Necrobiotic Xanthogranuloma - Case Report and Literature Review.

Necrobiotic xanthogranuloma is a rare type of non-Langerhans histiocytosis, whose main clinical features are the development of red-brown, purplish or yellowish skin papules and nodules, which evolve by forming infiltrated plaques. The periorbital region is the most commonly affected site. Some cases have lesions on the torso or extremities, with no facial involvement. Extracutaneous involvement of the ocular, respiratory, and cardiac tissues have also been described. Most patients have an associated monoclonal gammopathy (IgG k and λ). The treatment is difficult, with progression and recurrence. We present the clinical case of a 65-year-old woman, who was hospitalized for multiple erythematous plaques and placards, with fine squames and telangiectasis on the surface, disseminated within the scalp, ears, trunk, lower limbs; some plaques have a circinate border with reddish-purple, slightly protruding edges and a whitish and erosive atrophic center. The lesions within the scalp are alopecic. The disease began 15 years ago, the patient being diagnosed with Psoriasis vulgaris and treated with dermatocorticoids and Cignolin, with no remarkable results. Paraclinical investigations did not reveal any associated pathologies. Histopathological and immunohistochemical examination confirmed the diagnosis of necrobiotic Xanthogranuloma. The patient was treated with antihistamines, Neuromultivit, Vit E 100mg/day, Oximed spray, Atoderm emollient cream, Neopreol ointment, with slow favorable evolution. The physical examination and laboratory investigations for the diagnosis and surveillance of malignant diseases should be performed on a regular basis in patients with NXG. Our patient had lesions with a course of 15 years, with no development of multiple myeloma or other systemic involvement.

Adenoid Cystic Basal Cell Carcinoma Arising in Rhinophyma.

Adenoid cystic is a rare histological subtype of basal cell carcinoma. Basal cell carcinoma (BCC), the most common form of skin cancer is a slow-spreading tumor with local malignancy, with a high cure rate and favorable prognosis when is diagnosed in the early stages. Rhinophyma is one of the four subtypes of rosacea and it is histopathologically characterized by sebaceous glands hypertrophy. Disseminated annular granuloma (GAD) is a rare inflammatory dermatosis with uncertain etiopathogenesis, clinically represented by papules with annular or arciform aspect. UV radiations aggravates rosacea and are involved in the etiopathogenesis of BCC and can have a triggering or an aggravating effect on GAD. The association of adenoid cystic BCC with rhinophyma it is rare and more than that, the presence of both in association with GAD is not described in medical literature. We present the case of a 78-year old male patient, with personal history of prolonged exposure to UV radiations, that was admitted to the Dermatology for 4 nodular tumors, located on the face. The skin of the nose and menton with thickened, hypertrophied, irregular, red appearance and dilated pores. Also, on the upper limbs and trunk, the patient had, erythematous papules with arciform and ring shape with hypopigmented centers. We performed surgical excision of the tumors and biopsied an annular lesion. Microscopic examination showed nodular basal cell carcinoma with areas of adenoid cystic carcinoma and actinic degeneration of collagen and gigantocellular granulomatous inflammation. The skin biopsied from the upper limb showed annular granuloma appearance.

Recurrent Idiopathic Sweet Syndrome - Case Report and Literature Review.

INTRODUCTION: Sweet syndrome (SS), also denominated as acute febrile neutrophilic dermatosis, is a rare disease characterized by the sudden onset of painful, erythematous, firm skin lesions (papules, plaques, and nodules) which show, upon histologic examination, the presence of a diffuse infiltrate of mature neutrophils. The cutaneous manifestation typically involves the face, neck, trunk, and upper limbs and is associated with fever, general malaise, arthralgia. CASE REPORT: A female patient, 60 years old, attended the Dermatology Clinic due to the appearance of violaceous erythematous-oedematous infiltrated plaques, located on the face, neck, upper limbs, trunk and knees. The onset of the cutaneous manifestation had occurred 2 months prior, accompanied by pain, chills, flares of fever and arthralgia. The onset coincided with the surgical treatment of an umbilical hernia. From the medical history we note that the patient was diagnosed in 2014 with histiocytoid SS. She followed a treatment with methylprednisolone, with positive response, but had many relapses after the discontinuation of treatment. In 2017, due to a new episode, the histopathological examination was repeated, which revealed classical SS. She received treatment with Disulone and Colchicine. She had not been administered any treatment throughout the previous year. Laboratory tests revealed leukocytosis with neutrophils, increased ESR, elevated C4, hyperglycemia. The current histopathological examination revealed lymphocytic SS. Under treatment with methylprednisolone 32mg/day, the evolution was favorable. DISCUSSIONS: The first case of SS was described by Robert Douglas Sweet in 1964. As known aetiological factors there have been described gastrointestinal and urinary tract infections, pregnancy, inflammatory bowel disease, drugs or malignancies. There have been described cases of SS that appeared after surgical treatment, as in our case, which registered a new outbreak following the umbilical hernia treatment. The histopathological variants of SS described in the literature are: subcutaneous, eosinophilic, histiocytoid, lymphocytic type. The first line-therapy consists in systemic corticosteroids, which induce a fast remission of lesions and general symptoms. Recurrence may occur in approximately 50% of patients and is common in idiopathic or paraneoplastic cases. CONCLUSIONS: In addition to the neutrophilic infiltrate that is typical for Sweet syndrome, different types of histological manifestations have been described in the literature: subcutaneous, eosinophilic, histiocytoid, lymphocytic. In our case, we noted that the histological profile changed over time, from a histiocytoid SS recorded in 2014, to a classical SS in 2017, followed by the appearance of lymphocytic SS in 2019. Due to the fact that SS can be associated with a numerous other disorders, our patient requires regular monitoring with a view to eliminate them, and potentially to make a diagnosis and initiate early specific treatment.

Immunoexpression of p53 and COX-2 in basal cell carcinoma.

Basal cell carcinoma (BCC) is a variety of cutaneous carcinoma associated with an excellent prognosis because it rarely metastasizes, but it can cause significant local destruction and morbidity if surgical excision is not made. In this study, we examined the immunohistochemical expression of p53 and cyclooxygenase-2 (COX-2) in 51 BCCs, nodular and infiltrative subtypes, with various Clark levels. The immunoexpression of p53 was identified in 74.5% BCC cases and COX-2 reactions in 88.2% of cases. The scores of p53 reactions revealed significant differences depending on Clark level and borderline significance with tumor type, the high positive scores being associated to infiltrative tumors and high Clark level. No differences were revealed between COX-2 scores with both Clark level and tumor type. The analysis of the percentage values of p53 and COX-2 indicated a positive linear correlation. The positivity of p53 and COX-2 in a large proportion of BCCs, regardless of histological type and of depth of invasion, supports the two markers involvement in tumor progression.

Prognostic factors in squamous cell carcinoma of the lower lip - an immunohistochemical study.

Squamous cell carcinoma (SCC) of the lip represents 15-30% of SCC of cephalic extremity, located on the lower lip in about 90% of cases. The present paper aimed to define the profile of SCC of the lip with major risk factors. The study included 20 selected cases diagnosed with lower lip SCC, using a panel of antibodies which addressed cell proliferation (Ki67), perturbation of the cell cycle (p53), angiogenesis (VEGF - vascular endothelial growth factor), factors related to tumor cell interaction with the extracellular matrix (CD44). Ki67 immunoexpression was identified in all the cases. Poorly differentiated (PD) SCC presented a mean value of Ki67 positivity index (PI) significantly higher compared to well-differentiated (WD) and moderately differentiated (MD) SCC. We found significantly higher mean values of Ki67 PI in pT3 lesion, compared with pT2 and pT lesions, and with no statistically significant differences in lip SCC with associated lymph node metastasis (pN1), compared to those with no lymph node metastasis (pN0). PD SCC presented a higher mean value of p53 PI compared to WD and MD SCC, but without significant differences. Analysis indicated significantly higher values in pT3 lesions and in pT2 and pT1 and in pN1 SCC. In WD SCC, CD44 immunoexpression had a higher intensity. For PD and MD SCC the immunolabelings presented low÷moderate heterogeneous intensity. WD lip SCC presented a statistically significant higher mean value for CD44 PI compared to MD and PD SCC, and not statistically significant higher in pT1, pT2 then in pT3 and in pN0 cases then in pN1. WD lip SCC presented statistically significant higher mean value of VEGF PI related to those with MD and PD SCC. VEGF PI values were higher in pT1, pT2 then in pT3 and in the pN0 SCC, but without statistically significant differences. We found a positive linear correlation for Ki67÷p53, although statistically not significant and for CD44÷VEGF statistically significant (p=0.001). Also, the analysis identified negative a linear statistically significant correlation for Ki67÷CD44 and for Ki67÷VEGF statistically significant as well (p=0.001). Immunohistochemical investigations in lip SCC, regarding the expression of p53, Ki67, CD44 and VEGF, revealed results that suggest their ability to assess the prognosis and progression of tumor evolution.

The immunohistochemical analysis of the proliferative activity and the maturity degree of lymphatic vessels in oral squamous cell carcinomas.

Oral cancers still represent a major health problem; regional lymph node metastases occur in 30-40% of head and neck squamous cell carcinomas and are associated with unfavorable prognosis and decreased survival. The study included 35 cases of oral squamous cell carcinomas (OSCC), which were analyzed by double reactions to determine the proliferative activity (anti-human D2-40/Ki67) and the maturity degree (anti-human D2-40/α-SMA) of lymphatic vessels, both intratumoral (IT) and in the advancing edge (AE), and in relation to clinicopathological prognostic parameters. The mean values of D2-40 lymphatic vessel density (LVD) were higher in AE then in IT level. Poorly differentiated carcinomas, T3/T4, presented the highest LVD values, both IT and in the AE. LVD was higher in advanced stages and metastasizing carcinomas. Ki67 was positive in all cases, Ki67 proliferation index (IP) indicated higher values in poorly differentiated carcinoma, T3/T4, metastasizing ones, both IT and in the AE. LVD and IP Ki67 showed a positive linear correlation. D2-40/Ki67-positive vessels were identified only at the AE or close to it. D2-40/Ki67 LVD had highest values in advanced stages carcinoma, with metastases. D2-40/α-SMA-positive vessels were identified only in the neighborhood of the tumor and LVD highest values were present in early-stage carcinomas and without metastases. A negative linear correlation between proliferation and maturity of the lymphatic vessels was found. The study indicated a strong association between lymphatic proliferative activity and lymph node metastases, suggesting the need for targeted antilymphangiogenic therapies in OSCC.

Solitary trichoepithelioma: clinical, dermatoscopic and histopathological findings.

UNLABELLED: Trichoepithelioma is part of the adnexal carcinomas. It is a benign, small tumor, usually under a centimeter in diameter, which develops in the basal cells of the hair follicle. CASE REPORT: We present the case of a 30-year-old female patient, from rural area, which was hospitalized in the Clinic of Dermatology, Emergency County Hospital, Craiova, Romania, in March 2015, for the presence of a skin tumor, with round-oval shape, 0.5 cm in diameter, brownish colored, located on the left cervical region. The patient had no past medical history. The skin tumor appeared two years ago, and it was slowly increasing in size. Physical examination was in normal ranges. The dermatoscopic examination reveal a pearly white background covered with tumor islands that were oval shaped, with hyperpigmentation, centered by keratin cysts and surrounded by collagen. During hospitalization, we performed tumor biopsy. The histopathological examination showed microscopic structure of trichoepithelioma. After history taking, physical examination, dermatoscopic examination and histopathological result, our diagnosis was left cervical trichoepithelioma. CONCLUSIONS: Trichoepithelioma is a rare trichogenic tumor, which appears at any age, including newborns, with potential for local recurrence. Our case report represents a classic dermatoscopic aspect of trichoepithelioma, that can have close resemblance to basal cell carcinoma and other skin adnexal tumors, clinically, dermatoscopic and histopathological.

An immunocompetent young patient with tuberculosis of the penis: a challenging case.

Tuberculous chancre is an extremely rare form of cutaneous tuberculosis. The genital area is a possible site of presentation. We present a case of a young male with a persistent balanopreputial ulceration resembling a luetic chancre with negative serology for syphilis. The diagnosis was based on the specific pathologic features and the positive intradermal reaction to tuberculin. A successful treatment was achieved by combining antituberculosis treatment and surgical approach with circumcision. After six months of antituberculosis treatment, the patient developed paradoxical inguinal lymph node enlargement, which, after surgical excision and biopsy, was not followed by a relapse of the disease and needed no further therapy. Tuberculosis should be considered a potential diagnosis in the case of a persistent genital ulcer.

Squamous cell carcinoma developed on chronic venous leg ulcer.

Chronic venous leg ulcers (VLU), especially long-lasting non-healing ulcers, are among the risk factors for squamous cell carcinoma (SCC). Malignant transformation of a VLU is a rare finding and the relative risk of carcinomatous transformation is quite low (about 5.8). SCC arising in the context of a VLU has a particularly aggressive behavior. A 76-year-old male patient with no relevant medical familial history, with chronic venous insufficiency CEAP C6 for 10 years [recurrent leg ulcers with favorable outcome (healing) after specific local and systemic treatment], showing for about three years one ulcerated lesion located on the anterior upper third of the right calf non-responsive to specific treatment, which subsequently increased their size and merged. Biopsy sample was taken. Histopathology showed epidermal acanthosis, papillomatosis, intense parakeratosis, pseudoepitheliomatous hyperplasia, dysplasia and moderately differentiated squamous cell carcinoma with areas of acantholysis. Immunohistochemistry (Ki67, EMA, cytokeratin 34ßE12 and p63) was performed and all types of immunostaining were moderately to intense positive. Above-knee leg amputation and specific oncologic treatment were proposed as possible curative solutions but the patient refused. Ten months after diagnosis and discharge form the Department of Dermatology, the patient died. Patients with chronic venous leg ulcers and clinically suspicious lesions should be evaluated for malignant transformation of the venous lesion. When diagnosed, malignancy complicating a chronic venous leg ulcer requires a resolute treatment as it may be fatal.

Cutaneous mastocytosis, problems of clinical diagnosis of four cases.

Mastocytosis is a rare disease characterized by a pathological increased of mast cells in one or more tissues, particularly in the skin, bone marrow, liver, spleen, lymph nodes and gastrointestinal tract. Cutaneous mastocytosis represents over 90% of cases found with predilection in children. The aim of the paper was to summarize the authors' clinical, histopathological and immunohistochemical observations on patients with cutaneous mastocytosis. We present four cases of cutaneous mastocytosis, sporadic form, customized by clinical presentation and age of onset: two installed in the neonatal period, a case with onset in infancy and another in adulthood. For the assessment of the severity and the effectiveness of the treatment, we used SCORMA Index. We performed in each patient histopathological examination of the skin (Hematoxylin-Eosin and Giemsa stains), the dosage of mediators (serum tryptase level, serum histamine levels, urinary histamine metabolites) and the balance of expansion (complete blood cell count, liver biological investigations, abdominal ultrasound, skeletal radiography, chest radiography). For the adult with mastocytosis, we performed abdominal scanner and cytological study of the bone marrow. Following investigations carried out in each case, we mentioned the diagnosis of cutaneous mastocytosis, and also excluded several diseases confounded by clinically and histologically aspect. Considering the fact that the balance expansion was negative, we excluded the diagnosis of systemic mastocytosis. The presence of anemia and protein energetic malnutrition in children with mastocytosis involves carrying out balance extension for the exclusion of a systemic form of the disease. Histopathological examination of the skin using special stains, the dosage of mediators (serum tryptase level, serum histamine levels, urinary histamine metabolites) and balance expansion establish the diagnosis of cutaneous mastocytosis and also exclude many confusions because of the clinical presentation.

Ulcerated necrobiosis lipoidica to a teenager with diabetes mellitus and obesity.

Many skin lesions are associated with diabetes mellitus (DM) type 1 or 2, due to the use of antidiabetics or to metabolic and endocrine disorders caused by this disease. Necrobiosis lipoidica (NL) occurs more frequently in patients with DM. Painful ulcerations may occur on NL areas in about 20-25% of the cases and usually they are related to trauma. We present the case of a teenager, male, 17-year-old, having NL with multiple plaques, some of them spontaneously ulcerated after about 33 months of onset. He is known with type 1 DM from 2.5 years and the NL preceding the diagnosis of diabetes mellitus with about six months, presented erythematous-infiltrative skin plaques, some ulcerated for about three months, interesting both shins. Based on clinical, histopathological and paraclinical examinations, we established the following diagnoses: ulcerated NL, type 1 DM, moderate mixed dyslipidemia, class I obesity; commissural candidiasis, juvenile acne. Under treatment with Pentoxifyllinum, Sulodexidum, Ketotifenum and topical therapy with 0.2% Hyaluronic acid two months later, we have managed to heal two of the three ulcerated plaques and of the third has become superficial. We applied 0.5% Fluocortolonum on non-ulcerated plaques recording an improvement after two weeks of treatment. NL is a skin disease with a predilection for the shins, more frequent in patients with diabetes and is a part of palisading granulomatous dermatitis, which leads to skin atrophy. NL is found in the 0.3-1.2% of diabetic patients and is rare in children with diabetes (0.006%). It is more common in the patients with type 1 DM. The onset is in the third decade in diabetic patients and in the fourth decade in non-diabetics. There is no consensus concerning the treatment of NL, and the results are often modest. Antiplatelet agents, corticosteroids (local and general), immunomodulatory drugs, cyclins, wide synthetic antipaludics, heparin, Thalidomide are used. NL treatment is very difficult, especially in the ulcerated forms. Many of the drugs listed have proven efficacy only in isolated cases. Studies are necessary on large series of patients to determine the optimal therapy of NL.

Epidemiological aspects of genital warts in romania - a 2012 retrospective survey.

OBJECTIVES: Genital infection with human papillomavirus (HPV) has become one of the most frequently viral sexually transmitted diseases. The infection may remain asymptomatic, may take the form of external genital warts and may give raise to cervical cancers. The aim of this study was to assess the frequency of the patients with genital warts addressing to five tertiary referral dermato-venereological units in Romania (where patients from several counties are referred) and to compare the results with the out-patient data reported by all Romanian hospitals. MATERIAL AND METHODS: Data regarding patients with external genital warts who addressed to the hospital emergency rooms, in five tertiary referral dermato-venerological units in Romania (Bucharest, Timisoara, Craiova, Constanta, Târgu-Mures) were collected for the year 2012. For comparison there have been used data collected by the National School of Public Health, Management and Professional Development, during the same year. OUTCOMES: Data reported at national level in 2012 included 952 patients (731 women and 221 men) with 26 males under 20 years of age and 251 female patients in the age group 0-20 years. In the overall population (males and females combined) the total number of genital warts cases registered at the hospital emergency rooms in the five centers, in the year 2012, was 266 patients (119 men and 147 women) with 4 girls under 14 years of age and 6 male patients in the age group 0-14 years. CONCLUSIONS: The high prevalence of the infection with HPV, the costs of treatment and the psychological impact prove that prevention of the disease is the most important step for decreasing the incidence of this disease. General measures for patients awareness regarding the disease and its complications need to be completed with the recommendation for vaccination. A closer monitoring of patients would provide information for a strategic national approach leading to a better outcome.

Recommended strategies for epidermolysis bullosa management in romania.

BACKGROUND: There are 72 families with epidermolysis bullosa (EB) in Romania. Since 2012 a National Program for the treatment of these patients is run by the Ministry of Health.The objectives of the strategies for EB patients are to optimize the management (diagnosis, treatment, monitoring) and to provide actual information on classification and patho-physiology which dictate the course of the disease. METHODS: An international expert panel of specialists produced by consensus the recommendations for the management of EB cases in Romania taking into account the local possibilities. Patient association proposals were included. A review of the literature was performed to up-date the information. OUTCOMES: A strategy for diagnosis, treatment and follow-up of the patients with EB was elaborated in clear steps. Pharmacological treatments and wound care indications are provided together with a useful score for patient evaluation. CONCLUSION: These recommended strategies are allowing dermatologists to generate an individualized care plan for patients with EB.