HIDRADENITIS SUPPURATIVA IN POSTMENOPAUSE.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic, debilitating disease with a profound impact on the quality of life of patients. OBJECTIVES: To describe a rare case of HS with postmenopausal onset, to review the literature data regarding late onset HS and to discuss the current knowledge on the role of endocrine abnormalities in the development of HS. CASE REPORT: We report the case of a 68-year-old patient in whom HS occurred 10 years after menopause. She was referred to our clinic for the presence of an open fistula on the left groin, fibrotic scars and visible alteration of the vulvar anatomy due to numerous surgical interventions. The patient shared features of the metabolic syndrome (obesity, arterial hypertension, dyslipidemia, aortic atherosclerosis), but showed no signs of virilism and no hormonal abnormality. HS was controlled using antiseptics, topical retinoids and antibiotics. CONCLUSIONS: This case is of particular interest given the late onset of HS, long time after menopause. The development of HS requires a complex interaction between genetic predisposing factors, endocrine dysregulation, metabolic alterations, bacterial overgrowth and an aberrant inflammatory response. Evidence points to an important role of sex-hormones in the emergence and progression of the disease, but the underlying mechanisms are still unclear. A better understanding of HS pathogenesis is needed to elucidate the precise way in which endocrine factors influence the disease onset and course. This would guide the way to novel therapies and a better control of this challenging disease.

POLYGLANDULAR AUTOIMMUNE SYNDROME ASSOCIATED WITH MULTIPLE AUTOIMMUNE CONDITIONS AND ATOPIC DERMATITIS - AN UNUSUAL MANIFESTATION OF A POLYAUTOIMMUNITY PHENOTYPE.

INTRODUCTION: The polyglandular autoimmune syndrome (PAS) type III is a rare condition defined as the coexistence of autoimmune thyroid disorder with other endocrine autoimmune diseases, including type 1 diabetes, without adrenal dysfunction. PAS may associate with other non-endocrine autoimmune diseases, overlapping with the multiple autoimmune syndromes (MAS). We present a case of PAS III/ MAS type 3, including autoimmune thyroiditis, autoimmune diabetes, vitiligo, lupus erythematosus, associated with adult-onset atopic dermatitis, a combination not reported previously. CASE REPORT: A 40 years old woman, registered as nurse working in dialysis unit, previously diagnosed with vitiligo, euthyroid autoimmune thyroiditis and disseminated granuloma annulare, with personal and familial history of atopic disorders, presented in our clinic for disseminated eczematous and lichenoid cutaneous rashes. She was tested positive for antinuclear, anti-double stranded DNA and anti-histone antibodies, with inflammatory syndrome and marginal lymphopenia and she was diagnosed with systemic lupus erythematosus (SLE). Subsequently, moderate hyperglycemia, positive anti-glutamic acid decarboxylase antibodies and low C-peptide level prompted the diagnosis of autoimmune diabetes. Recurrent flexural eczematous rashes, with negative epicutaneous tests but positive specific IgE tests for common allergens fulfilled the clinical criteria for the diagnosis of atopic dermatitis. The clinical, immunological and glycemic status were controlled with low doses of oral prednisone (<0.5 mg/kg), methotrexate (10mg/week), antimalarials, metformin, emollients and photoprotection. After changing her workplace, the immunosuppressive treatment could be discontinued, and the patient maintained normal immunological and biochemical profile at 6 months follow-up.This case brings a unique perspective on the evolution, associations spectrum and the management challenges of endocrine polyautoimmunity associated with atopic diathesis.

Cutaneous complications of molecular targeted therapy used in oncology.

The new molecular targeted therapy has been developed over the past decades by using the molecular targeted molecular changes discovered in specific types of cancer. Unfortunately, most of these agents (epidermal growth factor receptors, multi-targeted small molecule tyrosine kinase inhibitors, monoclonal antibodies) have severe cutaneous adverse reactions, that not only interfere with the patient's quality of life, but also are dose-limiting and may require treatment interruptions. These cutaneous complications and their management must be very well known by any oncologist and dermatologist who treat oncologic patients. ABBREVIATIONS: EGFR = epidermal growth factor receptors, EGFRI = epidermal growth factor receptors inhibitors.

Oxidative stress in androgenetic alopecia.

Rationale:Androgenetic alopecia is not considered a life threatening disease but can have serious impacts on the patient's psychosocial life. Genetic, hormonal, and environmental factors are considered responsible for the presence of androgenetic alopecia. Recent literature reports have proved the presence of inflammation and also of oxidative stress at the level of dermal papilla cells of patients with androgenetic alopecia Objective:We have considered of interest to measure the oxidative stress parameters in the blood of patients with androgenetic alopecia Methods and results:27 patients with androgenetic alopecia and 25 age-matched controls were enrolled in the study. Trolox Equivalent Antioxidant Capacity (TEAC), malondialdehyde (MDA) and total thiols levels were measured on plasma samples. Superoxide dismutase (SOD), glutathione peroxidase (GPx), catalase (CAT) activities, and also non protein thiols levels together with TEAC activity were determined on erythrocytes samples No statistically significant changes were observed for TEAC erythrocytes, non-protein thiols, GPx and CAT activities. Significantly decreased (p<0.01) SOD activity was found in patients with androgenetic alopecia. For plasma samples decreased TEAC activity (p<0.001), increased MDA levels (p<0.001) and no change in total thiols concentration were found in patients when compared with the controls. Discussions:Decreased total antioxidant activity and increased MDA levels found in plasma samples of patients with androgenetic alopecia are indicators of oxidative stress presence in these patients. Significantly decreased SOD activity but no change in catalase, glutathione peroxidase, non protein thiols level and total antioxidant activity in erythrocytes are elements which suggest the presence of a compensatory mechanism for SOD dysfunction in red blood cells of patients with androgenetic alopecia. ABBREVIATIONS: AAG = androgenetic alopecia, MDA = malondialdehyde, SOD = superoxide dismutase, CAT = catalase, GPx = glutathione peroxidase, GSH = glutathione, GST = glutathione transferase, SH = thiols, TEAC = trolox equivalent antioxidant capacity, ABTS = 2,2'-azino-bis (3-ethylbenzothiazoline-6-sulfonic acid), CDNB = 1-chloro-2,4-dinitrobenzene.

Oxidative stress and alopecia areata.

Alopecia areata (AA) is an inflammatory and autoimmune disease presenting with non-scarring hair loss. The aethiopathogenesis of alopecia areata is unclear and many factors including autoimmunity, genetic predisposition, emotional and environmental stress are thought to play important roles in its development. Antioxidant/ oxidant balance perturbation is a common feature in autoimmune, emotional and environmental stress. Therefore, our paper discusses the implications of oxidative stress in alopecia areata.

Cutaneous adverse reactions specific to epidermal growth factor receptor inhibitors.

Classical antineoplastic therapy is encumbered by extensively studied adverse reactions, most often of systemic nature. The emergence of new generations of anticancer treatments, including epidermal growth factor receptor inhibitors, besides improving the response to treatment and the survival rate, is accompanied by the occurrence of new specific side effects, incompletely studied. These side effects are most often cutaneous (hand foot syndrome, acneiform reactions), and in some cases are extremely severe, requiring dose reduction or drug discontinuation. The prevention of the cutaneous adverse effects and their treatment require a close collaboration between the oncologist and the dermatologist. The occurrence of some of these skin adverse effects may be a favorable prognostic factor for the response to the cancer treatment and the overall survival.

Thermal patterns in zoster.

RATIONALE: Varicella zoster virus is a neurotropic virus that causes an infectious disease characterized by skin changes and neuropathic pain. After the resolution of the first infection, the virus lies dormant within the sensory ganglia. The reactivation of the virus causes zoster. An alteration in skin infrared emission might be expected in the areas of the skin affected by inflammatory changes and demyelination of the affected peripheral nerve. OBJECTIVE: To establish the importance of thermal imaging in the follow up of Zoster Zone with different localization. An infrared thermal camera was used in order to assess if the evolution of the disease determines a thermal pattern. METHODS AND RESULTS: Infrared thermography can be used for the assessment of the affected area also by using a thermography camera that is sensitive to the infrared spectrum. An intense and diffuse infrared emission is highly suggestive for the inflammation and implies that a more aggressive treatment should be initiated. After the clinical resolution of the affected area, the symmetry of the thermal pattern should be restored. If the asymmetry persists, a neuropathic complication of the virus reactivation could be involved. DISCUSSIONS: The integration of infrared thermography with the clinical findings is very useful in order to create a complete picture of the zoster lesions and this method could determine the beginning of a correct treatment and, by doing so, minimizing the risk of complications.

Pyoderma gangrenosum, rare parietal complication after colorectal surgery.

Pyoderma gangrenosum (PG) is a rare chronic destructive inflammatory skin disease characterized by the presence of nodules and pustules with progressively enlarging ulcers and orcutaneous necrosis. In most cases PG is idiopathic, but sometimes it is associated with conditions that often have vasculitis, such as gammapathies, inflammatory bowel diseases or chronic arthritis. PG is neither infectious nor gangrenous,but some authors advanced the theory that an infectious etiology (streptococci and staphylococci) could be incriminated.Recently, there are reports in the literature stating that PG is a condition that can occur after local trauma, especially surgery.We report the case of a 73 year old man who presented himself to the Surgery Department with rectal adenocarcinoma and postoperatively developed well-demarcated, vegetative plaques,ranging from 2 to 5 cm in diameter, with central ulceration associated with necrosis and a purulent secretion, delimitated by raised, dusky erythematous borders. These lesions were located on the abdomen and it is important to mention that the injuries occurred at the site of surgical stitches used during the rectum amputation surgery, with no other anatomical locations,pruritus or pain associated. We performed two different histopathological examinations for this patient, regarding the rectal specimens and the cutaneous specimen. The first examinations revealed rectal adenocarcinoma and nonspecific colitis. The second examination was concluded with the diagnosis of pyoderma gangrenosum, the ulcerative stage. The patient had a very good clinical response to systemic steroid therapy.

Non-steroidal anti-inflammatory drug - induced transient reactive papulotranslucent acrokeratoderma.

Transient reactive papulotranslucent acrokeratoderma (TRPA) is an unusual skin condition characterized by the rapid and transient development of symmetric, edematous white papules with eccrine duct prominence on the palms after exposure to water. We present the case of a 28-year-old woman diagnosed in our clinic with TRPA induced by the use of a non-steroidal anti-inflammatory drug. The possible pathophysiology and treatment options are discussed.

Psoriasis and Metabolic Syndrome--scientific evidence and therapeutic implications.

Psoriasis is a chronic inflammatory disease, predominantly affecting the skin, being included in the group of Immune Mediated Inflammatory Diseases. Growing evidence from the last 10 years suggests that several systemic conditions like metabolic syndrome, cardiovascular disease, diabetes, psychological disorders or inflammatory bowel disease are prevalent in psoriasis patients. The linker might be the chronic secretion of pro-inflammatory cytokines. In this current review, the scientific evidence that explains the relationship between psoriasis and the metabolic syndrome in particular will be addressed, as the metabolic syndrome comprises a group of risk factors for cardiovascular disease, thus offering an overall picture of the systemic involvement in psoriasis. An integrated approach, with an early detection and treatment of the components of the metabolic syndrome, are important steps in psoriasis management. Attention should be paid on influence of psoriasis treatment upon comorbidities and vice-versa.

Photodynamic therapy in the treatment of basal cell carcinoma.

Photodynamic therapy (PDT) is a medical procedure based on the activation of the molecules of various exogenous or endogenous chemical substances called photosensitizers by a light source emitting radiation of an adequate wavelength, usually situated in the visible spectrum; photosensitizers are chemical compounds bearing the capacity to selectively concentrate in the neoplastic cells. The energy captured by the molecules of these substances pervaded in the tumor cells is subsequently discharged in the surrounding tissue, triggering certain photodynamic reactions that result in the destruction of the tumor. The procedure is applicable in numerous medical fields. Skin basal cell carcinoma (BCC), the most frequent type of cancer of the human species, is a cutaneous tumor that responds very well to this innovative treatment method. By reviewing numerous recent studies in the field, this article aims to present the role and the indications of photodynamic therapy in the management of basal cell carcinoma, as well as the most important results achieved so far by this therapy in the field of dermato-oncology.

Inadequate cortisol synthesis in prophyria cutanea tarda.

Many common clinical features suggest that between corticosuprarenal insufficiency (CSRI) and porphyria cutanea tarda (PCT) there may be some pathogenic relationships. In order to further understand these relations we have performed the ACTH-depot stimulation test (1 mg, i.m.) in 9 patients (from 13 males) with PCT. In 8 patients cortisolemia was assayed 1, 2, (12) and 24 hours post-stimulation. In all 13 cases the basal eliminations of cortisol metabolite (17-OH-corticosteroids) were under normal limits: 2.88 mg/24 h/g creatinine vs 15 controls with 7.06 mg/24h/g creatinine. After ACTH four cases showed lack of stimulation, considered on the second day for 17-OH-corticosteroids. In one case, after one year of PCT treatment, the early post-stimulation level is only moderately decreased. In one case, the test was normal. In four cases the ACTH stimulation was over-normal, i.e., greater than on the first day, suggesting supraphysiological responses. In this group 2 patients showed unexpectedly low early stimulation slopes on cortisolemia (at 1 and 2 hours) associated with concordant high late stimulation levels. This later phenomenon suggests a functional impaired secretion of cortisol in PCT, which seems to be similar to that of insulinemia after glucose in NIDDM, as a receptor lesion. The lesions of cortisol secretion in PCT could have been made by porphyrin storage, impaired hem-enzyme synthesis (cyt P-450) and as a new and attractive hypothesis, could be due to mitochondrial porphyrin receptor decreased activity.

Preliminary data concerning association of porphyria cutanea tarda and adrenocortical insufficiency. Report of two cases.

The paper presents two cases in which clinical and laboratory investigations have revealed the simultaneous presence of porphyria cutanea tarda (PCT) and adrenocortical insufficiency (ACI). Whether this association is a coincidence, whether there is a common cause, or a "cause to effect" type relationship between the two entities is a question of debate. Without ignoring other possible causes (tuberculosis, hemochromatosis, autoimmunity, drugs) it is suggested that heme deficit consequent to inefficient protoporphyrin synthesis results in impaired steroidogenesis, due to decreased levels of adrenocortical hemoprotein enzymes, especially cytochrome P-450 dependent hydroxylases. Cytochrome P-450 has a well known sine-qua-non activity in steroid hydroxylations within steroid tissues, and a decrease in the levels of this heme-enzyme might be expected to result in impaired steroidogenesis. However, the association between PCT and ACI (whether pathogenic or not) is pathophysiologically characterized by the coexistence of a disease (PCT) in which the anatomo-clinical manifestations are determined by oxygen derived free radicals ( ODFR ), and a disease (ACI) which, among other consequences, presents a marked reduction in ODFR -scavenge capacity. The authors consider this supposition an attractive working hypothesis, suitable to further clinical laboratory direct testing.

Application of mathematical methods to the study of familial aggregation in endemic nephropathy. I. The hypothesis of infectious etiology.

Among many etiological factors, infection and the genetic factor are the main two causes which could explain the familial aggregation of endemic nephropathy (EN). The data collected over all the EN cases during 1957--1976 in the endemic village Bistrita have been analysed for time-space clustering and a particular type of spatial clustering (within household) by the Knox's space-time interaction test and Walter's pair statistic test. The lack of clustering could be interpreted as suggesting the lack of infection (human transmission pattern) in the occurrence of endemic nephropathy.