Pathogenesis of Leprosy: An Insight Into B Lymphocytes and Plasma Cells.

The pathogenesis of leprosy is still not fully understood. Several studies have been performed on the involvement of T cells in leprosy and more recently have focused on genetic factors and innate immune response. There are still only few reports about the role of B cells in active leprosy lesions in different spectral forms of the disease. The literature on tuberculosis suggests that B cells play an important role in the regulation of the granulomas, in cytokine production, T-cell response, and antigen presentation. Only few studies investigated the role of B cell in leprosy. We investigated the distribution of B cells in 85 leprosy biopsies covering all forms of the disease and compared results with 13 biopsies of tuberculosis and atypical mycobacteriosis, expanding the previous experiences. A statistically significant difference in the number of CD20 (P = 0.014) and CD138+ (P = 0.01) cells between the different forms of leprosy was observed. A remarkable amount of CD138+ cells could also be detected in borderline tuberculoid. The median of the CD20 cells decreased from the bacilloscopy-negative samples to the bacilloscopy-positive samples by 50% (P = 0.004). Contrarily, the median of CD138+ cells showed an increase from bacilloscopy-negative to bacilloscopy-positive samples of 966.67% (P = 0.001). In our experience, tuberculoid leprosy showed more B cells and less plasma cells than lepromatous leprosy. Our results show that B cells might be implicated in leprosy pathogenesis, not only in the lepromatous pole as previously postulated, but also in tuberculoid granuloma formation and type 1 reactions.

Leprosy and Lobomycosis: First report from the Amazon Region.

Leprosy is still a relevant health problem in Brazil with 31 044 new cases diagnosed in 2013, of which 781 new cases diagnosed in the State of Amazonas. Lobomycosis is a cutaneous-subcutaneous mycosis caused by Lacazia loboi, an in vitro uncultivable fungus. Lobomycosis has been mainly reported in the Amazon region of Brazil and Colombia affecting mainly male farmers and workers in extraction of rubber. Lobomycosis is clinically characterised by keloid-like lesions and chronic evolution. Even if lobomycosis does not represent a major public health problem, it remains a serious condition for patients due to unsatisfactory treatment. We report a case of an old man with lepromatous leprosy diagnosed in 1983, treated with multidrug therapy until 1989 and presenting a leprosy relapse 15 years after treatment. At this time a lobomycosis was also diagnosed in a keloid-like lesion evolving for more than 30 years. This co-infection has been only rarely reported and this is the first detailed case report in the English literature.

OFLOXACIN multicentre trial in MB leprosy FUAM-Manaus and ILSL-Bauru, Brazil.

UNLABELLED: Recently antimicrobials of the fluoroquinolone class (pefloxacin and ofloxacin) were found far more effective against Mycobacterium leprae in studies with both mice and patients than dapsone and clofazimine. As multicentre trial participants, we evaluated the therapeutic efficacy, in terms of rate of relapse, of two new multidrug regimens containing ofloxacin, comparing them to 1 year and 2 years of standard WHO-MDT regimen in multibacillary (MB) leprosy patients. A total of 198MB patients were recruited to participate in a randomized, double-blind trial. Among those, 53 patients were treated with 1 year of WHO-MDT (a regimen including dapsone, clofazimine, and rifampin), 55 patients received 1 year of WHO-MDT plus an initial 1 month of daily ofloxacin, 63 patients were treated with 1 month of daily rifampin and daily ofloxacin, whereas 27 were treated with 2 years of WHO-MDT. Patients were regularly monitored for signs of relapse, in at least 7 years follow-up after being released from treatment. RESULTS: Relapse occurred in those treated with 1-month regimen alone at a significant higher rate (P < 0.001): 388%, whereas in the other three regimens that included WHO-MDT it ranged from 0 to 5%. This study found that a short-course treatment for MB patients with rifampicin-ofloxacin combination had a higher failure rate. The addition of one month of daily ofloxacin to 12 months MB WHO-MDT did not increase its efficacy.

Necrotic erythema nodosum reaction associated with histological alterations of Lucio's phenomenon.

Patients with lepromatous or borderline leprosy may present two types of vasculonecrotic reactions: Lucio's phenomenon (LP) and necrotic erythema nodosum leprosum (nENL). These are serious conditions, which mostly lead to life-threatening infectious and thrombotic complications. The authors report the case of a patient with leprosy recurrence associated with an atypical type II reaction with LP characteristics on histopathology.

Genital ulcers caused by sexually transmitted agents.

Genital ulcers (GUs) represent a diagnostic challenge and can be secondary to neoplastic and inflammatory processes of different causes. Among those of infectious etiology, there are sexually transmitted infections (STIs), a very frequent reason for seeking the health service. The most common agents are herpes simplex virus and Treponema pallidum and, more rarely, Haemophilus ducreyi, Klebsiella granulomatis and Chlamydia trachomatis. A careful dermatological examination offers important diagnostic elements; however, atypical manifestations are very common. Distinctive characteristics of ulcers to look out for include their margin, edge, bottom, and base. Regional lymph node chain alterations should be evaluated regarding their number, size, mobility, consistency, inflammation, and pain on palpation. Diagnostic tests have variable sensitivity and specificity, and molecular tests are currently considered the reference exams. The rapid immunochromatographic tests represented a significant advance, as they can be performed with blood obtained from the digital pulp, offer results in up to 30 minutes, and do not require a laboratory structure. The treatment of persons affected by GU/STIs must be immediate, as it aims to prevent complications, as well as reduce transmission. It is not always considered that people with GUs/STIs have varying degrees of depression, anxiety, and self-reproach, with an impact on relationships. Establishing a bond and trusting the professional is essential for adherence to treatment and preventive measures that must be discussed individually.

Subcutaneous phaeohyphomycosis in immunocompetent patients: two new cases caused by Exophiala jeanselmei and Cladophialophora carrionii.

Phaeohyphomycosis is a distinct mycotic infection of the skin or internal organs caused by darkly pigmented (dematiaceous) fungi, which are widely distributed in the environment. Phaeohyphomycosis is most frequently an opportunistic infection in immunosuppressed patients (HIV, corticotherapy, transplant patients) or is frequently associated with chronic diseases and diabetes. The spectrum of the disease is broad and includes superficial infections, onychomycosis, subcutaneous infections, keratitis, allergic disease, pneumonia, brain abscesses and disseminated disease. Rarely, immunocompetent patients may be affected. We describe two new cases of subcutaneous phaeohyphomycosis in immunocompetent patients: in the first patient, the causative agent was Exophiala jeanselmei, a common cause of phaeohyphomycosis; and in the second, Cladophialophora carrionii, which could be identified by culture. Cladophialophora carrionii is mainly the aetiological agent of chromoblastomycosis and only rarely the cause of phaeohyphomycosis. The first patient was treated with surgical excision and oral itraconazole, and the second patient responded to oral itraconazole only. Lesions improved in both patients and no recurrence was observed at follow-up visits.

Psoriasiform syphilis: A challenge.

Syphilis is one of the greatest masqueraders in dermatology, like mycosis fungoides or sarcoidosis. In fact, secondary syphilis can simulate different dermatological conditions, not only clinically but also histopathologically. A 34-year-old Brazilian man from Manaus (Amazonas) attended our Department complaining for 2 weeks of asthenia, weight loss and multiple asymptomatic disseminated erythematous papules and scaly plaques with well-defined borders and a tendency to confluence were observed on the trunk, neck and extremities, clinically similar to psoriasis guttata. The skin biopsy and laboratory tests disclosed secondary syphilis and the patient was treated with intramuscular benzathine penicillin with prednisolone, with a complete resolution. Psoriasiform syphilis, which imitates psoriasis, was well known by old dermatologists and was called syphilide psoriasiforme or psoriasis syphilitique. Psoriasiform syphilis is one of the atypical presentations of secondary syphilis and dermatologists should be aware of psoriasiform syphilis in order not to improperly treat syphilis patients with immunosuppressive drugs; also, pathologists should be aware of secondary syphilis histopathological features.

Case for diagnosis. Erythematous and infiltrated plaques in the infrahyoid region.

Leprosy is a chronic infectious disease caused by Mycobacterium leprae and, depending on the host immune status, presents different clinical forms. This report describes the case of a 46-year-old man who had hypoesthetic lesions in the infrahyoid region for 30 days. The bacilloscopy was negative. The anatomopathological examination showed alterations corresponding to the tuberculoid pole (epithelioid histiocytes) and virchowian pole (foamy histiocytes), compatible with borderline-virchowian leprosy (Ridley and Jopling classification). Rapid tests for HIV I, II, and syphilis were positive, with a CD4 count of 223. The patient started treatment with multibacillary multidrug therapy, antiretroviral therapy, and benzathine penicillin, with marked clinical improvement in two months.

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-regs) 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.

Osteonevus of Nanta: a case report in a combined melanocytic nevus.

Secondary osteoma cutis is a phenomenon that may occur in several conditions. When it occurs in a melanocytic nevus it is named osteonevus of Nanta, an event considered uncommon and characterized by the presence of bone formation adjacent or interposed with melanocytic cells. There are reports of its occurrence in various melanocytic lesions, being more frequently associated with intradermal nevus. We report a case of osteonevus of Nanta in combined nevus, possibly the first description of this association.

Subcutaneous phaeohyphomycosis due to Cladophialophora bantiana: a first case report in an immunocompetent patient in Latin America and a brief literature review.

We report a rare case of subcutaneous phaeohyphomycosis caused by Cladophialophora bantiana in an immunocompetent patient in Amazonas, Brazil. This dematiaceous fungus has been mainly associated with life-threatening infections affecting the central nervous systems of immunosuppressed patients. We present the clinical, laboratory, and therapeutic aspects, and in vitro susceptibility test results for different antifungal drugs. A brief review of the cases reported in the literature over the past 20 years has also been discussed. According to the literature review, the present case is the first report of subcutaneous phaeohyphomycosis due to C. bantiana in an immunocompetent patient in Latin America.

Case for diagnosis. Atypical genital lesion.

We present a case of a penile lesion with a clinical appearance similar to Mondor penile disease (thrombosis of the dorsal vein of the penis) or penile sclerosing lymphangitis. Laboratory evaluation, however, showed a solid lesion, with no vascular component to Doppler ultrasonography and no treponema to immunohistochemistry. Histological and serological tests were compatible with secondary syphilis. The authors reinforce the need for the inclusion of syphilis in the differential diagnosis of penile cord injuries.

Atypical crusted scabies in a patient with chronic liver disease caused by hepatitis B and D viruses.

Crusted scabies is a less common variant of scabies that is highly contagious, difficult to treat and involves infestation by Sarcoptes scabiei var. hominis. The classical clinical presentation includes crusted, scaly and generally non-pruritic lesions usually located on the head, neck, palmar, plantar and periungual region. It was first described in Norway in 1848 in patients with leprosy who presented with crusted lesions. In this study, we report the case of a patient with crusted scabies with florid clinical manifestations and chronic liver disease due to hepatitis B and delta virus infection.

Pigmented squamous cell carcinoma: case report and importance of differential diagnosis.

A few cases of pigmented squamous cell carcinoma affecting the skin and the ocular and oral mucosa of the elderly have been described in the literature. The disease manifests itself as papular and nodular erythematous or pigmented lesions. The main clinical differential diagnoses are pigmented basal cell carcinoma and melanoma. Histopathological examination is characterized by proliferation of atypical squamous cells with formation of horn pearls permeated by dendritic melanocytes. For this reason, a careful diagnosis is necessary to rule out other tumors that show melanin pigment. Surgical removal is the indicated treatment and the prognosis is similar to the classical squamous cell carcinoma. We report a case of pigmented squamous cell carcinoma with diagnosis confirmed by histological and immunohistochemical examinations.

Chronic tophaceous gout in patients with psoriasis.

Psoriasis is a chronic inflammatory disease of multifactorial etiology influenced by genetic, immunological, and environmental factors. We report the case of a patient with psoriasis for more than 25 years who developed hyperuricemia and chronic tophaceous gout with unusual appearance. In psoriasis, hyperuricemia may occur by increased epidermal cell turnover, which accelerates purine metabolism and has uric acid as the product of its catabolism. The association of psoriasis with hyperuricemia can trigger the onset of gouty arthritis, and pose a greater risk of developing other inflammatory comorbidities. Therefore, it is important to periodically investigate uric acid levels in order to treat changes triggered by hyperuricemia.

Necrotic erythema nodosum reaction associated with histological alterations of Lucio's phenomenon

Abstract Patients with lepromatous or borderline leprosy may present two types of vasculonecrotic reactions: Lucio's phenomenon (LP) and necrotic erythema nodosum leprosum (nENL). These are serious conditions, which mostly lead to life-threatening infectious and thrombotic complications. The authors report the case of a patient with leprosy recurrence associated with an atypical type II reaction with LP characteristics on histopathology.

Genital ulcers caused by sexually transmitted agents,

Abstract Genital ulcers (GUs) represent a diagnostic challenge and can be secondary to neoplastic and inflammatory processes of different causes. Among those of infectious etiology, there are sexually transmitted infections (STIs), a very frequent reason for seeking the health service. The most common agents are herpes simplex virus and Treponema pallidum and, more rarely, Haemophilus ducreyi, Klebsiella granulomatis and Chlamydia trachomatis. A careful dermatological examination offers important diagnostic elements; however, atypical manifestations are very common. Distinctive characteristics of ulcers to look out for include their margin, edge, bottom, and base. Regional lymph node chain alterations should be evaluated regarding their number, size, mobility, consistency, inflammation, and pain on palpation. Diagnostic tests have variable sensitivity and specificity, and molecular tests are currently considered the reference exams. The rapid immunochromatographic tests represented a significant advance, as they can be performed with blood obtained from the digital pulp, offer results in up to 30 minutes, and do not require a laboratory structure. The treatment of persons affected by GU/STIs must be immediate, as it aims to prevent complications, as well as reduce transmission. It is not always considered that people with GUs/STIs have varying degrees of depression, anxiety, and self-reproach, with an impact on relationships. Establishing a bond and trusting the professional is essential for adherence to treatment and preventive measures that must be discussed individually.

Rosacea fulminans: unusual clinical presentation of rosacea.

Rosacea fulminans or pyoderma faciale is a rare cutaneous disorder that usually affects women usually between the ages of 15-46. The disease is characterized by sudden onset of papules, pustules, cysts, and painful coalescing nodules with red-cyanotic centrofacial erythema. Although its etiology remains unknown, hormonal, immunological, and vascular factors have been reported. Early diagnosis and prompt treatment should minimize unsightly scars. We report a case of a 33-year-old female patient treated with traditional doses of doxycycline, with improvement of the lesions and regression of the condition in two months.

Case for diagnosis. Erythematous and infiltrated plaques in the infrahyoid region

Abstract Leprosy is a chronic infectious disease caused by Mycobacterium leprae and, depending on the host immune status, presents different clinical forms. This report describes the case of a 46-year-old man who had hypoesthetic lesions in the infrahyoid region for 30 days. The bacilloscopy was negative. The anatomopathological examination showed alterations corresponding to the tuberculoid pole (epithelioid histiocytes) and virchowian pole (foamy histiocytes), compatible with borderline-virchowian leprosy (Ridley and Jopling classification). Rapid tests for HIV I, II, and syphilis were positive, with a CD4 count of 223. The patient started treatment with multibacillary multidrug therapy, antiretroviral therapy, and benzathine penicillin, with marked clinical improvement in two months.

Generalized pustular psoriasis induced by systemic steroid dose reduction.

Generalized pustular psoriasis, or psoriasis of von Zumbusch, is an acute and severe clinical form of psoriasis, which usually occurs in patients with psoriasis undergoing aggravating factors. In this work, we report the case of a female patient, 70 years old, who developed generalized pustular psoriasis symptoms while reducing the dose of oral corticosteroids, improperly introduced for the treatment of alleged acute generalized exanthematous pustulosis. The differential diagnosis of generalized pustular psoriasis should be made with other pustular dermatoses, such as subcorneal pustulosis, IgA pemphigus and especially with acute generalized exanthematous pustulosis. Personal history of psoriasis and histopathological findings with psoriasiform changes and subcorneal pustule favored the diagnosis. She was treated with acitretin 30 mg / day, progressing to complete regression of the lesions.