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.

Autosomal recessive cutis laxa type Ib-Successful redo aortic root and arch replacement.

We present an adolescent girl with a highly stenotic ascending aortic conduit of her former during infancy corrected giant aneurysm. Genetic testing determined autosomal recessive cutis laxa type-Ib as the underlying connective tissue disorder. Re-do valve sparing root and arch replacement gained excellent restoration of the aorta; 1-year-follow-up was uneventful.

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.

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.

The influence of acclimatization on stress hormone concentration in serum during heat stress.

This study was aimed to examine the influence of acclimatization on the change of concentration of stress hormones in men's serum exposed to heat stress during physical training. The study included a total of 40 men, aged 19-21 years, divided randomly into four groups: CTRL group: control, exposed to the Exercise Tolerance Testing in comfortable conditions; O group: exposed to Exercise Tolerance Testing in a warm environment; P group: exposed to passive acclimation to heat for 10 days, followed by Exercise Tolerance Testing in a warm environment; A group: exposed to active acclimation to heat for 10 days, followed by Exercise Tolerance Testing in a warm environment. All participants were tested for thermoregulation and acclimatization, skin and tympanic temperature, heart rate (HR), hormonal status and sweating. The mean skin temperature was the lowest in the control group of subjects exposed to physical exertion under comfortable conditions, and at each point of measurement it was statistically significantly different from that of the other study groups (p < 0.001). Sweating intensity was statistically significantly the lowest in the CTRL group (0.32 ± 0.04 l/m2/h; p < 0.001), compared to all other groups. Cortisol was significantly altered in O group (632.2 ± 92.3; 467.2 ± 89.7), testosterone levels were significantly altered in P (19.2 ± 9.3; 16.4 ± 7.3) and in A groups (22.1 ± 12.4; 14.9 ± 9.9), while prolactin was changed in O (392.1 ± 51.3; 181.4 ± 42.3), P (595.1 ± 191.1; 191.2 ± 52.5), and A group (407.4 ± 189.3; 173.4 ± 43.9) after the experimental period. The impact of acclimatization on hormonal indicators emphasizes its importance in the response of the endocrine system of soldiers to perform military activities in warm climates.

[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.

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.

De novo mutation of emopamil binding protein (EBP) gene in a girl with Conradi-Hünermann-Happle syndrome.

Conradi-Hünermann-Happle syndrome is a rare X-linked dominant syndrome affecting the skin, skeletal system, and eyes. Here, we report on a female patient with a de novo heterozygous missense mutation c.301C>T (p.Trp101Arg) of the EMP (emopamil binding protein) gene.