Erosive pustular dermatosis of the scalp: a multicentre study.

BACKGROUND: Erosive pustular dermatosis of the scalp (EPDS) is characterized by crusted erosions or superficial ulcerations that lead to scarring alopecia. OBJECTIVES AND METHODS: We performed a multicentre retrospective clinical study including 56 patients (29 females and 27 males, mean age 62.7) with a confirmed EPDS in order to describe epidemiology, clinical findings and therapeutic choices of this disease. RESULTS: Mechanical/chemical trauma was reported in 28.6%, a previous infection in 10.7%, a previous cryotherapy in 5.4% androgenetic alopecia in 48.2% and severe actinic damage in 25%. Trichoscopy showed absence of follicular ostia, tufted and broken hair, crusts, serous exudate, dilated vessels, pustules and hyperkeratosis. Histopathology revealed three different features, depending on the disease duration. The most prescribed therapy was topical steroids (62.5%), followed by the combination of topical steroids and topical tacrolimus (8.9%), systemic steroids (7.1%) and topical tacrolimus (5.4%). A reduction of inflammatory signs was observed in 28 patients (50%) treated with topical steroids and in all three patients treated with topical tacrolimus. CONCLUSION: The relatively high number of patients collected allowed us to identify a better diagnostic approach, using trichoscopy and a more effective therapeutic strategy, with high-potency steroids or tacrolimus, which should be considered as first-line treatment.

Patient-derived iPSCs show premature neural differentiation and neuron type-specific phenotypes relevant to neurodevelopment.

Ras/MAPK pathway signaling is a major participant in neurodevelopment, and evidence suggests that BRAF, a key Ras signal mediator, influences human behavior. We studied the role of the mutation BRAFQ257R, the most common cause of cardiofaciocutaneous syndrome (CFC), in an induced pluripotent stem cell (iPSC)-derived model of human neurodevelopment. In iPSC-derived neuronal cultures from CFC subjects, we observed decreased p-AKT and p-ERK1/2 compared to controls, as well as a depleted neural progenitor pool and rapid neuronal maturation. Pharmacological PI3K/AKT pathway manipulation recapitulated cellular phenotypes in control cells and attenuated them in CFC cells. CFC cultures displayed altered cellular subtype ratios and increased intrinsic excitability. Moreover, in CFC cells, Ras/MAPK pathway activation and morphological abnormalities exhibited cell subtype-specific differences. Our results highlight the importance of exploring specific cellular subtypes and of using iPSC models to reveal relevant human-specific neurodevelopmental events.

Beard alopecia areata: a multicentre review of 55 patients.

BACKGROUND: Alopecia areata on the beard area (BAA) is a common clinical manifestation, but there are no studies about its characteristics. OBJECTIVE: To describe the epidemiology, comorbidities, clinical presentation, evolution, diagnostic findings and therapeutic choices in a series of patients with BAA. METHODS: This retrospective multicentre review included patients diagnosed with BAA as the first and unique clinical manifestation with at least 12 months of follow-up. Diagnosis was performed based on the typical clinical features. Extra-beard involvement was monitored in all cases. RESULTS: Overall, 55 male patients with a mean age of 39.1 years (range 20-74) were included. Twenty-five patients (45.5%) developed alopecia of the scalp during follow-up and more than 80% of cases appeared in the first 12.4 months. Clinical presentation of AA on the scalp was patchy AA (less than 5 patches) (52%), multifocal AA (28%), AA totalis (12%) and AA universalis (8%). Multivariate analysis revealed a trend of association between scalp involvement and family history of AA without statistical significance. CONCLUSIONS: According to this study, BAA may progress to scalp AA in a significant number of patients (45.5% of the patients with a follow-up interval of at least 12 months). In the group of patients who developed scalp AA, 80% of them did it within the first 12 months, so follow-up of patients with BAA is highly encouraged.

Folliculitis decalvans: a multicentre review of 82 patients.

BACKGROUND: Folliculitis decalvans (FD) is a rare neutrophilic scarring alopecia that represents a therapeutic challenge for dermatologists. OBJECTIVE: To describe the epidemiology, comorbidities, clinical presentation, diagnostic findings and therapeutic options in a large series of patients with FD. METHODS: This retrospective multicentre review includes patients diagnosed with FD based on clinical and histopathologic findings. The clinical severity was determined by the maximum diameter of the largest alopecic patch (slight: <2 cm, moderate: 2-4.99 cm, severe: 5 cm or more). Response to therapy was assessed as improvement, worsening or stabilization depending on the clinical symptoms (pruritus and trichodynia), inflammatory signs (erythema, pustules and crusts) and the extension of the alopecic patch. RESULTS: Overall, 82 patients (52 males and 30 females) with a mean age of 35 years were included. No significant comorbidities were present. A family history was present in three males. Severe FD was observed in 17 patients (21%). The independent factors associated with severe FD after multivariate analysis were: onset of FD before 25 years of age and presence of pustules. Oral antibiotics (tetracyclines and the combination of clindamycin and rifampicin) improved 90% and 100% of the patients, with a mean duration of response of 4.6 and 7.2 months respectively. CONCLUSIONS: The onset of FD before 25 years of age and the presence of pustules within the alopecic patch were associated with severe FD. Tetracyclines and the combination of clindamycin and rifampicin were the most useful treatments.

Study of frontal hairline patterns in Spanish Caucasian women.

BACKGROUND: Anterior hairline measurements and their possible relationship with androgen levels, sebum production, and skin hydration have not been reported in white Spanish women. MATERIAL AND METHODS: This was a prospective descriptive and analytical observational study conducted on 103 healthy premenopausal white Spanish women recruited from the health staff of Hospital Universitario Virgen Macarena in Seville, Spain. Measurements were made of anterior hairline implantation, sebum levels, and the degree of hydration of the stratum corneum. Androgen levels were also determined in 50 volunteers from this group 3 to 5 days after the end of the menstrual cycle. RESULTS: The mean age of the women was 29.7 years. A widow's peak was observed in 94.17% of the group. The mean dimensions of the widow's peak were a height of 1.01 cm and width of 2.13 cm. The mean hormone levels were within normal limits for our hospital's laboratory with the exception of 17-hydroxyprogesterone, with a mean level of 1.39 ng/mL (range, 0.6-5.9ng/mL; normal limits, 0.15-1.10 ng/mL). The mean prostate specific antigen level was 0.04ng/mL (range 0.02-0.08ng/mL; normal limits, 0.00-0.02ng/mL). CONCLUSIONS: The hairline measurements of the white Spanish women in this study differ from those reported in American women. Knowledge of this normal pattern of anterior hairline implantation can be important in the evaluation of women with female androgenetic alopecia with male pattern, frontal fibrosing alopecia, or other established scarring alopecia seeking a surgical solution by hair transplantation.

Dermatitis artefacta of the breast: a retrospective analysis of 27 patients (1976-2006).

BACKGROUND: Dermatitis artefacta (DA) is defined as all dermatological, self-inflicted skin lesions, where the patient denies having produced the lesions. OBJECTIVES: The purpose of this study is to make a single-centre retrospective clinical review of patients diagnosed as DA of the breast. MATERIALS AND METHODS: During a 30-year period (1976-2006), patients diagnosed as DA of the breast, seen in the Department of Dermatology of the Virgen Macarena Hospital in Seville, were recorded. Clinical and epidemiological features are described. RESULTS: A total of 27 women with a mean age of 34.33 years were selected representing 13.43% of the total of DA patients recorded (n=201) in this period. The most frequent clinical forms were: excoriations (nine patients, 33.33%) and ulcers (nine patients, 33.33%), followed by burns (six patients, 22.22%), blisters (one patient, 3.70%), contact dermatitis (one patient, 3.70%) and haematomas (one patient, 3.70%). Ten of the cases were located exclusively on the breasts, whereas 17 had also other locations such as face in seven cases, arms in five cases, abdomen in five cases and the entire body in two cases. Cutaneous lesions were treated with occlusive bandages using zinc paste or plaster splint when necessary. CONCLUSION: To our knowledge, this is the major series of DA of the breast studied. This complicated psychodermatological condition requires a correct diagnosis, appropriate management and psychiatric assessment.

Once weekly administration of etanercept 50 mg is efficacious and well tolerated in patients with moderate-to-severe plaque psoriasis: a randomized controlled trial with open-label extension.

BACKGROUND: In previous studies, etanercept 25 mg twice weekly (BIW) or 50 mg BIW significantly reduced disease severity in patients with plaque psoriasis and demonstrated a favourable safety profile. OBJECTIVES: To assess the efficacy and safety of etanercept 50 mg administered once weekly (QW) compared with placebo in patients with moderate-to-severe plaque psoriasis over 24 weeks. METHODS: This study was conducted in two parts: (i) a 12-week, double-blind, placebo-controlled phase, in which patients received etanercept 50 mg QW or placebo QW; and (ii) a 12-week, open-label extension phase, in which all patients received etanercept 50 mg QW. Primary endpoint was a 75% or greater improvement from baseline in the Psoriasis Area and Severity Index (PASI 75) at week 12. Secondary endpoints included percentage PASI improvement and Physician's Global Assessment (PGA). RESULTS: One hundred and forty-two patients were analysed in the double-blind phase; 126 patients entered the open-label phase. At week 12, significantly more patients receiving etanercept 50 mg QW (37.5%) achieved PASI 75 response than patients receiving placebo (2.2%; P < 0.0001). At week 24, 71.1% in the etanercept-etanercept group and 44.4% in the placebo-etanercept group achieved PASI 75. Mean percentage of PASI improvement from baseline was 55.4% with etanercept vs. 9.4% worsening with placebo at week 12 (P < 0.0001), with 77.4% and 57.7% improvement in the etanercept-etanercept and placebo-etanercept groups at week 24. A PGA score of 0-1 (clear-almost clear) was achieved by 64% and 42% in the etanercept-etanercept and placebo-etanercept groups at week 24, respectively. Etanercept 50 mg QW was well tolerated. No deaths, serious infections, opportunistic infections (including tuberculosis), demyelinating disorders, malignancies or new safety signals were reported. CONCLUSIONS: Nearly three-quarters of patients with moderate-to-severe psoriasis receiving etanercept 50 mg QW achieved significant improvement in disease severity over 24 weeks. This study also showed a favourable tolerability and safety profile with etanercept 50 mg QW.

Granulomatous slack skin disease in a child: the outcome.

Granulomatous slack skin syndrome is a rare clinical and pathologic disorder. Only 42 patients have been reported, one of whom we described in 1997--the only child so far reported. We now describe the evolution of this patient and the transformation of the disease into a peripheral T-cell lymphoma, and the complications resulting in the child's death.

The different faces of pili bifurcati. A review.

Pili bifurcati is an uncommon hair shaft dysplasia characterized by bifurcation of the hair shaft. The two characteristics that define the dysplasia are: 1. Each bifurcation produces two separate parallel branches which fuse again to form a single shaft. 2. Each branch of the successive bifurcations is covered with its own cuticle. Currently, there is confusion between the terms "pili bifurcati" and "pili gemini". The name "pili gemini" is used to define a kinetic papilla that splits at the upper end from single to double-tipped during the anagen phase and consequently the same follicular matrix produces two different-sized hair shafts having separate cuticles that emerge through a single pilary canal. Pili gemini maintains the double tipped papilla and consequently the hair shaft does not fuse again. Papillar tips that divide into several tips will produce several hair shafts, that characteristically do not fuse again. When the same papilla changes its shape repeatedly during the anagen phase, it can produce hair shafts with bifurcations at irregular intervals: pili multibifurcati. And, as it is also possible for one of the new papillae to split again in two, the hair shaft may be doubly bifurcated: pili bi, bifurcati. Pili bifurcati should be distinguished from acquired splitting of hair shafts that do not represent true bifurcations because the two split parts are never surrounded by a complete cuticle. Such acquired splitting may be called "central trichoptilosis".

Primary multifocal localized hypertrichosis.

We describe a new variant of congenital hypertrichosis, primary multifocal localized hypertrichosis in a mother and her son. They presented with long vellus hair on the trunk and arms in foci from birth. They also associated some forms of hair dysplasia.

M-plasty in the treatment of carcinomas located on the interglabellar region.

One of the main tasks of current cutaneous surgery is to completely eliminate the tumor while avoiding unnecessary normal tissue extirpation. This is especially important when removing facial tumors close to the external canthus, mouth angles and perialar regions. In all these areas we advise fusiform excision but with distal M-plasty in the nearest angle so as to avoid angle retraction. The same is true for mediofrontal tumors, as this often causes a drawing together of the eyebrows. We have reviewed all mediofrontal excisions carried out during the last six years. M-plasty following fusiform excision was always performed in the interglabellar area. The good results achieved allow us to recommend this technique in this location.