Clinical efficacy of fecal microbial transplantation treatment in adults with moderate-to-severe atopic dermatitis.

BACKGROUND: Atopic dermatitis (AD) is a remitting relapsing chronic eczematous pruritic disease. Several studies suggest that gut microbiota may influence AD by immune system regulation. METHODS: We performed the first in-human efficacy and safety assessment of fecal microbiota transplantation (FMT) for AD adult patients. All patients received 2 placebo transplantations followed by 4 FMTs each 2 weeks apart. AD severity and fecal microbiome profile were evaluated by the Scoring Atopic Dermatitis Score (SCORAD), the weekly frequency of topical corticosteroids usage, and gut microbiota metagenomic analysis, at the study beginning, before every FMT, and 1-8 months after the last FMT. RESULTS: Nine patients completed the study protocol. There was no significant change in the SCORAD score following the two placebo transplants. The average SCORAD score significantly decreased from baseline at Weeks 4-12 (before and 2 weeks after 4 times of FMT) (59.2 ± 34.9%, Wilcoxon p = .011), 50% and 75% decrease was achieved by 7 (77%) and 4 (44%) patients, respectively. At Week 18 (8 weeks after the last FMT) the average SCORAD score decreased from baseline at Week 4 (85.5 ± 8.4%, Wilcoxon p = .018), 50% and 75% decrease was achieved by 7 (77%) and 6 (66.7%) patients respectively. Weekly topical corticosteroids usage was diminished during the study and follow-up period as well. Two patients had a quick relapse and were switched to a different treatment. Two patients developed exacerbations alleviated after an additional fifth FMT. Metagenomic analysis of the fecal microbiota of patients and donors showed bacterial strains transmission from donors to patients. No adverse events were recorded during the study and follow-up period. CONCLUSIONS: FMT may be a safe and effective therapeutic intervention for AD patients, associated with transfer of specific microbial species from the donors to the patients. Further studies are required to reconfirm these results.

An Uncommon Presentation of Darier-White Disease with Hystrix-like Palmoplantar Keratoderma.

Darier-White disease is a relatively common autosomal dominant genodermatosis caused by mutation in the ATP2A2 gene. It is characterized by multiple warty papules coalescing into plaques in the seborrheic areas and by specific histological skin changes. Palm and sole involvement in Darier-White disease is usually mild, mainly featuring discrete and small keratotic papules. We present a unique case of Darier-White disease presenting with a diffuse, mutilating hystrix-like palmoplantar keratoderma.

FOXP3 Predicts Response to Treatment in Mycosis Fungoides.

BACKGROUND: The role of the T-regulatory cells (Tregs) marker forkhead box Protein 3 (FOXP3) in mycoses fungoides (MF) pathogenesis is unclear and the results of previous studies are inconclusive. OBJECTIVE: We aimed at ascertaining the possibility that FOXP3 expression may serve to predict MF stage and response to therapy. PATIENTS AND METHODS: Immunohistochemistry staining for FOXP3 was performed on 30 skin biopsies from patients with MF, and FOXP3 expression level was quantitatively graded. Disease stage, progression, and response to treatment were determined based on clinical and imaging evidence, and association with FOXP3 expression was assessed. RESULTS: FOXP3 expression in the dermis correlated with poor response to treatment (P=0.047). A negative non-significant relationship between epidermal FOXP3 expression and clinical stage severity was observed (P=0.17). CONCLUSIONS: Dermal FOXP3 expression in MF lesions could be used to predict response to treatment in patients with MF.

Diffuse Facial Hyperpigmentation as a Presenting Sign of Lupus Erythematosus: Three Cases and Review of the Literature.

Lupus erythematosus (LE) is an autoimmune disorder commonly affecting the skin; cutaneous lesions may indicate systemic involvement, warranting further evaluation. Photosensitivity, which may result in hyperpigmentation, is a well-known feature of the disease. In contrast, the prevalence of primary hyperpigmentation as a presenting sign of LE is not well established. Here, we compare 3 unique cases of diffuse facial hyperpigmentation as the primary manifestation of LE (cutaneous or systemic) and review previously reported cases. Our data highlight the need for considering LE in the differential diagnosis of facial hyperpigmentation and substantiate the importance of this unique lupus variant in early diagnosis and patient evaluation.

Pulse-Dye Laser Followed by Betamethasone-Calcipotriol and Fractional Ablative CO2-Laser-Assisted Delivery for Nail Psoriasis.

BACKGROUND: Nail psoriasis is a common and potentially debilitating condition for which no effective and safe nonsystemic therapy is currently available. Recently, laser-assisted drug delivery (LADD) is being increasingly used to facilitate transcutaneous penetration of topical treatments. OBJECTIVES: We set to assess the efficacy and safety of combined pulse-dye laser and fractional CO2 laser-assisted betamethasonecalcipotriol gel delivery for the treatment of nail psoriasis. MATERIAL AND METHODS: We conducted a prospective, intrapatient comparative study in a series of 22 patients with bilateral fingernail psoriasis. Nails on the randomized hand were treated with 3 monthly sessions of pulse-dye laser to the proximal and lateral nail folds followed by fractional ablative CO2 laser to the nail plate. Between treatments and one month following the last treatment, the participants applied betamethasone propionate-calcipotriol gel once daily to the nail plate. Clinical outcome was ascertained using nails photography, the Nail Psoriasis Severity Index (NAPSI) and patient satisfaction. RESULTS: Seventeen completed the study. Three participants withdrew from the study because of treatment-associated pain. Treatment was associated with a statistically significant improvement of the NAPSI scale (p < .002). Patient satisfaction was high. CONCLUSION: Combined PDL and fractional ablative CO2-LADD of betamethasone-calcipotriol gel should be considered for the treatment of nail psoriasis.

Solar urticaria: Clinic, diagnostic, course and therapy management in 27 patients.

BACKGROUND AND OBJECTIVES: Solar urticaria is a rare photodermatosis, the diagnosis and therapy of which have not yet been standardized. The aim of this research was to use innovative radiation sources for diagnostics with defined and reproducible emission spectra and doses. A uniform therapy step scheme was to be created. PATIENTS AND METHODS: In a longitudinal study, 27 patients with solar urticaria were examined over 13 years. With a characteristic anamnesis, the diagnosis was confirmed with phototesting (photoprovocation) from various radiation sources (UVB, UVB311nm, UVA, UVA-1, green light, red light) and a therapy step scheme was designed consisting of light protection, antihistamines, rush hardening with UVA-1, and administration of omalizumab. RESULTS: Action spectrum: UVB 44 %, UVA 70 %, UVA-1 89 %, green light 37 % and red light 22 %. Rush hardening with subsequent maintenance therapy was performed on 20 patients, 17 of whom were hereby adequately protected. In three further patients, omalizumab was additionally administered. CONCLUSIONS: Phototesting with UVB, UVB311nm, UVA, UVA-1, and visible light with innovative radiation sources is uniformly possible in every major skin clinic. With the help of the therapy step scheme the patients can be adjusted well. Rush hardening with UVA-1 is a safe method to help the patients during the sunny season. Omalizumab as the last therapy option is effective, but currently only possible in off-label use.

Lichturtikaria: Klinik, Diagnostik, Verlauf und Therapiemanagement bei 27 Patienten.

BACKGROUND AND OBJECTIVES: Solar urticaria is a rare photodermatosis, yet lacking standardization in diagnosis and therapy. The aim of this research was to use innovative radiation sources for diagnostics with defines and reproducible emission spectrum and doses. A uniform therapy step scheme was to be created. PATIENTS AND METHODS: In a longitudinal study, 27 patients were examined with solar urticaria over 13 years. With a characteristic anamnesis, the diagnosis was confirmed with phototesting from various radiation sources (UVB, UVB311nm, UVA, UVA1, green light, red light) and a therapy step scheme consisting of light protection, antihistamines, rush hardening with UVA-1 and the treatment with omalizumab. RESULTS: Action spectrum: UVB 44 %, UVA 70 %, UVA1 89 %, green light 37 % and red light 22 %. Rush hardening was performed on 20 patients, 17 of whom were adequately protected. In three further patients, omalizumab was additionally treated in off-label use. CONCLUSIONS: Photoprovocation with UVB, UVB311nm, UVA, UVA-1 and visible light with innovative radiation sources is uniformly possible in every major skin clinic. With the help of the therapy step scheme the patients can be adjusted well, a Rush Hardening with UVA-1 is a safe method to help the patients during the sunny season. Omalizumab as the last therapy option is effective, but currently only possible in off-label use.

Transient Pruritic Erythema as a Forme Fruste of Solar Urticaria.

BACKGROUND: Solar urticaria (SU) is a rare and disabling photodermatosis. SU typically manifests as urticarial wheals and erythema appearing shortly after sun exposure. SU is often initially diagnosed clinically with subsequent confirmation through photoprovocation tests. Early diagnosis is important for correct management of patients. OBJECTIVES: To present the clinical features of three cases of atypical presentation of SU and to discuss possible underlying mechanisms. METHODS: We report a series of three patients who presented with transient pruritic erythema without wheals after sun exposure. All patients had photoprovocation tests conducted to confirm SU diagnosis and to determine their action spectra. Treatment outcomes were recorded. RESULTS: All three patients developed classical manifestations of SU during photoprovocation tests within the UVA1 spectrum. Two patients required high-dose irradiation to provoke urticaria. CONCLUSIONS: Erythema without urticaria can be the primary manifestation of SU, especially in countries with sunny climates where natural skin hardening is common. Such cases require a high index of suspicion for SU and highlight the importance of photoprovocation testing to confirm the diagnosis.

Comparative Study of Frozen and Paraffin-Embedded Sections: Evaluation of Inflammatory Dermatoses.

BACKGROUND: Frozen section (FS) is often performed when histopathological evaluations are urgently required for implementation of therapeutic measures. In dermatology, this method is most commonly used to evaluate excision margins of tumors. FS are also routinely employed to differentiate toxic epidermal necrolysis from staphylococcal scalded skin syndrome. However, little is currently known about the performance of FS in the diagnosis of inflammatory dermatoses. OBJECTIVES: To compare histopathological diagnoses in a series of patients with a clinical diagnosis of an inflammatory dermatosis for which FS and paraffin-section (PS) specimens were obtained on the same day. METHODS: We conducted a single-center retrospective analysis of 43 cases. All histological slides were reviewed by a single dermato-pathologist. Concordance was calculated between FS and PS. RESULTS: Patients were divided into three groups according to diagnosis: papulosquamous diseases (group I), drug eruptions (group II), and a heterogeneous group (group III) that included cases of bullous vasculitis and Sweet syndrome. Among the three groups, the results of FS and of PS were discordant only in five cases (5/43, 11.6%). Compared to PS, FS had a sensitivity of 92.9% [95% confidence interval (95%CI) 64.17-99.63%] and a specificity of 100% in group I, sensitivity of 90.9% (95%CI 57.12-99.52%) and specificity of 100% in group II, and sensitivity of 83.33% (95%CI 60.78-94.16%) and specificity of 100% in group III. The degree of agreement between the results of the FS and of the PS was almost perfect (kappa = 0.95, 0.93 and 0.85 respectively). CONCLUSIONS: This study suggests that FS is a valid approach for the rapid diagnosis of inflammatory dermatoses. This method is as specific as PS, although it is less sensitive.