Orwik

Leprosy and HIV Coinfection: A Clinical, Pathological, Immunological, and Therapeutic Study of a Cohort from a Brazilian Referral Center for Infectious Diseases.

Background. Although awareness of the relevance of leprosy and human immunodeficiency virus (HIV) coinfection is increasing worldwide, several aspects of this co-occurrence are not fully understood. Methods. We describe clinical, pathological, immuno... expand abstractlogical, and therapeutic long-term follow-up of a cohort of 25 individuals with leprosy and HIV infection from Manaus, Amazonas. Results. Careful description of our cohort indicates a higher prevalence of leprosy in an HIV-positive population than that in the general population. We also observed upgrading shifting of leprosy clinical forms after initiation of highly active antiretroviral therapy and multidrug therapy and an impact of HIV infection on leprosy granuloma formation, among other features. Conclusion. Taken together, these new insights allow the proposition of a classification system that includes (1) leprosy and HIV true coinfection, (2) opportunistic leprosy disease, and (3) leprosy related to highly active antiretroviral therapy. collapse abstract

Dermatomyositis during adalimumab therapy for rheumatoid arthritis.

Histopathologic diagnosis of leprosy in a nonendemic area.

T regulatory cells and plasmocytoid dentritic cells in hansen disease: a new insight into pathogenesis?

Leprosy is characterized by spectrum of histologically different granulomatous skin lesions that reflects the patient's immune response to Mycobacterium leprae. Presence, frequency, and distribution of both CD4+ CD25+ FoxP3+ T regulatory cells (T-reg... expand abstracts) and CD123+ plasmacytoid dendritic cells in leprosy have never been investigated. We performed a retrospective immunohistochemical study on 20 cases of leprosy [tuberculoid tuberculoid (TT): 1 patient; borderline tuberculoid (BT): 3 patients; borderline lepromatous (BL): 5 patients; lepromatous lepromatous (LL): 5 patients; borderline borderline in reversal reaction (BB-RR): 1 patient; BT-RR: 2 patients; and erythema nodosum leprosum (ENL): 3 patients]. FoxP3-positive cells were present in 95% of the cases with an average density of 2.9% of the infiltrate. Their distribution was not related to granulomatous structures or special locations. There was no statistical difference of FoxP3 expression between TT, BT, BL, and LL, whereas a statistical significant increment (P = 0.042) was observed in patients affected by reversal leprosy reactions (BT-RR and BB-RR) compared with patients affected by ENL and patients with nonreactional disease forms (BL, LL, BT, TT). CD123 expression was not observed in any of the biopsy specimens evaluated; with the exception of 2 cases of ENL, in which a focal positivity for CD123 was observed. Our results show that plasmacytoid dendritic cells are not involved in the immune response against M. leprae while T-regs are present in leprosy skin lesions. These data raise the question if T-regs have a pathogenetic role in HD as previously demonstrated in Leishmania major and Mycobacterium tuberculosis. collapse abstract

Can we really separate palmoplantar pustulosis from psoriasis?

Borrelia Burgdorferi "sensu lato" in Brazil: Occurrence confirmed by immunohistochemistry and focus floating microscopy.

In the present study, we report the occurrence of Lyme's borreliosis in patients from the Brazilian Amazon Region. Borreliosis was investigated by immunohistochemistry and focus floating microscopy for Borrelia burgdorferi in skin biopsy samples from... expand abstract 22 patients with both clinical and histopathology evidences compatible with Erythema Migrans. Spirochetes were detected by specific immunohistochemistry and focus floating microscopy for B. burgdorferi in samples from five patients. Clinical cure of the cutaneous lesions was observed in all the patients after treatment with doxycycline regimen as proposed by the Center Disease Control guidelines. A limitation of our study was the fact that we were not able to isolate and culture these organisms. These are the first known Brazilian cases of borreliosis to have Focus Floating Microscopy confirmation. collapse abstract

Paradoxical reactions to targeted biological treatments: A way to treat and trigger?

Teledermoscopy: education, discussion forums, teleconsulting and mobile teledermoscopy.

Teledermoscopy has become in the last years one of the most florid reality of teledermatology. Parallel to the achievement of dermoscopy in clinical settings, teledermoscopy has grown in different fields, namely tele-education and teleconsulting. Blo... expand abstractgs, atlases, discussion forums, on line courses and Diploma Courses do not only offer a second opinion consultation but give the opportunity to residents in dermatology and dermatologists with different level of expertise in dermoscopy to easily learn at home, to train or to improve their level in dermoscopy. On the other side, in some countries demand for melanoma screening has led to commercialization of "teledermoscopy" by different companies. Images nowadays can be transmitted over telecommunication networks not only via e-mail or a specific web application but also with last generation cellular phones. This reality opens the new incoming field of mobile teledermatology. Mobile teledermoscopy is a new horizon that might become in the future the basis of the self examination of pigmented skin lesions as a screening tool for malignant cutaneous tumors or to follow-up of high risk patients. collapse abstract

Erythematous macules in a 14-year-old girl.

Widespread crusted lesions in a patient with HIV--quiz case.

Vincristine, idarubicin, dexamethasone and thalidomide in scleromyxoedema.

Scleromyxoedema is a rare disease of unknown aetiology that is characterized by progressive cutaneous mucinosis and paraproteinaemia. A variety of systemic (e.g. gastro intestinal, neurological, pulmonary, cardiac and renal) complications may lead to... expand abstract significant morbidity and mortality necessitating therapeutic intervention. The latter remains challenging. Numerous treatment modalities have been reported in the literature, often, however, with inconsistent responses, frequent relapses and potentially serious side-effects. Moreover, the rarity of scleromyxoedema has prevented the execution of controlled therapeutic trials. This paper discusses current proposed therapeutic strategies and reports the case of a 64-year-old male patient with progressive scleromyxoedema associated with IgG-lambda paraproteinaemia in whom monthly administrations of vincristine, idarubicin and dexamethasone in addition to daily oral thalidomide led to clinical and laboratory remission within 12 weeks. collapse abstract

Unilesional pemphigus vulgaris of the scalp.

Do you know this syndrome?

Brooke-Spiegler syndrome is an autosomal dominant inherited disease with predisposition to cutaneous adnexal neoplasms, most commonly cylindromas and trichoepitheliomas. Its onset is in the second or third decades of life. The histopathological exams... expand abstract of the lesions revealed a plethora of benign adnexal neoplasms, showing apocrine, follicular, and sebaceous differentiation. The treatment can be performed by excisional surgery, laser, cryotherapy, electrofulguration and dermabrasion. Due to the risk of malignancy, there is the need for clinical follow-up and genetic counseling. collapse abstract

Mobile teledermoscopy--melanoma diagnosis by one click?

Mobile telemedicine integrates wireless communications for different telemedical applications, such as mobile phones and personal digital assistants, and with the implementation of modern wireless telecommunication, wireless local area network and sa... expand abstracttellite communication is a reality. New generation cellular phones or personal digital assistants have overcome limitations of image quality seen in older devices and, with dermatology being a visual profession, mobile teledermatology is perhaps the most recent development in this field. Mobile teledermatology may provide a triage service aimed toward management of patients with emergent skin disease or for follow-up with patients requiring systemic treatment. Teledermoscopy enables rapid transmission of dermoscopic images via e-mail or specific web-application and studies have demonstrated a high, 91%, concordance between face-to-face diagnosis and remote diagnosis of such images. Further to this, telediagnosis of melanocytic skin neoplasms achieved a diagnostic accuracy of 83% versus the conventional histopathologic diagnosis. Mobile teledermoscopy is the combination of such approaches enabling transfer of images captured with cellular phones coupled with a pocket dermatoscope and preliminary studies have demonstrated the feasibility and potential of its use in triage of pigmented lesions. Such applications are of benefit to physicians in enabling easy storage of data for follow-up or referral of images for expert second opinion and may facilitate a "person-centered health system" for patients with numerous moles and pigmented skin lesions who could forward images for evaluation. The incidence of skin cancers has reached epidemic proportions among whites and the trend is still going upward. Mobile teledermatology and teledermoscopy may be implemented as a triage or screening tool for malignant tumors to facilitate early detection and diagnosis, which is crucial for improved patient outcomes. While the legal aspects concerning teleconsultations need to be evaluated, the communications technologies provide a unique opportunity for physicians and patients alike and we foresee a place for these tools in dermatology soon. collapse abstract

Confocal laser scanning microscopy-guided surgery for neurofibroma.

The neurofibromatoses comprise at least two separate genetic disorders with variable clinical features and an unpredictable course. The most common type, neurofibromatosis 1, is characterized by > or = 6 café-au-lait spots and the occurrence of neuro... expand abstractfibromas, which may present as cutaneous, subcutaneous or plexiform lesions. Normally, excision of neurofibromas is only indicated in the presence of neurological symptoms, suspicion of malignancy or for exceptional cosmetic reasons. For a good functional and aesthetic result with the least danger of recurrence, the surgeon's goal is to excise as much tissue as necessary and as little tissue as possible. One of the main issues during the surgical procedure is to distinguish between neurofibroma and surrounding tissue. We report for the first time the use of confocal laser scanning microscopy to differentiate between neurofibroma and healthy skin. collapse abstract

Reflectance confocal microscopy of facial lentigo maligna and lentigo maligna melanoma: a preliminary study.

BACKGROUND: Facial lentigo maligna (LM) and lentigo maligna melanoma (LMM) may be difficult to diagnose clinically and dermoscopically. Reflectance confocal microscopy (RCM) enables the in vivo assessment of equivocal skin lesions at a cellular level... expand abstract. OBJECTIVES: To assess cytomorphological and architectural RCM features of facial LM/LMM. METHODS: Four women and eight men aged 58-88 years presenting with facial skin lesions suspicious of LM/LMM were included. In total, 17 lesion areas were imaged by RCM before biopsy. The histopathological diagnosis of LM was made in 15 areas; the other two were diagnosed as early LMM. RESULTS: A focal increase of atypical melanocytes and nests surrounding adnexal openings, sheets of mainly dendritic melanocytes, cord-like rete ridges at the dermoepidermal junction (DEJ) and an infiltration of adnexal structures by atypical melanocytes were found to be characteristic RCM features of facial LM/LMM. Areas with a focal increase of atypical melanocytes and nests surrounding adnexal openings were observed at the basal layer in three cases. The remaining cases displayed these changes at suprabasal layers above sheets of mainly dendritic melanocytes. Cord-like rete ridges at the DEJ and an infiltration of adnexal structures by atypical melanocytes were observed in all cases. Previously described criteria for RCM diagnosis of melanoma, such as epidermal disarray, pleomorphism of melanocytes and pagetoid spreading of atypical melanocytes, were additionally observed. CONCLUSIONS: We observed a reproducible set of RCM criteria in this case series of facial LM/LMM. collapse abstract

The influence of clinical information in the histopathologic diagnosis of melanocytic skin neoplasms.

BACKGROUND: We tested the relevance of clinical information in the histopathologic evaluation of melanocytic skin neoplasm (MSN). METHODS: Histopathologic specimens from 99 clinically atypical MSN were circulated among ten histopathologists; each cas... expand abstracte had clinical information available in a database with a five-step procedure (no information; age/sex/location; clinical diagnosis; clinical image; dermoscopic image); each step had a histopathologic diagnosis (D1 through D5); each diagnostic step had a level of diagnostic confidence (LDC) ranging from 1 (no diagnostic certainty) to 5 (absolute diagnostic certainty). The comparison of the LDC was employed with an analysis of variance (ANOVA) for repeated measures. FINDINGS: In D1 (no information), 36/99 cases (36.3%) had unanimous diagnosis; in D5 (full information available), 51/99 cases (51.5%) had unanimous diagnosis (p for difference between proportions <0.001). The observer agreement expressed as kappa increased significantly from D1 to D5. The mean LDC linearly increased for each observer from D1 through D5 (p for linear trend <0.001). On average, each histopathologist changed his initial diagnosis in 7 cases (range: 2-23). Most diagnostic changes were in D2 (age/sex/location). INTERPRETATION: The histopathologic criteria for the diagnosis of MSN can work as such, but the final histopathologic diagnosis is a clinically-aided interpretation. Clinical data sometimes reverse the initial histopathologic evaluation. collapse abstract

Efalizumab for severe palmo-plantar psoriasis: an open-label pilot trial in five patients.

BACKGROUND: Palmo-plantar psoriasis (PPP) is a disabling condition that significantly impairs quality of life. PPP tends to be resistant to conventional therapies and may last for several years. Topical treatments are usually ineffective. Systemic th... expand abstracterapy with oral retinoids and psoralen plus ultraviolet A is frequently required, although it rarely leads to remission. STUDY DESIGN: We conducted an open-label, pilot study to evaluate treatment of PPP with efalizumab, an anti-CD11a monoclonal antibody approved for the treatment of chronic, refractory moderate to severe plaque psoriasis in adults. METHODS: Five patients with severe PPP received efalizumab treatment for 24 weeks. RESULTS: All five patients responded favourably by week 12 and showed further improvement at week 24 of uninterrupted therapy. Mean physician-assessed severity scores and patient-reported outcome scores improved almost 75% after 12 weeks and 90% after 24 weeks. At week 32, three patients maintained the response seen at week 24, while two patients suspended efalizumab. CONCLUSIONS: Efalizumab therapy was well tolerated and effective in five patients with severe PPP, allowing a significant improvement in quality of life. collapse abstract

Bullous papular-purpuric gloves and socks syndrome in a 42-year-old female: molecular detection of parvovirus B19 DNA in lesional skin.

Papular-purpuric gloves and socks syndrome is a self-limited febrile illness of children and young adults. Only 50 well documented cases have been reported, most of which were associated with parvovirus B19 infection. Molecular detection of the virus... expand abstract from lesional skin has been described in only 5 patients. The syndrome is characterized by a papular-purpuric edematous rash in a distinct "gloves and socks" distribution. Extracutaneous manifestations are usually mild and transient. We report a 42-year-old female with a highly unusual expression of the syndrome, including bullous lesions, lingual aphthae, and conjunctivitis, accompanied by arthritis and a high-grade fever. She had immunoglobulin M and immunoglobulin G antibodies to parvovirus B19, which was detected from lesional skin with the use of polymerase chain reaction for the first time in such a clinical constellation. Because parvovirus B19 infections are generally more severe in adults, we suspect that the unusual disease expression in our patient was related to being more than 40 years of age. collapse abstract

Foreign body granuloma due to Matridex injection for cosmetic purposes.

A new resorbable filler, Matridex, became commercially available during the last years with scarce evidence regarding side effects. A 43-year-old woman complained of multiple, painful, reddish, nonulcerated, hard nodules on both cheeks and periocular... expand abstract regions. Four weeks before, she had been injected by a general practitioner with Matridex for aesthetic purposes to correct wrinkles in the same areas of the nodular eruption. Histopathology showed a diffuse suppurative granulomatous reaction with the presence of multinucleate giant cells and many neutrophils involving the entire dermis. No areas of caseation were observed. The inflammatory granulomatous reaction surrounded 2 different types of nonpolarizing, bluish, exogenous material: one arranged in filamentous structures and the second composed by large spherical particles. All nodules were incised and drained; the patient received systemic antibiotic treatment for 2 consecutive weeks. The nodules progressively regressed and almost complete resolution was seen after 6 months. Matridex is a new resorbable filler constituted by a mixture of nonanimal-stabilized hyaluronic acid (HA), cross-linked HA, and dextranomer microspheres. Foreign body reactions have been described in association with other HA fillers, but a granulomatous reaction after the injection of Matridex has not been reported yet. Interestingly, in our patient, we were able to identify both fragments of HA: the filamentous particles and the spherical particles of dextranomer microspheres within the infiltrate, these last giving a characteristic and recognizable appearance to the histopathological picture. collapse abstract

Papules arising after radiotherapy for rhabdomyosarcoma.

Radiation therapy, even at low doses, can induce a wide spectrum of vascular skin proliferations ranging from nonmalignant ones, such as benign lymphangiomatous papules (BLAP), to frankly malignant pathologies, such as angiosarcoma. We describe a 50-... expand abstractyear-old Caucasian woman with a past history of uterine rhabdomyosarcoma, treated 22 years prior with surgical excision, chemotherapy, and radiotherapy. She presented with a few skin-colored papules and a clear discharge located in the previously irradiated area (right inguinal region). Histopathology showed a proliferation of irregular, interanastomosing vascular channels, thin walled and lined by prominent endothelial cells with focally hobnail features. Cytological atypia of endothelial cells, mitotic figures, hemorrhagic areas, and necrosis were not observed. The endothelial cells expressed D2-40 and CD31. A diagnosis of BLAP following radiotherapy for uterine rhabdomyosarcoma was made. The patient was treated with complete excision using electrodessication. At the 20-month follow-up visit the patient was still free of recurrence. collapse abstract

Thrombocytopenia associated with the use of anti-tumor necrosis factor-alpha agents for psoriasis.

BACKGROUND: Thrombocytopenia has been reported to be associated with efalizumab therapy, but has only sporadically been reported with other anti-tumor necrosis factor alfa (TNF-alpha) agents. OBJECTIVE: To describe the frequency of thrombocytopenia i... expand abstractn a cohort of patients who underwent biological therapies for psoriasis. METHODS: This was a retrospective observational study of 93 patients. RESULTS: One hundred eighteen courses of biological therapies were administered to 93 patients. Four of 67 patients who received anti-TNF-alpha agents developed drug-induced thrombocytopenia during treatment, compared with none of the 51 patients receiving efalizumab therapy. The platelet count recovered after suspension of anti-TNF-alpha agents in 3 patients and relapsed after re-exposure in two patients. The overall estimated frequency of thrombocytopenia in our cohort was 4.30% (95% confidence interval [CI], 0% to 6.2%). LIMITATIONS: These findings should be validated in larger studies. CONCLUSIONS: Drug-induced thrombocytopenia is a potential side effect of anti-TNF-alpha agents. Immediate monitoring of platelet counts is recommended if autoimmunity is suspected. collapse abstract

Laser for the treatment of granular parakeratosis.

Clinical and dermoscopic features of porokeratosis of Mibelli.

Clinical images. Sweet syndrome during pregnancy.