Outcome Measures for the Evaluation of Treatment Response in Hidradenitis Suppurativa for Clinical Practice: A HiSTORIC Consensus Statement.

Importance: Although several clinician- and patient-reported outcome measures have been developed for trials in hidradenitis suppurativa (HS), there is currently no consensus on which measures are best suited for use in clinical practice. Identifying validated and feasible measures applicable to the practice setting has the potential to optimize treatment strategies and generate generalizable evidence that may inform treatment guidelines. Objective: To establish consensus on a core set of clinician- and patient-reported outcome measures recommended for use in clinical practice and to establish the appropriate interval within which these measures should be applied. Evidence Review: Clinician- and patient-reported HS measures and studies describing their psychometric properties were identified through literature reviews. Identified measures comprised an item reduction survey and subsequent electronic Delphi (e-Delphi) consensus rounds. In each consensus round, a summary of outcome measure components and scoring methods was provided to participants. Experts were provided with feasibility characteristics of clinician measures to aid selection. Consensus was achieved if at least 67% of respondents agreed with use of a measure in clinical practice. Findings: Among HS experts, response rates for item reduction, e-Delphi round 1, and e-Delphi round 2 surveys were 76.4% (42 of 55), 90.5% (38 of 42), and 92.9% (39 of 42), respectively; among patient research partners (PRPs), response rates were 70.8% (17 of 24), 100% (17 of 17), and 82.4% (14 of 17), respectively. The majority of experts across rounds were practicing dermatologists with 18 to 19 years of clinical experience. In the final e-Delphi round, most PRPs were female (12 [85.7%] vs 2 males [11.8%]) and aged 30 to 49 years. In the final e-Delphi round, HS experts and PRPs agreed with the use of the HS Investigator Global Assessment (28 [71.8%]) and HS Quality of Life score (13 [92.9%]), respectively. The most expert-preferred assessment interval in which to apply these measures was 3 months (27 [69.2%]). Conclusions and Relevance: An international group of HS experts and PRPs achieved consensus on a core set of HS measures suitable for use in clinical practice. Consistent use of these measures may lead to more accurate assessments of HS disease activity and life outcomes, facilitating shared treatment decision-making in the practice setting.

Hidradenitis Suppurativa is Associated with Non-alcoholic Fatty Liver Disease: A Cross-sectional Study.

Non-alcoholic fatty liver disease (NAFLD) is currently the most common cause of end-stage liver disease. The aim of this controlled cross-sectional study was to assess the association between NAFLD and hidradenitis suppurativa. NAFLD was assessed using hepatic ultrasound. A total of 125 patients with hidradenitis suppurativa and 120 patients without hidradenitis suppurativa were recruited, matched for age, sex and body mass index (< 25 or ≥ 25 kg/m2), a risk factor related to NAFLD. Both groups presented similar proportions of overweight or obesity (89.6% vs 90%). Patients with hidradenitis suppurativa presented significantly higher prevalence of NAFLD compared with those with non- hidradenitis suppurativa (57.6% vs 31.7%, p < 0.001). Multivariable analysis confirmed independent association between hidradenitis suppurativa and NAFLD (odds ratio 2.79, 95% confidence interval 1.48-5.25; p = 0.001) besides age, body mass index, hypertension and hypertransaminasaemia. Hidradenitis suppurativa is significantly associated with the development of NALFD regardless of the presence of classic metabolic risk factors.

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.

Mast cells in hidradenitis suppurativa: a clinicopathological study.

Mast cells (MC) have been observed in hidradenitis suppurativa (HS) lesions. Their potential role in the pathogenesis of HS is unknown. The aim of this study was to assess the number and distribution MC in HS lesions, and its association with disease and itch severity. We studied biopsies from HS-lesions and perilesional skin from 34 HS patients. The samples were stained with CD117 and toluidine blue, and the number of MC determined semi-quantitatively (40 × magnification). The distribution of MC was also noted. The clinical features of the disease were extracted from patients' case records and a questionnaire-based database. MC were present to a greater degree in HS-lesions than in perilesional skin (P = 0.004). Disease severity (Sartorius score) was correlated to with MC count and itch when adjusted for sex and age (P = 0.042). Duration of the disease could not been significantly correlated with MC count. A positive correlation between MC count and HS activity was detected, suggesting a potential link between MC and HS.

Cutaneous Metastases From Malignant Mesothelioma of the Tunica Vaginalis Testis.

Mesotheliomas are uncommon tumors arising from mesothelial cells lining the serous membranes of the pleura, pericardium, peritoneum, and tunica vaginalis testis. Less than 100 cases arising from the tunica vaginalis testis have been published and, to our knowledge, only 5 cases of cutaneous involvement from these tumors have been reported. We report an additional case with fatal outcome. A 93-year-old man presented with multiple polypoid nodules on the left scrotum. Ulceration was also present, and a firm 5-cm palpable testicular mass was also found. The patient had been exposed to asbestos for 40 years. Histologic examination of a skin biopsy from one of the nodules showed diffuse dermal infiltration of markedly atypical cuboidal cells, with polymorphous and hyperchromatic nuclei. Mitotic figures were common. These cuboidal cells lined clefts, forming a tubular and micropapillary pattern throughout papillary and reticular dermis. Immunohistochemical study showed strong nuclear and cytoplasmic positivity for calretinin, epithelial membrane antigen (cytoplasmic), and cytokeratin-7 (cytoplasmic) and nuclear positivity for Wilms tumor-1. These findings were consistent with cutaneous infiltration from malignant mesothelioma of the tunica vaginalis testis. Treatment of this rare tumor remains challenging because there are currently no recommended guidelines, but radical inguinal orchiectomy is an optimal choice.

Use of the Barthel index, activities of daily living, in dermatologic surgery in patients aged 80 years and older.

BACKGROUND: Dermatologists often attend elderly patients with non-melanoma skin cancer (NMSC). There is a lack of information regarding the optimum treatment for elderly patients with NMSC. METHODS: The objective of this study was to describe changes in the Barthel Index (BI) after dermatologic surgery for NMSC in patients aged 80 years and older. A prospective observational study was carried out in patients aged 80 years and older diagnosed with NMSC and treated with conventional dermatologic surgery. BI was performed by direct interview with the patients and/or their caregiver before the surgery, seven and 30 days after the surgery. RESULTS: A total of 180 dermatologic surgeries were performed in 144 patients. The average age of the patients was 84.2 years. There were 84 men (58.3%) and 60 women (41.7%). Mean BI score was 85.9 at baseline, 85.3 on day 7 after surgery, and 85.6 on day 30, showing minimal changes after dermatologic surgery. Changes in the BI occurred mainly in dressing, toilet use, transfers, mobility (on level surfaces) and walking stairs. CONCLUSIONS: Activities of daily living were not significantly affected in patients aged 80 years and older, after dermatologic surgery for NMSC.

Extramammary Paget disease successfully treated with topical imiquimod 5% and tazarotene.

Extramammary Paget disease (EMPD) is an intraepithelial adenocarcinoma usually localized in areas rich in apocrine sweat glands. Surgery remains the treatment of choice for EMPD. However, several nonsurgical treatments have been also described. Around 40 cases of EMPD treated with imiquimod 5% have been published; of these, only six correspond to nonresponses. We describe a recurrent vulvar EMPD with failure to respond to topical imiquimod 5% in monotherapy but a favorable response to its association with tazarotene.

Acrodermatitis enteropathica-like dermatosis associated with ornithine transcarbamylase deficiency.

The urea cycle is the major metabolic pathway for excretion of waste nitrogen. Ornithine transcarbamylase deficiency is the most frequent urea cycle disorder. It is a hereditary-X-linked disease with over 150 mutations described. Ornithine transcarbamylase deficiency causes vomiting, lethargy, hyperventilation, and even death, mainly in the neonatal period. Ammonia, an extremely toxic molecule for the organism, is generated during protein catabolism and is accumulated in patients with this deficiency. Part of the treatment consists of a low-protein diet, to avoid hyperammonemia episodes, which can even have a fatal outcome. Patients can become deficient in several amino acids, either through the low-protein diet or directly through the primary enzyme deficiency; this in turn can cause an acrodermatitis enteropathica-like dermatosis.

Dermatomiositis amiopática asociada a recurrencia de cáncer de mama / Amyopathic dermatomyositis associated with a recurrence of breast cancer

El término dermatomiositis amiopática, o dermatomiositis sine miositis designa aquellos pacientes que presentan las manifestaciones cutáneas típicas de la dermatomiositis pero sin evidencia de miopatía inflamatoria. La dermatomiositis amiopática puede asociarse a neoplasia subyacente, al igual que sucede con la dermatomiositis clásica. Se presenta el caso de una paciente de 59 años, con hallazgos cutáneos característicos de dermatomiositis, sin debilidad muscular proximal y con enzimas musculares séricas normales, que se mantuvieron en el rango de la normalidad a lo largo del seguimiento posterior, aunque el electromiograma realizado 6 meses más tarde mostró alteraciones con patrón miopático. Esta sintomatología cutánea permitió sospechar una neoplasia oculta y finalmente encontrar una recidiva de su cáncer de mama aparentemente inactivo desde hacía muchos años. La asociación de dermatomiositis amiopática con recurrencia de cáncer de mama es excepcional

The term «amyopathic dermatomyositis», or dermatomyositis «sine myositis» is used to describe those patients who present with the skin manifestations typical of dermatomyositis, but with no evidence of inflammatory myopathy. Amyopathic dermatomyositis may be associated with an underlying neoplasm, the same as with classic dermatomyositis. We present the case of a 59-year-old female patient, with cutaneous findings typical of dermatomyositis, with no proximal muscle weakness and with normal serum muscle enzymes, which stayed in a normal range throughout the later follow-up period, although the electromyogram performed six months later showed alterations with a myopathic pattern. These skin symptoms raised the suspicion of an occult neoplasm, and a recurrence of the patient's breast cancer, apparently inactive for many years, was finally found. The association of amyopathic dermatomyositis with a recurrence of breast cancer is exceptional