Clinical characteristics of pediatric hidradenitis suppurativa: a cross-sectional multicenter study of 140 patients.

Hidradenitis suppurativa (HS) rarely affects pediatric patients. The literature on pediatric HS patients is scarce. This is a cross-sectional study based on case note review or interviews and clinical examination of 140 pediatric patients undergoing secondary or tertiary level care. Patients were predominantly female (75.5%, n = 105) with a median age of 16. 39% reported 1st-degree relative with HS. Median BMI percentile was 88, and 11% were smokers (n = 15). Median modified Sartorius score was 8.5. Notable comorbidities found were acne (32.8%, n = 45), hirsutism (19.3%, n = 27), and pilonidal cysts (16.4%, n = 23). Resorcinol (n = 27) and clindamycin (n = 25) were the most frequently used topical treatments. Patients were treated with tetracycline (n = 32), or oral clindamycin and rifampicin in combination (n = 29). Surgical excision was performed in 18 patients, deroofing in five and incision in seven patients. Obesity seemed to be prominent in the pediatric population and correlated to parent BMI, suggesting a potential for preventive measures for the family. Disease management appeared to be similar to that of adult HS, bearing in mind that the younger the patient, the milder the disease in majority of cases.

Hidradenitis Suppurativa in Sweden: A Registry-Based Cross-Sectional Study of 13,538 Patients.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic, inflammatory skin condition with nodules and fistula formation and scarring. It is a debilitating disease with a severe negative impact on quality of life. There is a need for increased knowledge about the social and lifestyle characteristics of patients with HS in general, and pregnant women in particular. OBJECTIVES: The aims of this study were to investigate and describe social characteristics and comorbidity in all HS patients in Sweden as well as to study the prevalence of lifestyle factors associated with negative impact on health and pregnancy in Swedish pregnant women with HS. METHODS: A registry-based cross-sectional study was performed by record linkage between Swedish registers covering the entire population. A cohort of 13,538 HS patients diagnosed with HS in specialised care during the years 2001-2014 and a subgroup of 1,368 HS patients who had undergone pregnancy during 2010-2015 were defined and described. Aggregated public data on the entire Swedish population and all pregnancies in 2014 were described for reference. RESULTS: The HS population had an average age of 44 years on December 31, 2014. The prevalence of HS was 0.14%. In comparison to the Swedish reference population the HS patients were more often women, unmarried (36 vs. 44% married), and had lower education (68 vs. 82% with an upper-secondary school degree or higher) and lower income (39 vs. 16% made SEK <100,000 a year). Comorbidity was 3% for inflammatory bowel disease and 8% for type 2 diabetes. The subgroup analysis showed high prevalence of overweight, obesity, and smoking in pregnant women with HS. CONCLUSIONS: The results from this comprehensive characterisation of Swedish HS patients may be used to improve preventive measures, information, and care for this vulnerable group.

[Cutaneous mastocytosis - update and clinical guidelines]. / Mastocytos i huden ­ uppdatering av kunskapsläget och riktlinjer för handläggning.

Mastocytosis is a rare and multifaceted disease group characterized by mast cell accumulation in the skin and/or internal organs. In its most common form solitary or widespread, often itchy, red-brown skin lesions appear in childhood or during adulthood (cutaneous mastocytosis). The skin lesions are not always easy to recognize by medical professionals; hence, a correct diagnosis is often delayed. In children, the lesions tend to resolve before puberty, whereas most post-adolescent patients experience a chronic course combined with extra-cutaneous mast cell infiltration (systemic mastocytosis). Therefore, adult patients with cutaneous mastocytosis should be examined for signs of systemic involvement. This article describes the symptoms and signs in cutaneous mastocytosis, and provides guidelines based on international consensus documents. In addition, a newly updated classification of different forms of cutaneous mastocytosis is given.

Photochemotherapy and Graft-versus-Leukemia Reaction in Acute Leukemia: Tumor Immunity and Survival Are Dependent on Timing of Photochemotherapy of the Skin.

BACKGROUND: Cure of acute leukemia after transplantation is mediated by the grafted cells. We investigated the graft-versus-leukemia effect (GVL) in patients with cutaneous acute graft-versus-host disease (GVHD) treated with photochemotherapy (psoralen and ultraviolet light type A). METHOD: Forty-seven patients with acute leukemia were followed 5,000 days after transplantation to assess survival and GVL by multivariate analysis. The primary predictor was time to treatment of cutaneous acute GVHD by photochemotherapy separated into treatment start during the first week of acute GVHD versus after the first week of acute GVHD. RESULTS: Photochemotherapy started after the first week of acute GVHD predicted GVL with a hazard ratio (HR) of 3.94 (95% confidence interval, CI, 1.67-9.33, p = 0.0018) and survival with preserved GVL with an HR of 2.63 (95% CI 1.30-5.32, p = 0.007). The effects on GVL and survival with preserved GVL were present regardless of whether the patients were transplanted in remission or relapse (p < 0.05). Chronic GVHD came earlier in the group that started photochemotherapy after 1 week of acute GHVD, but chronic GVHD did not increase the GVL. CONCLUSION: The timing of photochemotherapy after cutaneous acute GVHD may direct the GVL and predict long-term leukemia-free survival.

Photochemotherapy of Cutaneous Graft-versus-Host Disease May Reduce Concomitant Visceral Disease.

BACKGROUND: Photochemotherapy may be used to treat cutaneous graft-versus-host disease (GvHD). Animal models show that in the days after photochemotherapy and antigen provocation, cells with an antigen-specific suppressive phenotype are elicited in the lymphoid organs. In GvHD, host antigens are present not only in the skin treated by photochemotherapy but also in the visceral tissues. OBJECTIVE: The aim of this paper was to evaluate the effect on visceral acute GvHD (aGvHD) of photochemotherapy of the skin. METHODS: We retrospectively evaluated 33 patients with aGvHD of the skin, the liver, and/or the gastrointestinal tract treated with photochemotherapy for their aGvHD of the skin and did a long-term follow-up of 10 years on survival. RESULTS: The complete response (CR) to photochemotherapy was 39%, the complete and partial response was 64% and the 6-month survival was 64%. Total body irradiation (TBI) before hematopoietic stem cell transplantation predisposed for CR of aGvHD of the liver and the gastrointestinal tract (p = 0.045). In the TBI group, the accumulated dose (numbers of treatments) for CR of visceral aGvHD increased with the body surface area affected by disease, from 8 (min-max: 5-14) for skin disease stage 1 to 10.5 (6-33) for stage 2 and 13 (11-21) for stage 3 (p = 0.04). Skin disease stage 1 showed a trend to be associated with CR in visceral disease at 28, 56, and 100 days (p = 0.07). Overall CR in visceral disease predicted a better 10-year overall survival (p = 0.0036). Finally, after TBI aGvHD of the gastrointestinal tract without anti-thymocyte globulin (ATG), clearance of T cells and dendritic cells responded better than aGvHD of the liver and aGvHD of the gastrointestinal tract with ATG (p = 0.01). CONCLUSION: Photochemotherapy after ionizing irradiation regulates the cell-mediated immunity in the viscera, and the systemic efficacy increases when the skin itself is less affected by disease. ATG modulates the regulatory effect of the gastrointestinal tract.

Hidradenitis suppurativa: a common and burdensome, yet under-recognised, inflammatory skin disease.

Hidradenitis suppurativa (HS) is a chronic, relapsing, inflammatory skin condition that typically occurs after puberty. The primary clinical presentation is painful inflamed nodules or boils in the apocrine gland-bearing regions (armpits, genital area, groin, breasts and buttocks/anus) that progress to abscesses, sinus tracts and scarring. Severity is typically described according to three Hurley categories, with most patients having mild or moderate disease. Estimated prevalence is 1-4% worldwide and HS is three times more common in women than men. Patients' disease burden includes intense pain, work disability and overall poor quality of life. Although the clinical signs of the disease can often be hidden by clothing, active HS is associated with a malodorous discharge that contributes to the disabling social stigma. Risk factors include smoking and obesity. Comorbidities include inflammatory bowel disease and spondyloarthropathies. The presentation of the disease is distinct, yet HS is not well-recognised except in dermatology clinics.

Topical methyl aminolaevulinate photodynamic therapy versus cryotherapy for superficial basal cell carcinoma: a 5 year randomized trial.

This multicentre, randomized study compared photodynamic therapy using topical methyl aminolaevulinate (MAL PDT), a non-invasive modality, with cryotherapy for treatment of superficial basal cell carcinoma. Sixty patients with 114 lesions were treated with MAL cream (160 mg/g) applied for 3 hours before illumination (570-670 nm, light dose 75 J/cm) (1 session), and 58 with 105 lesions received cryotherapy (2 freeze-thaw cycles). Patients with an incomplete response at 3 months received 2 further MAL PDT sessions (n = 20) or repeat cryotherapy (n = 16). 100 lesions treated with MAL PDT and 93 lesions treated with cryotherapy were in complete response at 3 months after the last treatment and evaluable for recurrence over 5 years. There was no difference in 5-year recurrence rates with either treatment (20% with cryotherapy vs. 22% with MAL PDT, p = 0.86). However, more patients had an excellent cosmetic outcome with MAL PDT (60% vs. 16% with cryotherapy, p = 0.00078). These results provide support for the use of MAL PDT as a non-invasive, selective treatment alternative for primary superficial basal cell carcinoma.

Bacteraemia in patients with hidradenitis suppurativa undergoing carbon dioxide laser surgery: detection and quantification of bacteria by lysis-filtration.

BACKGROUND: Hidradenitis suppurativa (HS) is a cicatrising and persistent disease of apocrine gland-bearing areas in adults. The severity of this condition varies from a few suppurating lesions to widespread, disabling disease. The aetiology is obscure, but suggested contributory factors include a genetic predisposition, comedones occluding the pilosebaceous apparatus, bacterial infections, and hormonal factors. Treatment consists mainly of surgery, while medical therapies serve principally as adjunct therapy. OBJECTIVES: The aim of the study was to determine the number and type of bacteria circulating in the bloodstream in patients with HS undergoing surgical treatment with a carbon dioxide laser stripping-secondary intention technique. METHODS: Twenty-one patients (20 females and 1 male, mean age 36, range 20-55 years) were included in the study. One blood sample (8.3 ml) was taken before surgery, one during the operation and the last one 10 min after surgery. Five healthy persons (all females, mean age 36, range 23-48 years) not undergoing any operation were used as the controls. The blood was cultured by a lysis-filtration technique which had been shown to be very sensitive. Since the filter catches the microorganisms and colonies are formed during culturing, the number of bacteria in the samples is easily determined. RESULTS: In 6 patients, all samples were negative, which indicates that the method of surgery itself caused no spread of bacteria from the lesions. Bacterial growth in the first blood sample was found in 9 patients, from the second sample in 10 and from the third one in 6. In 1 patient, bacteria were detected in three samples. At least 12 bacterial species were identified. The dominating bacteria were coagulase-negative staphylococci of which most were subtyped as Staphylococcus warneri. Among the anaerobic microorganisms, Propionibacterium acnes and P.granulosum were the most frequently isolated bacteria. The bacterial findings in the blood samples accord well with the results from a previous study in which cultures were taken from the deep parts of the HS lesions. In the 5 controls, no microbial growth was detected. CONCLUSION: The carbon dioxide laser stripping technique caused no additional spread of bacteria into the bloodstream. The evaluation of cultures containing microorganisms from normal skin flora is controversial. Since the bacteria encountered in this study are in close agreement with the findings in cultures from the deeper parts of HS lesions they seem to be relevant. The growth of bacteria in the first blood sample taken before surgery may indicate that some of these patients have bacteria continuously circulating in their blood.

Increased nerve growth factor and its receptors in atopic dermatitis: an immunohistochemical study.

Evidence suggests that neurotrophins may regulate certain immune functions and inflammation. In the present study, the localization and distribution of nerve growth factor (NGF) and its receptors were explored using immunohistochemical methods, with the aim of detecting the cause of the neurohyperplasia in early lesions of atopic dermatitis (AD). In AD involved skin, strong NGF-immunoreactive (IR) cells were observed in the epidermis. In some cases, a huge number of infiltrating cells with stronger NGF immunoreactivity was seen mainly in the dermal papillae. Some trkA immunoreactivity was observed in the outer membrane of cells in the basal and spinal layers of the epidermis. In the papillary dermis, a larger number of cells demonstrated strong trkA immunoreactivity. The p75 NGFr-IR nerve fibre profiles were increased (900 per mm(2); p<0.001) compared to normal [the involved skin also differed from the uninvolved skin (p<0.05)] in the dermal papillae. These nerve fibres were larger, coarser and branched, some of them terminated at p75 NGFr-IR basal cells, and also revealed a stronger fluorescence staining than the controls or the uninvolved skin. In normal healthy volunteers and AD uninvolved skin, the NGF immunoreactivity was weak in the basal layer of epidermis. Only a few trkA positive cells were seen in the basal layer of the epidermis and upper dermis. The IR epidermal basal cells revealed a striking patchy arrangement with strong p75 NGFr immunostaining in the peripheral part of the cells, and short and thick NGFr-IR nerve fibre profiles appeared as smooth endings scattered in the dermis including the cutaneous accessory organs. Using NGF and p75 NGFr double staining, both immunoreactivities showed a weak staining in the epidermis and dermis in normal and uninvolved skin. In the involved dermis of AD, the intensity of p75 NGFr-IR nerves was stronger in areas where there were also increased numbers of NGF-IR cells. These findings indicate that NGF and its receptors may contribute to the neurohyperplasia of AD.

Incidence of cancer among patients with atopic dermatitis.

OBJECTIVE: To assess the risk of skin cancer and other cancers among patients with atopic dermatitis. DESIGN: Register-based retrospective cohort study. SETTING: Sweden. Patients A total of 15 666 hospitalized patients identified in the national Inpatient Register as having discharge diagnoses of atopic dermatitis between January 1, 1965, and December 31, 1999. Interventions The National Swedish Cancer Register coded malignant neoplasms during the entire period of study. Follow-up time was calculated from the date of entry in the cohort until the occurrence of a first cancer diagnosis, emigration, death, or the end of the observation period, whichever occurred first. MAIN OUTCOME MEASURES: Follow-up by means of record linkages to several nationwide registers, among them the National Swedish Cancer Register. Standardized incidence ratios (SIRs) (the ratios of numbers of observed patients with cancer to expected numbers of incident cases of cancer) estimated the risk of developing cancer relative to the risks in the age-, sex-, and calendar year- matched general Swedish population. RESULTS: After excluding the first year of follow-up, the risk of developing any cancer was increased by 13% (95% confidence interval [CI] of SIR, 1.01-1.25, based on 311 observed patients with cancer). Excess risks were observed for cancers of the esophagus (SIR, 3.5; 95% CI, 1.3-7.7; 6 patients), pancreas (SIR, 1.9; 95% CI, 1.0-3.4; 11 patients), brain (SIR, 1.6; 95% CI, 1.1-2.4; 27 patients), and lung (SIR, 2.0; 95% CI, 1.3-2.8; 31 patients) and for lymphoma (SIR, 2.0; 95% CI, 1.4-2.9; 29 patients). There was a nonsignificant 50% excess risk for nonmelanoma skin cancer (SIR, 1.5; 95% CI, 0.8-2.6; 12 patients), seemingly confined to men and to the first 10 years of follow-up. Malignant melanoma did not occur more often than expected. CONCLUSIONS: The observed risk elevations, all of borderline statistical significance, should be interpreted cautiously. We could not control for possible confounding by cases of cancer caused by smoking, and the combination of multiple significance testing and few observed patients may have generated chance findings.

Non-invasive preoperative assessment of basal cell carcinoma of nodular and superficial types.

BACKGROUND/AIMS: Although various biophysical properties can be used to distinguish basal cell carcinoma (BCC) tissue from normal skin, none permits typing of the tumour. In this study, we assessed nodular (NBCC) and superficial (SBCC) types of BCC using three different non-invasive instruments and placed special emphasis on their clinical value as diagnostic tools. METHODS: We included 35 patients with 35 tumours (15 NBCC and 20 SBCC), which had been diagnosed clinically. All lesions were evaluated preoperatively with an instrument measuring electrical impedance (IMP). Methods for determining transepidermal water loss (TEWL) and laser Doppler (LD) were also used. Measurements were also made in healthy skin on the contralateral side as reference. The diagnosis was confirmed by histological examination. RESULTS: We found clear differences between the lesions and their reference values, using all three bioengineering techniques for NBCC and SBCC. The biophysical parameters of all types vary with anatomical location. Since most of the NBCC were located on the face and most SBCC on the trunk, their baseline impedance characteristics (i.e., impedance indices magnitude index (MIX) and imaginary part index (IMIX)) differed significantly. We therefore compared delta (a difference between the reference and tumour) MIX and IMIX of NBCC and SBCC instead of the absolute figures. We found no significant differences in TEWL, blood flow and IMP between the two types of BCC and attribute this to biological variation and electromagnetic noise. CONCLUSIONS: As with LD and TEWL, definite differences in IMP were detected between healthy skin and BCC lesions. However, at this stage of development of the bioimpedance technique, we were unable to distinguish between the two types of BCC. An improved IMP device with semi- invasive probes or a more sophisticated type of data analysis may increase the diagnostic usefulness of the IMP method.

Effects of pretreatment with a urea-containing emollient on nickel allergic skin reactions.

The aim of this study was to evaluate the effect of a moisturizer containing urea on allergic contact dermatitis. Twenty-five nickel-sensitized patients and five controls (non-sensitized volunteers) applied such a moisturizer on the volar side of one forearm twice daily for 20 days, while the other forearm served as the control. After treatment with the moisturizer, patch tests with 0%, 0.5% and 2% NiSO4 in petrolatum were applied in a randomized manner on each arm. After 72 h, the skin reactions were blindly evaluated by clinical scoring and by measuring transepidermal water loss and electrical impedance. After treatment, the baseline transepidermal water loss values were lower and the baseline magnitude impedance index values were higher on the pretreated forearm. According to clinical scoring and measurements with the two physical measurement techniques, the degree of the patch test reactions was equal. All control subjects had negative nickel tests. We concluded that the skin reactivity to nickel in nickel-sensitized patients is not significantly affected by use of the urea-containing moisturizer.

Scanner-assisted carbon dioxide laser surgery: a retrospective follow-up study of patients with hidradenitis suppurativa.

BACKGROUND: The chronic fistulating lesions of hidradenitis suppurativa spread by contiguous growth, and all affected tissue needs to be surgically removed. OBJECTIVE: The aim of our study was to evaluate a surgical method for treatment of Hurley stage II hidradenitis suppurativa (HS), the carbon dioxide laser rapid-beam optomechanical scanner system in continuous mode. METHODS: Thirty-four patients were evaluated after treatment; 31 patients were women, and the mean age was 33.9 years (range, 15-55 years). All patients had had HS for a mean of 13.4 years (range 1-35 years) and more than 3 recurrences of suppurating lesions in the year before inclusion in the study. All lesions had been classified as Hurley stage II. The mean follow-up time after carbon dioxide laser surgery was 34.5 months (range, 7-87 months), and patients were later contacted by telephone about recurrences and the end results. RESULTS: The mean healing time was about 4 weeks (range, 3-5 weeks). During follow-up, 4 of the 34 patients had recurrences at the surgical site, that is, locoregional HS. Thirty had no recurrences in the treated area, but in 12 cases de novo suppurating lesions, separated from the initial surgical site by >5 cm, developed. Twenty-five patients had flares of HS lesion(s) in an area other than the treated site. Eight had no recurrences. CONCLUSION: Macroscopically controlled tissue-selective carbon dioxide laser treatment of HS is a fast, efficient treatment and well accepted by the patients.

Increased nerve growth factor- and tyrosine kinase A-like immunoreactivities in prurigo nodularis skin -- an exploration of the cause of neurohyperplasia.

Neurotrophins and their receptors play an important role in cutaneous nerve development and reconstruction after injury. Recent developments indicate that this group of molecules not only exert a neurotrophic action, but are also involved in immune responses and inflammation. Prurigo nodularis is a skin disease characterized by neurohyperplasia and intense itch. In the present study, the localization and distribution of nerve growth factor (NGF) and its receptors were explored by immunohistochemical methods, with the aim of detecting the cause of the neurohyperplasia in the disease. In normal healthy volunteers and in uninvolved skin, NGF immunoreactivity was seldom seen in the basal layer of the epidermis or in the dermis. In prurigo nodularis skin, there was also very little NGF immunoreactivity in the epidermis. However, in the dermis, a huge number of cells showed an NGF-like immunoreactivity. In normal skin of healthy volunteers, only a weak staining for tyrosine kinase A (trkA) was seen in the epidermis, whereas in the dermis, there was no trkA staining seen at all. However, in the prurigo nodularis tissue, the hyperplastic nerves clearly showed trkA immunoreactivity, and it seemed that the staining was only present in the axons. By NGF and p75 NGF receptor double-labelling, both immunoreactivities showed weak staining in the epidermis and dermis of normal skin. However, in the dermis of prurigo nodularis, strong staining for both NGF and NGF receptor antibodies was seen. NGF receptor-immunoreactive nerves were more dense in areas where there were more NGF-immunoreactive cells. The results indicate that in prurigo nodularis skin, NGF is overexpressed, locally infiltrated inflammatory cells may be the source of this NGF, and NGF and its receptors may contribute to the neurohyperplasia of the disease.