Consensus statement on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxoedema and scleroedema.

The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present consensus provides an update to the 2017 European Dermatology Forum Guidelines, focusing on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, updated strategies for the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 2 of this consensus provides clinicians with an overview of the diagnosis and treatment of scleromyxoedema and scleroedema (of Buschke).

Benign cephalic histiocytosis in a 2-year-old boy with an inconspicuous clinical presentation at onset.

We report on a 2-year-old boy whose initially inconspicuous skin lesions later on evolved into a typical clinical presentation of benign cephalic histiocytosis (BCH). The diagnosis of BCH can often be made on clinical grounds without the need for an extensive diagnostic work-up. Given the benign and self-limited course of the disease treatment is not required and the clinical management can be limited to a watchful waiting approach.

Efficacy of two different methods of cold air analgesia for pain relief in PDT of actinic keratoses of the head region - a randomized controlled comparison study.

BACKGROUND: Photodynamic therapy (PDT) is an effective method for treating actinic keratosis (AK) with pain during illumination representing the major side effect. The efficacy of two different cooling methods for pain relief in PDT of AK in the head region was compared. METHODS: Randomized, assessor-blinded, half side comparison study in 20 patients with symmetrically distributed AK on the head. Conventional PDT was performed on both halves of the scalp or face by applying 20% aminolevulinic acid cream (ALA) and subsequent illumination with incoherent red light. During illumination one side was cooled with a cold air blower (CAB) and the other with a standard fan (FAN) in a randomized fashion. Pain and skin temperature were recorded during and after PDT. The phototoxic skin reaction was evaluated up to seven days after PDT. The clearance rate of AK was assessed at 3 and 6 months after PDT. RESULTS: Mean pain (VASmean), maximum pain intensity (VASmax) and the mean skin temperature during PDT were significantly lower with CAB as compared to FAN (VASmean: 2.7 ± 1.4 vs. 3.7 ± 2.1, p = 0.003; VASmax: 3.8 ± 2.0 vs. 4.8 ± 2.5, p = 0.002; 26.8 ± 2.0 °C vs. 32.1 ± 1.7 °C; p=<0.001). The severity of the phototoxic skin reaction and the clearance rate of AK did not differ between the two cooling methods. CONCLUSION: Cooling with CAB during PDT has a greater analgesic effect than cooling with FAN. Patients with a lower skin temperature during illumination tended to experience less pain, however, this effect did not reach the level of statistical significance.

S1 Guideline: Diagnosis and therapy of vitiligo.

Vitiligo is a common skin disorder characterized by immune-mediated destruction of melanocytes. Non-segmental vitiligo, the most common clinical subtype, has usually a chronic course and often results in significant psychosocial consequences for the affected patient. Early recognition, awareness of comorbidity, precise assessment of disease extent and activity, evaluation of impairment of quality of life as well as rapid initiation of treatment based on currently available evidence-based therapies are crucial cornerstones in the management of vitiligo. This S1 guideline helps German dermatologists to better diagnose and treat vitiligo.

Effectiveness of narrowband UVB phototherapy and psoralen plus UVA photochemotherapy in the treatment of generalized lichen planus: Results from a large retrospective analysis and an update of the literature.

BACKGROUND: The aim of this retrospective study was to compare the efficacy and safety of different phototherapeutic modalities in the treatment of cutaneous lichen planus (LP). METHODS: We retrospectively analyzed the chart data of 53 patients with generalized LP who had been subjected to narrowband UVB (NB-UVB) or photochemotherapy (PUVA) between January 1997 and April 2020. Of these, 30 patients had received NB-UVB, 18 patients oral PUVA and 5 patients bath PUVA. RESULTS: Fifty patients completed a full treatment course. The percentage of patients with a complete (>90% clearing) or good (51%-90% clearing) response was similar for NB-UVB versus PUVA (86.2% vs. 90.5%; P = 1.00). The number of exposures required for obtaining a complete or good response was also comparable for both treatment groups (NB-UVB: 28.9 ± 12.3 vs. PUVA: 25.4 ± 10.1; P = .209). Adverse events, in particular gastrointestinal upsets, were recorded in 26.1% of patients treated with oral PUVA while none were observed with NB-UVB. CONCLUSION: The therapeutic outcome and the number of treatments required for achieving a complete or good response were comparable for NB-UVB and PUVA; however, PUVA therapy was associated with a substantially higher rate of moderate adverse events.

Effectiveness and clinical predictors of drug survival in psoriasis patients receiving apremilast: A registry analysis.

BACKGROUND: Little is known about the effectiveness and drug survival associated with apremilast under real-world conditions. OBJECTIVE: To investigate the influence of patient and disease characteristics on drug survival associated with apremilast and to elucidate clinical effectiveness with regard to the psoriasis area and severity index (PASI) reduction. METHODS: This was an observational, retrospective, multicenter analysis from the Austrian Psoriasis Registry. RESULTS: Data from 367 patients were eligible for analysis. The 12-month drug survival rate associated with apremilast (ie, the proportion of patients on the drug) was 57.3% and decreased significantly in patients younger than 40 years (relative hazard ratio = 1.49, P = .007918). Sex; concomitant arthritis; previous biologic therapy; obesity; and palmoplantar, scalp, nail, and intertriginous involvement did not significantly affect drug survival. At 12 months, the response rates in patients receiving apremilast per protocol with a PASI of 50, 75, 90, and 100 were 80.0%, 56.4%, 38.2%, and 22.7%, respectively. LIMITATIONS: Inclusion of a substantial number of patients with no record of absolute PASI at study entry and lack of PASI reduction follow-up data of 103 patients (28.1%) after starting apremilast treatment. CONCLUSION: Apremilast is a robust antipsoriatic drug for which the drug survival is not strongly influenced by most patient- or disease-related factors except age. Drug survival is significantly shorter in patients younger than 40 years.

[Eosinophilic annular erythema in a 20-month-old girl]. / Eosinophiles anuläres Erythem bei einem 20 Monate alten Mädchen.

We report on a 20-month-old girl with urticarial and partially annular skin lesions that were disseminated over the whole integument. The lesions persisted over 1 week and then gradually faded and reappeared on new body sites. The histological examination of a skin biopsy revealed an urticarial inflammation pattern with interstitial edema and a diffuse infiltration with many eosinophilic granulocytes without flame figures, neutrophils and lymphocytes. Laboratory investigations were inconspicuous and there was no eosinophilia. A diagnosis of eosinophilic annular erythema (EAE) of childhood was made which is a benign self-limiting skin disorder belonging to the group of eosinophilic dermatoses.

Coxsackie B3 Virus-induced Acute Hemorrhagic Edema of Infancy.

Acute hemorrhagic edema of infancy (AHEI) is a cutaneous leukocytoclastic small vessel vasculitis of unknown incidence. It affects mostly infants aged 4 to 24 months. The distinctive features of AHEI include a generally healthy-appearing child with low-grade or absent fever and rarely painful targetoid purpuric edematous lesions. The disease usually resolves spontaneously within 3 weeks without late sequelae. The main differential diagnosis of AHEI is Henoch-Schönlein purpura (HSP). Initially, purpura fulminans should also be ruled out. We report the case of a 5-year-old girl with low fever and rapidly progressive skin lesions who had been admitted to the pediatric clinic. The child presented with palpable annular targetoid and purpuric plaques of different size predominantly affecting the face and extremities. In addition, there was a painful, hemorrhagic edema on the dorsum of her hands and feet. Based on the course of the disease and the typical clinical presentation, i.e., extensive characteristic skin lesions in a young child in a good general health condition, a diagnosis of AHEI was established. A virus serology test showed increased titers of enterovirus and coxsackievirus. Isolation of virus from feces confirmed an infection with coxsackie B3 virus. To our knowledge, this is the first report linking coxsackie B3 virus infection to AHEI.

The impact of occlusive vs non-occlusive application of 5-aminolevulinic acid (BF-200 ALA) on the efficacy and tolerability of photodynamic therapy for actinic keratosis on the scalp and face: A prospective within-patient comparison trial.

BACKGROUND: Photodynamic therapy (PDT) is an effective treatment for actinic keratoses (AK). PDT is usually performed with occlusion of the photosensitizer prior to subsequent illumination. OBJECTIVES: This study aimed to compare the efficacy and tolerability of occlusive versus non-occlusive application of a 5-aminolevulinic gel (BT-200 ALA) for PDT of multiple AK on the scalp or face. METHODS: Prospective, investigator-blinded, within-patient comparison study on 45 patients. PDT with occlusion of ALA was performed in a target area on one randomized side of the scalp or face. One week later a contralateral target area received the same treatment except that no occlusion of the ALA gel was performed. 3 and 6 months after PDT, the clearance rate of a predetermined target lesion and the total clearance rate of all AK within the treated areas were determined. PDT-induced pain and skin phototoxicity and cosmetic outcome were also recorded. RESULTS: Clearance rate of the target AK and total AK clearance rate at 3 months after PDT was 88.4% and 90.6% for occlusive PDT and 58.1% (P = .001) and 70.4% (P = .04) for non-occlusive PDT. The corresponding values at 6 months after PDT were 69.7% and 72.1% for occlusive PDT and 30.2% (P < .001) and 35.6% (P = .001) for non-occlusive PDT. Pain score and skin phototoxicity were significantly higher after occlusive ALA application. No difference was observed with respect to cosmetic outcome. CONCLUSIONS: Occlusive application of ALA significantly improves the efficacy of PDT but is associated with more pain and increased phototoxicity.

Association of Clinical and Demographic Factors With the Severity of Palmoplantar Pustulosis.

Importance: Although palmoplantar pustulosis (PPP) can significantly impact quality of life, the factors underlying disease severity have not been studied. Objective: To examine the factors associated with PPP severity. Design, Setting, and Participants: An observational, cross-sectional study of 2 cohorts was conducted. A UK data set including 203 patients was obtained through the Anakinra in Pustular Psoriasis, Response in a Controlled Trial (2016-2019) and its sister research study Pustular Psoriasis, Elucidating Underlying Mechanisms (2016-2020). A Northern European cohort including 193 patients was independently ascertained by the European Rare and Severe Psoriasis Expert Network (2014-2017). Patients had been recruited in secondary or tertiary dermatology referral centers. All patients were of European descent. The PPP diagnosis was established by dermatologists, based on clinical examination and/or published consensus criteria. The present study was conducted from October 1, 2014, to March 15, 2020. Main Outcomes and Measures: Demographic characteristics, comorbidities, smoking status, Palmoplantar Pustulosis Psoriasis Area Severity Index (PPPASI), measuring severity from 0 (no sign of disease) to 72 (very severe disease), or Physician Global Assessment (PGA), measuring severity as 0 (clear), 1 (almost clear), 2 (mild), 3 (moderate), and 4 (severe). Results: Among the 203 UK patients (43 men [21%], 160 women [79%]; median age at onset, 48 [interquartile range (IQR), 38-59] years), the PPPASI was inversely correlated with age of onset (r = -0.18, P = .01). Similarly, in the 159 Northern European patients who were eligible for inclusion in this analysis (25 men [16%], 134 women [84%]; median age at onset, 45 [IQR, 34-53.3] years), the median age at onset was lower in individuals with a moderate to severe PGA score (41 years [IQR, 30.5-52 years]) compared with those with a clear to mild PGA score (46.5 years [IQR, 35-55 years]) (P = .04). In the UK sample, the median PPPASI score was higher in women (9.6 [IQR, 3.0-16.2]) vs men (4.0 [IQR, 1.0-11.7]) (P = .01). Likewise, moderate to severe PPP was more prevalent among Northern European women (57 of 134 [43%]) compared with men (5 of 25 [20%]) (P = .03). In the UK cohort, the median PPPASI score was increased in current smokers (10.7 [IQR, 4.2-17.5]) compared with former smokers (7 [IQR, 2.0-14.4]) and nonsmokers (2.2 [IQR, 1-6]) (P = .003). Comparable differences were observed in the Northern European data set, as the prevalence of moderate to severe PPP was higher in former and current smokers (51 of 130 [39%]) compared with nonsmokers (6 of 24 [25%]) (P = .14). Conclusions and Relevance: The findings of this study suggest that PPP severity is associated with early-onset disease, female sex, and smoking status. Thus, smoking cessation intervention might be beneficial.

Quality of Life, Anxiety, and Depression in Patients With Early-Stage Mycosis Fungoides and the Effect of Oral Psoralen Plus UV-A (PUVA) Photochemotherapy on it.

Background: Little is known about psychological discomfort and quality of life (QoL) in early stage mycosis fungoides (MF) and the effect of psoralen plus UV-A (PUVA) on it. Objective: To evaluate QoL, anxiety, and depression with validated instruments in early stage MF patients and whether PUVA treatment improves it. Methods: Patients with stage IA to IIA MF were treated with PUVA twice weekly for 12-24 weeks, followed by maintenance treatment or not, in a prospective randomized clinical trial. Patients completed a questionnaire on DLQI as well as the Hospital Anxiety and Depression Scale (HADS) prior to therapy, after their last PUVA exposure, and after the PUVA maintenance or observance phase. Results: For 24 patients with early stage MF, completed questionnaires were available and analyzed. Prior to treatment, 17% reported strong (DLQI > 10) and 29% moderate impairment (DLQI 6-10) in QoL; 33% of patients reported HADS scores indicating anxiety, and 21% reported scores indicating depression. PUVA significantly improved overall QoL by reducing mean DLQI scores by 58.6% (p = 0.003), HADS-A by 30% (p = 0.045), and HADS-D by 44% (p = 0.002). Improvements in QoL and psychological well-being seemed to be sustained, irrespective of maintenance treatment or not. Limitations: Small sample size. Conclusions: PUVA sustainably improves QoL and psychological well-being in patients with early stage MF. Clinical trial registration: ClinicalTrials.gov identifier: NCT01686594.

Apremilast for extensive and treatment-resistant alopecia areata: a retrospective analysis of five patients.

BACKGROUND: Recent experimental data suggest a role for apremilast in the treatment of alopecia areata. Small clinical studies have so far provided contradictory results. OBJECTIVES: We retrospectively evaluated the efficacy and safety of apremilast in five cases of extensive and treatment-resistant alopecia areata. MATERIALS AND METHODS: Apremilast was given at a dose of 30 mg, twice daily, over six months. The efficacy of apremilast treatment was determined by monthly assessment of the affected scalp surface area using SALT scoring. RESULTS: In four out of five patients, no sustained improvement in SALT score was observed within the treatment period. Two of these patients had a slight but only transient improvement after two months of treatment. One patient responded to apremilast treatment with a progressive and marked improvement, as reflected by an 83% reduction in SALT score. CONCLUSION: Our results are in line with the varying treatment responses in previous studies. Future studies on the efficacy of apremilast treatment in confirmed alopecia areata patient groups are warranted.