Leishmaniasis recidivans in Ethiopia: cutaneous and mucocutaneous features.

Cutaneous leishmaniasis (CL) is endemic in Ethiopia. An unusual clinical form of this disease is leishmaniasis recidivans (LR), a prolonged, relapsing form of cutaneous leishmaniasis resembling tuberculosis of the skin that may persist for many years with a chronic and relapsing course. This rare variant has been shown to be caused by Leishmania tropica species in the Old World and by Leishmania braziliensis, Leishmania amazonensis, Leishmania panamensis, and Leishmania guyanensis in the New World, as reported in various studies. To our knowledge, there are no reports from Ethiopia, and mucocutaneous involvement of LR has not been described to date. This was a retrospective analysis of the patients seen at the Italian Dermatological Center in Mekelle on the Tigrean highlands over a three-year period (2008-2011). Seven patients with typical clinical features of LR were seen. Two of them presented with signs of mucosal involvement. To date, Leishmania aethiopica is shown to be the only species causing CL that is endemic in the Ethiopian highlands. Therefore, it had to be assumed that the lesions in these patients were caused by this species. The aims of this communication are to report, for the first time, the presence of LR, most likely due to Leishmania aethiopica, in Ethiopia, and to report mucosal involvement in this rare clinical form of CL.

Leprosy type 1 reaction (formerly reversal reaction).

Nerve damage leading to impairment and permanent disability is the major problem in the course of a leprosy infection. Most of the damage occurs during two types of leprosy reactions, type 1 reaction (T1R) and type 2 reaction (T2R). Timely and adequate treatment may prevent this damage. Particular T1R reactions, however, are often diagnosed too late and are even missed. Clinical symptoms and warning signs are therefore covered, as are the immunology and pathophysiology of nerve damage. The differences between upgrading and downgrading, old terms but still relevant, are explained. Methods to detect reactions and to monitor their treatment are given. Triggering factors, the mechanisms of the reactions, including autoimmunity, and the presence of physical compression are discussed. Treatment over the years is placed in its context, and based on this information a treatment schedule is recommended.

Leprosy type 1 reaction (formerly reversal reaction)

Nerve damage leading to impairment and permanent disability is the major problem in the course of a leprosy infection. Most of the damage occurs during two types of leprosy reactions, type 1 reaction (T1R) and type 2 reaction (T2R). Timely and adequate treatment may prevent this damage. Particular T1R reactions, however, are often diagnosed too late and are even missed. Clinical symptoms and warning signs are therefore covered, as are the immunology and pathophysiology of nerve damage. The differences between upgrading and downgrading, old terms but still relevant, are explained. Methods to detect reactions and to monitor their treatment are given. Triggering factors, the mechanisms of the reactions, including autoimmunity, and the presence of physical compression are discussed. Treatment over the years is placed in its context, and based on this information a treatment schedule is recommended.

Post-kala-azar dermal leishmaniasis and leprosy: case report and literature review.

BACKGROUND: Post-kala-azar dermal leishmaniasis (PKDL) is a dermal complication of visceral leishmaniasis (VL), which may occur after or during treatment. It has been frequently reported from India and the Sudan, but its occurrence in South America has been rarely reported. It may mimic leprosy and its differentiation may be difficult, since both diseases may show hypo-pigmented macular lesions as clinical presentation and neural involvement in histopathological investigations. The co-infection of leprosy and VL has been reported in countries where both diseases are endemic. The authors report a co-infection case of leprosy and VL, which evolved into PKDL and discuss the clinical and the pathological aspects in the patient and review the literature on this disease. CASE PRESENTATION: We report an unusual case of a 53-year-old female patient from Alagoas, Brazil. She presented with leprosy and a necrotizing erythema nodosum, a type II leprosy reaction, about 3 month after finishing the treatment (MDT-MB) for leprosy. She was hospitalized and VL was diagnosed at that time and she was successfully treated with liposomal amphotericin B. After 6 months, she developed a few hypo-pigmented papules on her forehead. A granulomatous inflammatory infiltrate throughout the dermis was observed at histopathological examination of the skin biopsy. It consisted of epithelioid histiocytes, lymphocytes and plasma cells with the presence of amastigotes of Leishmania in macrophages (Leishman's bodies). The diagnosis of post-kala-azar dermal leishmaniasis was established because at this time there was no hepatosplenomegaly and the bone marrow did not show Leishmania parasites thus excluding VL. About 2 years after the treatment of PKDL with liposomal amphotericin B the patient is still without PKDL lesions. CONCLUSION: Post-kala-azar dermal leishmaniasis is a rare dermal complication of VL that mimics leprosy and should be considered particularly in countries where both diseases are endemic. A co-infection must be seriously considered, especially in patients who are non-responsive to treatment or develop persistent leprosy reactions as those encountered in the patient reported here.

High frequency of symptomatic zinc deficiency in infants in northern ethiopia.

Background. Zinc deficiency occurs in infants when its demand exceeds its supply. It presents with cutaneous signs which, in severe cases, are associated with diarrhea, alopecia, and irritability. Genetic and acquired forms of zinc deficiency have been reported and often overlap clinical features. Malnutrition, prematurity, malabsorption syndromes, and burns may cause an increased demand for zinc. Methods. Cases of acquired transient infantile zinc deficiency (TIZD) observed during a period of 3 years at Ayder Referral Hospital of Mekelle, Northern Ethiopia, are reported here. Since no sophisticated tests were available at our center, the diagnosis was based on the clinical signs and prompt response to oral zinc supplementation. Results. We observed 18 cases of TIZD at our center. All patients were full-term and breastfeeding infants with no relevant associated diseases. Conclusions. In this region, a high incidence of this condition is observed. We could not rule out whether heterozygosity for the genetic mutation was present or that the disease was caused by a nutritional deficiency in the mothers or more probably because both the factors coexisted together. However, further studies are necessary to better understand the causes of the increased incidence of this disease in Northern Ethiopia.

Quality of life of patients with vitiligo attending the Regional Dermatology Training Center in Northern Tanzania.

BACKGROUND: Vitiligo is an acquired, predominantly asymptomatic, depigmenting disorder with profound psychological effects. METHODS: This was a cross-sectional study conducted at the Regional Dermatology Training Center in Moshi, Tanzania. All 88 patients with vitiligo older than 15 years of age who attended the skin clinic from October 2009 to April 2010 were recruited. Data were collected using a structured questionnaire, Dermatology Life Quality Index questionnaire (DLQI), and Vitiligo European Task Force form. RESULTS: Vitiligo moderately affects patient's quality of life, as indicated by a DLQI mean score of 7.2 ± 4.8. The mean age was 41 years with a male/female ratio of 1:1.7. The mean age of disease onset was 33.5 years (range 16-83 years); vitiligo vulgaris was the most common disease form seen (n = 49). None of the factors considered were found to be significantly associated with impaired quality of life on multivariate analysis. The majority of patients (73.8%) perceived that their disease was moderate to severe in contrast to the clinical grading in which only 49.2% patients were classified as having mild disease. This difference in classification of disease severity was statistically significant (Fishers exact test = 0.001). CONCLUSION: Patients with vitiligo of African descent have a moderate impairment of quality of life.

Cutaneous and mucocutaneous leishmaniasis resembling borderline-tuberculoid leprosy: a new clinical presentation?

Both cutaneous and mucocutaneous leishmaniasis are endemic in Northern Ethiopia. The different clinical presentations depend on the responsible organism and the host's immune response. Localized cutaneous leishmaniasis is the type most frequently seen. Diffuse cutaneous leishmaniasis is relatively rare and usually associated with mucous membrane involvement. Diffuse cutaneous leishmaniasis presents with multiple lesions, can be difficult to diagnose and responds less favourably to treatment. We report here 2 patients with unusual presentations of diffuse cutaneous leishmaniasis presenting with large hypopigmented skin lesions mimicking borderline-tuberculoid leprosy. To our knowledge this presentation has not been described before and may present difficulties in making a definite diagnosis in regions where both leprosy and cutaneous leishmaniasis are endemic. Lepromatous leprosy and diffuse cutaneous leishmaniasis are regularly confused, particularly when no skin smears for acid-fast bacillus or Leishman-Donovan bodies are performed.

Leprosy and tuberculosis co-infection: clinical and immunological report of two cases and review of the literature.

A review of the records of patients seen between 2004 and 2011 at the Dermatology Clinic of the São Paulo University Medical School showed that only two leprosy patients had been co-infected with tuberculosis (TB). One patient showed a type 1 leprosy reaction during the first 3 months of treatment of pleural TB and in the other patient, pulmonary TB was diagnosed during the first 3 months of treatment of a type 1 leprosy reaction. Both patients showed normal cellular immune response tests, including those of the interferon-gamma (IFN-γ)/interleukin 12 (IL-12) axis. Although both mycobacterial infections are endemic in developing countries like Brazil, the co-infection has hardly been reported in the last decade. There is no suitable explanation for this observation. The reports on the interaction between the two mycobacteria are highly speculative: some studies suggest that leprosy, especially the anergic form, would predispose to TB, whereas other investigations suggested an antagonism between the two diseases.

Skin flora: Differences between people affected by Albinism and those with normally pigmented skin in Northern Tanzania - cross sectional study.

BACKGROUND: Skin flora varies from one site of the body to another. Individual's health, age and gender determine the type and the density of skin flora. METHODS: A 1 cm² of the skin on the sternum was rubbed with sterile cotton swab socked in 0.9% normal saline and plated on blood agar. This was cultured at 35 °C. The bacteria were identified by culturing on MacConkey agar, coagulase test, catalase test and gram staining. Swabs were obtained from 66 individuals affected by albinism and 31 individuals with normal skin pigmentation. Those with normal skin were either relatives or staying with the individuals affected by albinism who were recruited for the study. RESULTS: The mean age of the 97 recruited individuals was 30.6 (SD ± 14.9) years. The mean of the colony forming units was 1580.5 per cm2. Those affected by albinism had a significantly higher mean colony forming units (1680 CFU per cm²) as compared with 453.5 CFU per cm² in those with normally pigmented skin (p = 0.023). The skin type and the severity of sun- damaged skin was significantly associated with a higher number of colony forming units (p = 0.038). CONCLUSION: Individuals affected by albinism have a higher number of colony forming units which is associated with sun- damaged skin.

Acral peeling skin syndrome in two East-African siblings: case report.

BACKGROUND: Acral peeling skin syndrome is a rare autosomal recessive genodermatosis due to a missense mutation in transglutaminase 5. The skin peeling occurs at the separation of the stratum corneum from the stratum granulosum. CASE PRESENTATION: We present a case of two siblings who developed continuous peeling of the palms and soles from the first year of life. This peeling was more severe on the soles than palms and on younger sibling than elder sibling. Peeling is worsened by occlusion and sweating. CONCLUSIONS: Sporadic cases of Acral Peeling Skin Syndrome occur in African population. There is variability in time of presentation and clinical severity even within families.

Association between neuropathic pain and A-waves in leprosy patients with type 1 and 2 reactions.

Neuropathic pain (NP) is a well-recognized feature of leprosy neuropathy. However, the diagnosis of NP is difficult using only clinical criteria. In the study reported here, by means of conventional nerve conduction studies, the authors sought for an association between long-latency responses and NP complaints in leprosy patients with type 1 and 2 reactions. Of the 27 ulnar nerves of leprosy patients, 18 with type 1 reaction (T1R) and 9 with type 2 reaction (T2R) were followed-up for 6 months before and after steroid treatment. Clinical characteristics of pain complaints and clinical function were assessed, as well as the presence of F- and A-waves of the ulnar nerve using nerve conduction studies. The clinical and the neurophysiologic findings were compared to note positive concordances (presence of NP and A-waves together) and negative concordances (absence of NP and A-waves together) before and after treatment. Both reactions presented a high frequency of A-waves (61.1% in T1R and 66.7% in T2R, P < 0.05) and prolonged F-waves (69.4% in T1R and 65.8% in T2R, P = 0.4). No concordances were seen between pain complaints and F-waves. However, significant concordances between NP and A-waves were observed, although restricted to the T2R group (χ(2) = 5.65, P = 0.04). After treatment, there was a significant reduction in pain complaints, as well as the presence of F- and A-waves in both groups (P < 0.05 for all comparisons). In conclusion, the presence of A-waves correlates well with pain complaints of neuropathic characteristics in leprosy patients, especially in those with type 2 reaction. Probably, such response shares similar mechanisms with the small-fiber dysfunction seen in these patients with NP, such as demyelination, intraneural edema, and axonal sprouting. Further studies using specific tools for small-fiber assessment are warranted to confirm our findings.

Leprosy and HIV coinfection: a critical approach.

An increase in leprosy among HIV patients, similar to that observed in patients with TB, was expected approximately 20 years ago. Studies conducted in the 1990s together with those reported recently seemed to indicate that a coinfection with HIV did not alter the incidence and the clinical spectrum of leprosy and that each disease progressed as a single infection. By contrast, in countries with a high seroprevalence of HIV, TB was noted to increase. Explanations may be provided by the differences in the incubation time, the biology and toxicity of Mycobacterium leprae and Mycobacterium tuberculosis. After the introduction of HAART the leprosy-HIV coinfection manifested itself as an immune reconstitution inflammatory syndrome (IRIS), typically as paucibacillary leprosy with type 1 leprosy reaction. The incidence of leprosy in HIV-infected patients has never been properly investigated. IRIS-leprosy is probably underestimated and recent data showed that the incidence of leprosy in HIV patients under HAART was higher than previously thought.

Association between neuropathic pain and A-Waves in leprosy patients with type 1 and 2 reactions

Neuropathic pain (NP) is a well-recognized feature of leprosy neuropathy. However, the diagnosis of NP is difficult using only clinical criteria. In the study reported here, by means of conventional nerve conduction studies, the authors sought for an association between long-latency responses and NP complaints in leprosy patients with type 1 and 2 reactions. Of the 27 ulnar nerves of leprosy patients, 18 with type 1 reaction (T1R) and 9 with type 2 reaction (T2R) were followed-up for 6 months before and after steroid treatment. Clinical characteristics of pain complaints and clinical function were assessed, as well as the presence of F- and A-waves of the ulnar nerve using nerve conduction studies. The clinical and the neurophysiologic findings were compared to note positive concordances (presence of NP and A-waves together) and negative concordances (absence of NP and A-waves together) before and after treatment. Both reactions presented a high frequency of A-waves (61.1% in T1R and 66.7% in T2R, P < 0.05) and prolonged F-waves (69.4% in T1R and 65.8% in T2R, P = 0.4). No concordances were seen between pain complaints and F-waves. However, significant concordances between NP and A-waves were observed, although restricted to the T2R group (χ(2) = 5.65, P = 0.04). After treatment, there was a significant reduction in pain complaints, as well as the presence of F- and A-waves in both groups (P < 0.05 for all comparisons). In conclusion, the presence of A-waves correlates well with pain complaints of neuropathic characteristics in leprosy patients, especially in those with type 2 reaction. Probably, such response shares similar mechanisms with the small-fiber dysfunction seen in these patients with NP, such as demyelination, intraneural edema, and axonal sprouting. Further studies using specific tools for small-fiber assessment are warranted to confirm our findings.

Enhancing sensitivity of detection of immune responses to Mycobacterium leprae peptides in whole-blood assays.

Although worldwide leprosy prevalence has been reduced considerably following multidrug therapy, new case detection rates remain relatively stable, suggesting that transmission of infection still continues. This calls for new efforts, among which is development of assays that can identify subclinical/early-stage Mycobacterium leprae-infected subjects, a likely source of transmission. Areas in which leprosy is endemic often lack sophisticated laboratories, necessitating development of field-friendly immunodiagnostic tests for leprosy, like short-term whole-blood assays (WBA). In classical, peripheral blood mononuclear cell (PBMC)-based gamma interferon (IFN-gamma) release assays, M. leprae peptides have been shown to discriminate in a more specific fashion than M. leprae proteins between M. leprae-exposed contacts and patients as opposed to healthy controls from the same area of endemicity. However, peptides induced significantly lower levels of IFN-gamma than did proteins, particularly when whole blood was used. Therefore, possibilities of specifically enhancing IFN-gamma production in response to M. leprae peptides in 24-h WBA were sought by addition of various cytokines and antibodies or by mannosylation of peptides. In addition, other cytokines and chemokines were analyzed as potential biomarkers in WBA. We found that only interleukin 12 (IL-12), not other costimulants, increased IFN-gamma production in WBA while maintaining M. leprae peptide specificity, as evidenced by lack of increase of IFN-gamma in control samples stimulated with IL-12 alone. The IL-12-induced increase in IFN-gamma was mainly mediated by CD4+ T cells that did not produce IL-2 or tumor necrosis factor (TNF). Mannosylation further allowed the use of 100-fold-less peptide. Although not statistically significantly, macrophage inflammatory protein 1beta (MIP-1beta) and macrophage c protein 1 (MCP-1) levels specific for M. leprae peptide tended to be increased by IL-12. IP-10 production was also found to be a useful marker of M. leprae peptide responses, but its production was enhanced by IL-12 nonspecifically. We conclude that IFN-gamma-based WBA combined with IL-12 represents a more sensitive and robust assay for measuring reactivity to M. leprae peptides.

Rural dermatology in the tropics.

Rural dermatology in the tropics is determined by socioeconomic factors, climate, and skin type. Lack of interest of the profession leads to late and inadequate diagnosis and treatment. No proper data on the magnitude of the problem are available, even though a great number of the world population live under those conditions. This contribution provides an overview of the most common skin diseases, in particular, infections and infestations. Other skin conditions, both eczematous and immune-mediated, that are typical for the rural tropics also are discussed. Etiology, pathology, and treatments are presented against the socioeconomic background.

A randomized clinical trial of oral steroids for ulnar neuropathy in type 1 and type 2 leprosy reactions.

BACKGROUND: Steroids regimens in leprosy neuropathies are still controversial in botth types of reactions. METHOD: For this trial, 21 patients with ulnar neuropathy were selected from 163 leprosy patients, 12 with type 1 reaction (T1R) and nine with type 2 (T2R). One experimental group started with prednisone 2 mg/kg/day and the control group with 1 mg/kg/day. A clinical score based on tests for spontaneous pain, nerve palpation, sensory and muscle function was used. Neurophysiological evaluation consisted on the motor nerve conduction of the ulnar nerve in three segments. Student "t" test for statistical analysis was applied on the results: before treatment, first week, first month and sixth month, between each regimen and types of reaction. CONCLUSION: In both reactions during the first month higher doses of steroids produced better results but, earlier treatment with lower dose was as effective. Short periods of steroid, 1 mg/Kg/day at the beginning and,tapering to 0,5 mg/Kg/day or less in one month turned out to be efficient in T2R.

A randomized clinical trial of oral steroids for ulnar neuropathy in type 1 and type 2 leprosy reactions / Ensaio clínico sobre o tratamento com esteróides via oral da neuropatia ulnar em reação tipo 1 e tipo 2 da hanseníase

BACKGROUND: Steroids regimens in leprosy neuropathies are still controversial in botth types of reactions. METHOD: For this trial, 21 patients with ulnar neuropathy were selected from 163 leprosy patients, 12 with type 1 reaction (T1R) and nine with type 2 (T2R). One experimental group started with prednisone 2 mg/kg/day and the control group with 1 mg/kg/day. A clinical score based on tests for spontaneous pain, nerve palpation, sensory and muscle function was used. Neurophysiological evaluation consisted on the motor nerve conduction of the ulnar nerve in three segments. Student "t" test for statistical analysis was applied on the results: before treatment, first week, first month and sixth month, between each regimen and types of reaction. CONCLUSION: In both reactions during the first month higher doses of steroids produced better results but, earlier treatment with lower dose was as effective. Short periods of steroid, 1 mg/Kg/day at the beginning and,tapering to 0,5 mg/Kg/day or less in one month turned out to be efficient in T2R.

INTRODUÇÃO: O tratamento da neuropatia da hanseníase com esteróides é ainda controverso nos dois tipos de reações. MÉTODO: Neste ensaio, de 163 pacientes foram selecionados 21 com neuropatia ulnar, 12 com reação tipo 1 e 9 com tipo 2. Um grupo experimental iniciou com 2 mg/kg/dia e o grupo controle com 1 mg/kg/dia. Foi composto um escore clínico pela avaliação da sensação dolorosa espontânea, palpação de nervos e funções sensitiva e motora. Realizou-se a condução nervosa motora do nervo ulnar em três segmentos. Aplicaram-se os estudos estatísticos com o teste t de Student nos resultados: antes do tratamento, primeira semana, primeiro mês e sexto mês. CONCLUSÃO: Em ambas as reações dosagens mais elevadas iniciais produziram melhores resultados, mas a dose menor quando administrada precocemente foi igualmente efetiva. Períodos curtos com doses efetivas, 1 mg/Kg/dia no início e reduzindo-se para 0,5 mg/Kg/dia ou menos em um mês foram eficientes na reação tipo 2.