patients were selected from the Instituto de Estudios de Mastocitosis de Castilla La Mancha and the Department of Dermatology of Hospital Universitario Ramón y Cajal from April 1 through September 30, 2009. Patients Overall, 127 consecutive patients (70 females [55.1%] and 57 males [44.9%]; median age, 17 years; range, 0-81 years) with mastocytosis in the skin were included in the study. MAIN OUTCOME MEASURES Evaluation of dermoscopic patterns and investigation of potential predictive factors for more symptomatic forms of the disease according to the need for daily antimediator therapy. RESULTS Four distinct dermoscopic patterns were observed: yellow-orange blot, pigment network, reticular vascular pattern, and (most frequently) light-brown blot. A reticular vascular pattern was identified in all telangiectasia macular eruptiva and some maculopapular mastocytosis. In turn, all patients with mastocytoma displayed the yellow-orange blot pattern. The reticular vascular dermoscopic pattern was associated with the need for daily antimediator therapy; this pattern, together with serum tryptase levels and plaque-type mastocytosis, represented the best combination of independent factors to predict the need for maintained antimediator therapy. CONCLUSIONS Dermoscopy is a feasible method for the subclassification of mastocytosis. Of note, a reticular vascular pattern is more frequently associated with the need for antimediator therapy.

Folliculocystic and collagen hamartoma of tuberous sclerosis complex

BACKGROUND Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by tumors and hamartomas in several organs including the skin. OBJECTIVE We sought to describe a new type of complex hamartoma in patients with TSC. METHODS This was a retrospective clinical and histopathologic evaluation of 6 cases. RESULTS The skin lesions consisted of large, painless, infiltrated plaques that were first noticed at birth or during early infancy on the abdomen, thigh, back, or scalp. In time, the plaques became studded with numerous follicular comedo-like openings and cysts containing and draining a keratinous or purulent material. The main histopathologic features were: abundant collagen deposition in the dermis and extending into the underlying fat; concentric, perifollicular fibrosis surrounding hair follicles; and comedones and keratin-containing cysts lined by infundibular epithelium, some of which were ruptured with secondary granulomatous reaction. Five of the 6 patients had a clinical diagnosis of TSC. LIMITATIONS Genetic testing was performed in only one patient. CONCLUSION This distinctive folliculocystic and collagen hamartoma has not been recognized previously in association with TSC.

Trichoscopic features of frontal fibrosing alopecia: Results in 249 patients

To the Editor: Currently, dermoscopy constitutes an essential noninvasive tool for dermatologists. It helps discern between different types of alopecia, it provides a more precise follow-up, and it can be used to identify an adequate biopsy site. Frontal fibrosing alopecia (FFA) is a primary lymphocytic scarring alopecia with a distinctive clinical pattern of progressive frontotemporal hairline recession and eyebrow loss that mainly affects postmenopausal women. The main objective of our study was to describe the trichoscopic features of FFA in a large series of patients and to correlate these findings with several relevant parameters of FFA. We performed a descriptive, retrospective, observational, multicenter study of digital trichoscopic images, obtained between 1994 and 2013, of 238 women with FFA at 12 Spanish centers. Diagnostic criteria included typical clinical signs and/or histopathologic features consistent with FFA. Images were obtained with either a nonpolarizing or a polarizing dermoscope. Two dermatologists expert in dermoscopy evaluated the images if the registered trichoscopic features of FFA (cicatricial white patches, perifollicular erythema, follicular hyperkeratosis, lonely hairs, and hair diameter diversity) and yellow dots typical of androgenetic alopecia were present on the frontotemporal hairline. Both a descriptive and an analytic study to correlate these parameters with the degree of severity and other clinical variables were performed using SPSS 15.0 software. Clinical and severity variables included female pattern hair loss (FPHL), presence or absence of menopause, FFA severity (I: 1 cm, II: 1-2.99 cm, III: 3-4.99 cm, IV: 5-6.99 cm, and V: [7 cm), and years of evolution, pruritus, trichodynia, facial papules, occipital involvement, eyebrow and eyelash loss, pubis hair loss, and body hair involvement. A bivariate analysis including trichoscopic findings and the aforementioned variables was carried out, and those variables with statistical significance in X test were included in a multivariate logistic regression analysis adjusted for age and excluding lost cases. No new dermoscopic signs were found. Descriptive dermoscopic results are listed in Table I. Both the intraobserver and interobserver agreement for the assignment of a dermoscopic pattern for each lesion were excellent (! 1⁄4 0.82, P\.001; ! 1⁄4 0.80, P\.001, respectively). The trichoscopic features that were statistically significantly associated (P \ .05) with some clinical parameters in the bivariate and multivariate logistic regression analysis are listed in Table II. Dermoscopic features of FFA have previously been described in some isolated studies. Toledo-Pastrana et al retrospectively analyzed the dermoscopic images of 79 patients with FFA. They found that 100% of the patients showed no follicular openings, 72.1% showed follicular hyperkeratosis, 66.3% showed perifollicular erythema, and 44.8% showed follicular plugs. Interestingly, they also found that perifollicular erythema was statistically associated to the activity of FFA. In our study, we correlated the dermoscopic features with another outcome: the severity of the disease in terms of extension of the hairline. Remarkably, we found that the presence of cicatricial white patches was statistically associated with the severity of FFA. This dermoscopic feature correlates with the histopathologic findings of hair follicle destruction and severe tissue fibrosis. Therefore, it

Folliculitis decalvans: a multicentre review of 82 patients

Folliculitis decalvans (FD) is a rare neutrophilic scarring alopecia that represents a therapeutic challenge for dermatologists.

Black hairy tongue.

A 71-year-old man presents for evaluation of an asymptomatic black discoloration of the tongue. What is the most likely diagnosis?

Classic Kaposi's sarcoma treated with topical rapamycin

Kaposis sarcoma (KS) is an angioproliferative disorder caused by human herpesvirus 8 (HHV‐8). Current research efforts have focused on the study of the relative role of KSHV‐encoded genes in Kaposis sarcomagenesis in order to identify novel mechanism‐based therapies for patients suffering from this tumor. Although several viral genes have potential for KS pathogenesis, compelling data point to the KSHV‐encoded G protein‐coupled receptor (vGPCR) as a leading candidate viral gene for the initiation of KS. Interestingly, the oncogenic potential of vGPCR seems to correlate with its capacity to activate the mammalian target of rapamycin (mTOR) signaling pathway. Rapamycin, the prototypical inhibitor of the mTOR signaling pathway, has recently emerged as an effective treatment for KS when administered orally. In this case report, we present an immunocompetent patient with KS lesions treated with topical rapamycin achieving clinical and histologic healing after 16 weeks of treatment. The topical application of rapamycin could be a novel therapeutic option for the treatment of KS.

Bowel-associated dermatosis-arthritis syndrome: another cutaneous manifestation of inflammatory intestinal disease.

Actinomadura madurae. The patient was treated for actinomycetoma with oral amoxicillin-clavulanic acid (500/125 mg) three times per day for 12 months. The foot showed a remarkable reduction in diameter from 42 to 27 cm and the patient was able to wear an ordinary shoe for the first time in 10 years. Mycetoma is a chronic cutaneous infection caused by either fungi (eumycetoma) or Actinomycetes (actinomycetoma). Of all cases of mycetoma, 60% are actinomycetomas and 40% are eumycetomas. Nocardia brasiliensis is the most common cause of actinomycetoma. Classically, mycetoma presents as a painless nodule and slowly progresses to cutaneous and subcutaneous edema and sinus tract formation. There is usually extrusion of purulent material containing the characteristic grains. Treatment regimens vary according to the organism causing the infection. Eumycetomas are usually more challenging as they may respond poorly to antifungal therapy. Surgical intervention is often required for the debridement of infected tissue. By contrast, actinomycetomas are generally more responsive to drug therapy, especially if treatment is initiated early in the course of the disease. Different therapeutic options have been used to treat actinomycetomas and include dapsone plus sulfamethoxazol-trimethoprim, and streptomycin or amikacin or amoxicillin plus clavulanic acid. Other authors have suggested the use of a combination therapy for the cure of actinomycetomas and to prevent the emergence of resistance; however, no prospective, randomized therapeutic trials of these therapies have been conducted. Actinomycetomas have a favorable prognosis with cure rates ranging from 60% to 90%. Nevertheless, therapy must be continued for a prolonged period.

Successful treatment of large venous malformation with dual wavelength 595 and 1064 nm system

Venous malformations (VMs) are congenital soft, compressible, non‐pulsatile blue‐purple masses. VMs present a therapeutic challenge. They can be treated with surgery or sclerotherapy as well as with several lasers. We report the successful treatment of two patients with large VMs with combined sequential application of 595 nm pulsed dye laser and 1064 nm Nd:YAG wavelengths. At the end of the treatment course, total volume reduction was observed in both. All sessions were well tolerated without anesthesia. In our patients, using this sequential laser device achieved an almost complete clearance of the VM with no scarring.

Teledermatología diferida: análisis de validez en una serie de 2.000 observaciones

BACKGROUND AND OBJECTIVES The aim of this study was to assess the validity of store-and-forward teledermatology as a tool to support physicians in primary care and hospital emergency services and reduce the requirement for face-to-face appointments. Diagnostic validity and the approach chosen for patient management (face-to-face vs teledermatology) were compared according to patient origin and diagnostic group. MATERIAL AND METHODS Digital images from 100 patients were assessed by 20 different dermatologists and the diagnoses offered were compared with those provided in face-to-face appointments (gold standard). The proposed management of the different groups of patients was also compared. RESULTS The percentage complete agreement was 69.05% (95% confidence interval [CI], 66.9%-71.0%). The aggregate agreement was 87.80% (95% CI, 86.1%-89.0%). When questioned about appropriate management of the patients, observers elected face-to-face consultation in 60% of patients (95% CI, 58%-61%) and teledermatology in 40% (95% CI, 38%-41%). Diagnostic validity was higher in patients from primary care (76.1% complete agreement and 91.8% aggregate agreement) than those from hospital emergency services (61.8% complete agreement, 83.4% aggregate agreement) (p < 0.001) and teledermatology was also chosen more often in patients from primary care compared with those from emergency services (42% vs 38%; p=0.003). In terms of diagnostic group, higher validity was observed for patients with infectious diseases (73.3% complete agreement and 91.3% aggregate agreement) compared to those with inflammatory disease (70.8% complete agreement and 86.4% aggregate agreement) or tumors (63.0% complete agreement and 87.2% aggregate agreement) (p <0.001). Teledermatology was also chosen more often in patients with infectious diseases (52%) than in those with inflammatory disease (40%) or tumors (28%) (p <0.001). CONCLUSIONS Store-and-forward teledermatology has a high level of diagnostic validity, particularly in those cases referred from primary care and in infectious diseases. It can be considered useful for the diagnosis and management of patients at a distance and would reduce the requirement for face-to-face consultation by 40%.