Penicillamine-Induced Localised Cutis Laxa in a Patient with Wilson Disease: A Case Report.

Introduction: Wilson disease is a rare genetic disorder, characterised by excessive deposition of copper in the liver, brain, and other tissues. Penicillamine, a copper-chelating agent, is used in high doses in the treatment of Wilson disease leading to a variety of cutaneous reactions, including hyper-sensitivity reactions, pseudoxanthoma elasticum, elastosis perforans serpiginosa, anetoderma, and cutis laxa (CL). We present a rare case of localised CL induced by penicillamine for Wilson disease, in the absence of elastosis perforans serpiginosa. Case Description: A 41-year-old male with Wilson disease treated with long-term high-dose penicillamine was referred to us for a basal cell carcinoma on the scalp. On physical examination, diffusely flaccid and redundant skin on the right side of the neck were observed. Histopathology revealed findings consistent with CL. Conclusion: Long-term treatment with penicillamine for Wilson disease may induce localized CL, possibly by direct inhibition of cross-linkage of collagen fibres.

Hair Dyes Sensitization and Cross-Reactions: Challenges and Solutions: A Systematic Review of Hair Dye Allergens' Prevalence.

Widespread use of oxidative hair dyes during the past decades has raised questions on the potential allergy reactions and their management, as well as prevention measures for both professionals and consumers. Allergic contact dermatitis can be elicited by various hair dye-related allergens, though the main problem remains with p-phenylenediamine and related aromatic amines. If allergy is suspected, patch testing identifies the responsible hapten. Individuals sensitized to specific permanent hair dyes substances should avoid the exposure to these chemicals, but also be aware of possible cross-sensitization to other similar compounds. Cross-reactions detected in patch-tested populations indicate that one cannot safely use alternatives, although cross-reactivity is not always clinically relevant. An open application hair dye allergy self-test is recommended by manufacturers for early detection of allergy predisposition in consumers, although the lack of standardized conditions makes the efficacy of this process doubtful. Appropriate use of hand gloves, especially nitrile, is the most efficient prevention measure for professional hand eczema. In this systematic review, we focus on cross-reactions among hair dye-related allergens and make an attempt to answer some, frequently encountered by physicians, questions, while presenting the prevalence of the hair dye-related allergens.

Case of Polyethylene Glycol Allergy Confirmed with Basophil Activation Test and Oral Challenge Successfully Immunized with SARS-CoV-2 Vaccine.

Polyethylene glycols (PEG) or macrogols are polymers of ethylene oxide widely used in drugs either as active substances or, more commonly, as excipients. We report a Caucasian 32-year-old woman with referred anaphylaxis almost instantly after oral intake of a macrogol-containing laxative. Despite an anaphylactic reaction, the patient showed negative results for both the skin test and specific IgE to monomer, while the basophil activation test and oral challenge were positive. The patient was later successfully vaccinated with a polysorbate 80-containing SARS-CoV-2 vaccine following an additional work-up. As a result, the inactive form of PEG cannot be fully diagnosed, and it is considered a "hidden" allergen. Allergens like polysorbates need special consideration due to their possible cross-reactivity by their specific derivatives.

Diagnosis and Differential Diagnosis of Poikiloderma of Civatte: A Dermoscopy Cohort Study.

INTRODUCTION: Poikiloderma of Civatte (PC) is a common, acquired, chronic, benign poikiloderma of the neck and face, most commonly affecting peri-menopausal females. At the time of writing, few studies have been published regarding the dermoscopy of PC. OBJECTIVE: To describe the dermoscopic picture of PC, so as to provide a clinico dermoscopic diagnosis and differential diagnosis for PC. METHODS: Twenty-eight patients with PC, aged 26-73 years, of whom 19 females (67.86%) were evaluated by detailed history, clinical examination, and dermoscopic examination with hand-held dermoscope. RESULTS: The reticular pattern was observed in 15 cases (53.6%); the white dot in 10 (35.7%); the non-specific in 9 (32.1%); and the combination of linear and dotted vessels in 8 (28.6%). Regarding local dermoscopic features, converging curved vessels were observed in 18 cases (64.3%); linear irregular vessels in 17 (60.7%); rhomboidal/polygonal vessels in 15 (53.6%); dotted/globular vessels in 10 (35.7%); white macules in 23 (82.1%); brown macules in 11 (39.3%); and whitish follicular plugs in 6 (21.4%). CONCLUSIONS: The dermoscopic picture of PC is highly characteristic and corresponds well to both clinical and histological findings. Dermoscopy may assist clinical diagnosis, as well as the differentiation from other dermatoses of the neck and face, especially poikilodermas with guarded prognosis.

Systemic Mastocytosis in a Patient with BCR-ABL1-Positive Chronic Myeloid Leukemia in the Remission Phase.

Systemic mastocytosis (SM) comprises a group of rare disorders resulting from tissue infiltration by pathological mast cells. In a percentage ranging from 5 to 40% in various patient series, SM appears to be associated with an accompanying hematologic neoplasm (SM-AHN). The coexistence of SM with chronic myelogenous leukemia (CML) is extremely rare with only 3 cases in the literature. The natural course of CML has changed dramatically over the past 2 decades with the use of tyrosine kinase inhibitors (TKIs). We report a case of diagnosing SM in a patient in complete molecular remission of CML after stopping TKI treatment.

Clinical and Epidemiological Characteristics of Hidradenitis Suppurativa Patients with Paradoxical Psoriasiform Reactions following Treatment with Adalimumab.

Introduction: Immunomodulation using TNF-α inhibitors (anti-TNF-a), especially adalimumab, is highly effective in the treatment of hidradenitis suppurativa (HS) in cases that are poorly controlled by conventional treatments. However, paradoxical psoriasis represents a peculiar type of psoriasis that may occur de novo or as the worsening of pre-existent psoriatic lesions during treatment with adalimumab. Case Presentation: We reported 4 cases of patients suffering from HS, who developed paradoxical psoriasis after treatment with adalimumab for their HS, analyzing their clinical and epidemiological characteristics. All 4 cases were middle aged, smokers, overweight or obese. Half of the patients were males (50%). All of them were classified as Hurley stage III, with a mean duration of HS of 20 years. Two patients had a family history of psoriasis. All 4 patients had been on at least 5 months of successful treatment with adalimumab before the onset of the lesions. Conclusions: Paradoxical psoriasis emerged in 4 patients who received at least 5-month regimen of adalimumab for long-lasting HS. Although different mechanisms have been hypothesized for such events, the exact underlying pathogenetic pathway remains unclear. Consistent reporting of such rare cases, and on a larger scale, is encouraged in order to enrich the available literature.

Occupational contact allergy of construction workers in Greece: Trends during 2009-2018.

BACKGROUND: Recent trends in the sensitization of construction workers show a decrease in potassium dichromate and an increase in epoxy resin sensitization. OBJECTIVES: To present the trends of occupational contact allergy of construction workers in Greece from 2009 to 2018. METHODS: We retrospectively reviewed the files of patients with eczema patch tested in our Contact Dermatitis Clinic who were construction workers. RESULTS: A total of 191 construction workers initially reported contact dermatitis. Of these, 138 had occupation-relevant allergic contact dermatitis (ACD) or irritant contact dermatitis (ICD). All patients were men. After being patch tested, 98 (71.0%) were diagnosed with ACD and 40 with ICD. Median duration of occupation till onset of ACD was 2 years (interquartile range [IQR] 0.8-7). The hands were the most common location for ACD (73.5%), followed by the trunk (39.8%), the legs (38.8%), and the face (11.2%). Of the patients, 74.6% had lesions affecting multiple body sites. Potassium dichromate (67%) was the most frequent allergen, followed by thiuram mix (37.4%) and cobalt chloride (31.8%). Sensitization to epoxy resins was lower (12.1%). CONCLUSION: The sensitization pattern of Greek construction workers does not follow the trends in Central or Northern Europe, rather sharing attributes with less industrialized countries.

Perifolliculitis Capitis Abscedens et Suffodiens Treated with Systemic Isotretinoin Monotherapy: Case Report and Review of Current Therapeutic Options.

Perifolliculitis capitis abscedens et suffodiens (PCAS) is a rare, suppurative dermatosis of the scalp, the etiology of which remains unknown. It is characterized by the development of comedones, perifollicular pustules, firm or fluctuant and itchy or painful nodules and abscesses of the scalp, connected by communicating sinuses that may lead to the formation of scarring and irreversible alopecia. Treatment of PCAS is challenging, often leading to unsatisfactory results. We present a case of a 23-year-old Caucasian man with PCAS who was treated successfully with systemic isotretinoin monotherapy and we review the current therapeutic options.

Perception and Knowledge of Hidradenitis Suppurativa in Greece: A Cross-Sectional Study of 1301 Individuals.

Background: Hidradenitis suppurutiva (HS) is a chronic auto-inflammatory disease with recurrent painful deep-seated nodules. Aims: The aim of this study was to qualitatively assess patients' perception for HS. Methods: A descriptive two-step questionnaire survey was conducted from January 2017 to December 2018. The survey was performed through self-assessed, online, standardized questionnaires. Clinico-epidemiological characteristics, medical history, comorbidities, personal perceptions and the impact of the disease on participants' professional and everyday life were recorded. Results: A total number of 1301 Greek people completed the questionnaire. Of them, 676 (52%) reported symptoms resembling HS, whereas 206 (16%) reported that have been officially diagnosed with HS. The mean age of the study group was 39.2 ± 11.3 years. More than half of the diagnosed patients (n = 110, 53.3%) reported that they developed their first symptoms between 12 and 25 years of age. Of the diagnosed patients (n = 206), the majority (n = 140, 68.0%) were females and active smokers (n = 124, 60.1%). Seventy-nine (n = 79, 38.3%) patients reported a positive family history for HS. Ninety-nine (n = 99, 48.1%) patients reported that HS has a negative effect on their social life, 95 (46.1%) on personal life, 115 (55.8%) on sexual life, 163 (79.1%) on their mental health and 128 (62.1%) on their overall quality of life. Conclusion: Our study showed that HS seems to be an underteated, time-consuming and cost-intensive disease.

Colchicine Improves Clinical Outcomes and Quality of Life in Hidradenitis Suppurativa Patients: A Retrospective Study.

Introduction: Hidradenitis suppurativa (HS) is a chronic inflammatory skin disorder of the follicular epithelium. The aim of the study was to investigate the effectiveness of colchicine on the clinical outcomes of HS patients, and to evaluate wither colchicine as monotherapy or in combination with doxycycline would provide better outcomes. Methods: A retrospective study was conducted including 44 patients with established HS, divided into three groups. The first group (n = 15 patients) received colchicine as monotherapy, the second group (n = 14 patients) received colchicine and doxycycline 100 mg/d, while the third group (n = 15 patients) received colchicine and doxycycline 40 mg/d. Disease severity during treatment was assessed at baseline and follow-up, using the Hurley Scoring System and the International Hidradenitis Suppurativa Severity Score System (IHS4). All patients were also asked to complete a Dermatology Life Quality Index (DLQI) questionnaire. These scores were compared among the study groups. Results: The DLQI and IHS4 scores significantly improved after treatment with colchicine (p < 0.001) in all groups. All colchicine regimes, including the single colchicine regime, colchicine plus doxycycline 100 mg regime, and colchicine plus doxycycline 40 mg regime, resulted in significant improvements in the DLQI and IHS4 scores (p < 0.001). Clinical improvement based on DLQI and IHS4 scores was similar in all groups. None of the patients had to discontinue the treatment due to adverse events. Discussion: In conclusion, our findings suggest that colchicine may improve clinical severity and quality of life in HS patients, either as monotherapy or in combination with doxycycline, both at antimicrobial (100 mg) and sub-antimicrobial (40 mg) doses.

The efficacy and tolerability of tetracyclines and clindamycin plus rifampicin for the treatment of hidradenitis suppurativa: Results of a prospective European cohort study.

BACKGROUND: Tetracyclines and clindamycin plus rifampicin combination therapy are both considered first-line therapy in current hidradenitis suppurativa guidelines. However, evidence for their efficacy is drawn from small studies, often without validated outcomes. OBJECTIVE: To assess the 12-week efficacy of oral tetracyclines and a combination of clindamycin and rifampicin. METHODS: A prospective, international cohort study performed between October 2018 and August 2019. RESULTS: In total, 63.6% of the included 283 patients received oral tetracyclines, and 36.4% were treated with clindamycin and rifampicin. Both groups showed a significant decrease in International Hidradenitis Suppurativa Severity Score System from baseline (both P < .001). The Hidradenitis Suppurativa Clinical Response (HiSCR) was achieved in 40.1% and 48.2% of patients, respectively (P = .26). Patient characteristics or disease severity were not associated with the attainment of HiSCR or the minimal clinically important differences for the Dermatology Life Quality Index and pain. LIMITATIONS: Cohort study. Respectively, 23.9% and 19.4% of patients had to be excluded from the HiSCR analysis for the tetracycline and combination therapy group because of a low abscess and nodule count at baseline. CONCLUSION: This study shows significant efficacy of both tetracycline treatment and clindamycin and rifampicin combination therapy after 12 weeks in patients with hidradenitis suppurativa. No significant differences in efficacy were observed between the 2 treatments, regardless of disease severity.

Comparative Study of Quality of Life and Psychological Aspects in Patients with Psoriasis and Hidradenitis Suppurativa.

Psoriasis and hidradenitis suppurativa are two common dermatological diseases that affect physical, social, and psychological aspects of the patients' lives. The aim of this study was to compare quality of life, depression, anxiety, self-esteem, and loneliness in patients with psoriasis and hidradenitis suppurativa. One hundred and eight patients with psoriasis, 113 patients with hidradenitis suppurativa and 116 healthy controls were included in the study. The quality of life, depression, anxiety, and loneliness of the patients as well as their self-esteem were assessed using the Dermatology Life Quality Index (DLQI), Hospital Anxiety and Depression Scale (HADS), the UCLA loneliness Scale (UCLA-Version 3) and the Rosenberg's Self-esteem Scale (RSES), respectively. Patients with psoriasis (12.77±4.43) reported a higher mean impairment in DLQI than patients with hidradenitis suppurativa (11.10±6.53, P=0.028), in the univariate comparisons. Patients with psoriasis presented statistically significantly higher levels of anxiety and depression than both patients with hidradenitis suppurativa and healthy controls, while patients with hidradenitis suppurativa also had higher anxiety and depression compared with healthy controls. Patients with psoriasis (46.31±6.36) reported statistically significantly higher loneliness than both patients with hidradenitis suppurativa (43.18±7.40) and controls (40.42±4.41), while the patients with hidradenitis suppurativa also presented higher loneliness in comparison with controls. Lower levels of self-esteem were found in patients with psoriasis (15.08±3.11) compared with both patients with hidradenitis suppurativa (18.89±1.69) and controls (20.25±2.60), while patients with hidradenitis suppurativa also had lower self-esteem than controls. Significant levels of depression, anxiety, and impaired quality of life were strongly associated with disease severity. When patients with mild disease were compared, those with psoriasis presented with both higher levers of loneliness and lower scores for quality of life. Although both psoriasis and hidradenitis suppurativa are associated with impaired quality of life and psychological aspects, significantly worse scores were recorded in patients with psoriasis.