Acquired disorders with hypopigmentation: A clinical approach to diagnosis and treatment.

Acquired hypopigmented skin changes are commonly encountered by dermatologists. Although hypopigmentation is often asymptomatic and benign, occasional serious and disabling conditions present with cutaneous hypopigmentation. A thorough history and physical examination, centered on disease distribution and morphologic findings, can aid in delineating the causes of acquired hypopigmented disorders. The second article in this 2-part continuing medical education series focuses on conditions with a hypopigmented phenotype. Early diagnosis and appropriate management of these disorders can improve a patient's quality of life, halt disease progression, and prevent irreversible disability.

Leukemia cutis in a patient of relapsed leprosy- Coincidence or predisposition?

Leprosy, a disease of skin and peripheral nerves has varied manifestations which principally affect the immune status of the host. Leukemic skin infiltrations in patients with leukemia are referred to as leukemia cutis. It can be seen in all types of leukemia, especially in patients with acute myelomonocytic leukemia (AML). In majority of cases, the cutaneous lesions are nonspecific manifestations associated with an impaired immune system.1 Though various malignancies have been documented with leprosy, no case of borderline-tuberculoid (BT) Hansen's disease with coexisting leukemia cutis has ever been reported in literature to the best of our knowledge.

Role of CD8(+) T cells in triggering reversal reaction in HIV/leprosy patients.

It has been reported that the initiation of highly active anti-retroviral therapy (HAART) is associated with the development of reversal reaction (RR) in co-infected HIV/leprosy patients. Nevertheless, the impact of HIV and HAART on the cellular immune response to Mycobacterium leprae (ML) remains unknown. In the present study, we observed that ex vivo peripheral blood mononuclear cells (PBMCs) of both RR and RR/HIV patients presented increased percentages of activated CD4(+) T cells when compared with the healthy individuals (HC) group. The frequency of CD8(+)  CD38(+) cells increased in the PBMCs of RR/HIV patients but not in RR patients when compared with the HC group. Both RR and RR/HIV skin lesion cells presented similar percentages of activated CD4(+) cells, but the numbers of activated CD8(+) cells were higher in RR/HIV in comparison to the RR group. The frequency of interferon-γ-producing cells was high in response to ML regardless of HIV co-infection. In ML-stimulated cells, there was an increase in central memory CD4(+) T-cell frequencies in the RR and RR/HIV groups, but an increase in central memory CD8(+) T-cell frequency was only observed in the RR/HIV group. ML increased granzyme B(+) effector memory CD8(+) T-cell frequencies in the RR/HIV PBMCs, but not in the HC and RR groups. Our data suggest that the increased expression of effector memory CD8(+) T cells, together with greater perforin/granzyme B production, could be an additional mechanism leading to the advent of RR in co-infected patients. Moreoever, this increased expression may explain the severity of RR occurring in these patients.

[Malignant transformation of plantar ulcers in leprosy: experience of National Leprosy Center in Casablanca]. / Dégénérescence carcinomateuse des maux perforants plantaires d'origine lépreuse: expérience du Centre national de léprologie de Casablanca.

Malignant degeneration is a late complication of plantar ulcers in leprosy. The objective of this study is to describe and analyze the epidemiological, clinical, and therapeutic aspects of this complication in Morocco. A retrospective study was conducted from January 2000 to December 2009 at the National Center of Leprosy (CNL) in Casablanca. All our patients had a histological confirmation. Ten patients were included in this study. There were seven men and three women, with a mean age of 58.8 years. Six patients had a multibacillary form of leprosy and four had a paucibacillary form. The average duration of the plantar ulcers was 34.4 years. Clinical appearance at diagnosis was an ulcerative and vegetative tumor. Treatment was by radical amputation. Evolution was marked by metastatic spread in six patients. One patient died of disseminated disease. In Morocco, leprosy has been on the decline since 1990, but the occurrence of late complications in the leprosy patient as carcinoma of plantar ulcers persists. Prevention of these complications should be part of the national fight against leprosy.

Appropriately Selected Nerve in Suspected Leprous Neuropathy Yields High Positive Results for Mycobacterium leprae DNA by Polymerase Chain Reaction Method.

Identification of Mycobacterium leprae DNA by polymerase chain reaction (PCR) is a reliable and an affordable method to confirm leprosy. DNA from 87 nerve samples (61 from paraffin blocks and 26 fresh samples) was extracted. Mycobacterium leprae DNA was amplified by PCR from 80/87 (92%) specimens. Patients were seen over a period of 11 years (2007-2019), and leprosy was diagnosed based on clinical and characteristic histopathology findings. The clinical diagnostic possibilities were as follows: leprous neuropathy in 73/80 (91.3%), mononeuritis multiplex of unknown etiology in four (5.0%), vasculitic neuropathy in two (2.5%), and distal symmetric sensory motor neuropathy in one (1.3%). The biopsied nerves were as follows: superficial radial = 34 (42.6%), dorsal cutaneous branch of ulnar = 19 (23.8%), sural = 18 (22.5%), and superficial peroneal = 9 (11.3%), and corresponding neurological deficits were recorded in 77 (96.3%) cases. The histopathological diagnoses in total group were as follows: (borderline tuberculoid (BT) = 52, tuberculoid (TT) = 8, borderline lepromatous (BL) = 8, borderline borderline (BB) = 3, nonspecific inflammation = 3, healed/fibrosed = 4, and axonopathy = 2). Acid fast bacilli (AFB) was demonstrated in 11 (13.7%) samples. For comparison, 31 clinically and histopathologically defined non-leprous disease control nerves (inherited neuropathy = 20, vasculitis = 8, and nutritional neuropathy = 3) subjected to PCR were negative for M. leprae DNA. In most instances, there are multiple thickened peripheral nerves in suspected cases of leprosy, but neurological deficits pertaining to the thickened nerve are not as widespread. The current findings emphasize the importance of selecting the most appropriate nerve for biopsy to obtain a positive PCR result. We infer that clinical, histopathological, and PCR tests complement each other to help achieve a definitive diagnosis of leprosy particularly in pure neuritic leprosy and in leprous neuropathy with negative skin smears/biopsy.

Ocular morbidity in leprosy patients with lagophthalmos.

Lagophthalmos is a well known complication in leprosy due to the involvement of seventh cranial nerve resulting in incomplete closure of the eyelids. The real magnitude of ocular morbidity as a consequence of lagophthalmos is unknown, as several ocular complications can occur independently due to involvement of the fifth (trigeminal) nerve or due to secondary infection. Therefore, a study was designed to carefully examine the eyes of 100 consecutive leprosy patients with lagophthalmos seeking treatment at a leprosy referral centre in Delhi. Among the eyes examined, 145 had lagophthalmos. The symptomatology and anterior-posterior chamber morbidity in eyes with lagophthalmos were significantly higher as compared to unaffected eyes. Significantly, higher morbidity was seen regardless of sex or type of leprosy or deformity. Capacity building of the health professionals regarding ocular morbidity and increased emphasis on the importance of self care among patients can significantly reduce ocular morbidity.

Comparison of quality of life evaluated by SF-36 and DLQI in multibacillary and paucibacillary leprosy patients from Sao Paulo, Brazil.

BACKGROUND: Dermatological diseases have a negative impact on quality of life (QoL), affecting mental and physical health. Leprosy patients usually present with a worse QoL compared with those affected by other conditions. Reactions, neural damage, and pain are some of the consequences that contribute to the lower QoL. However, due to the wide spectrum of the disease, symptoms vary according to leprosy's subtype. This study aimed to compare the QoL between paucibacillary and multibacillary leprosy patients. Individuals were also compared considering the presence of reactions and a correlation between questionnaires was performed. METHODS: A total of 104 patients with leprosy aged 18 years old and over were selected. QoL was assessed by the Brazilian-Portuguese validated versions of the Medical Outcomes Study 36-item short-form health survey (SF-36) and the Dermatology Life Quality Life Index (DLQI). RESULTS: Multibacillary patients showed a more impaired physical function, worse bodily pain, lower score of SF-36, and higher interference of skin on the performance of daily activities when compared to the paucibacillary group. Individuals without reactions presented lower bodily pain and less effect of the skin on clothing choices compared to those with reactions. The SF-36 domains exhibited weak correlations with most DLQI questions, and the linear regression model showed that 32% of changes in QoL were related to the skin aspect. CONCLUSIONS: Multibacillary leprosy patients have a worse QoL when compared to paucibacillary patients. Reactions played a small role in the QoL of our cohort of patients.

Surgical treatment of type I neuritis in a teenage boy with borderline tuberculoid leprosy.

Exacerbation of the immune response against Mycobacterium leprae can lead to neuritis, which is commonly treated via immunosuppression with corticosteroids. Early neurolysis may be performed concurrently, especially in young patients with a risk of functional sequelae. We report the case of a young patient experienced intense pain in the left elbow one year after the treatment of tuberculoid-tuberculoid leprosy. The pain was associated with paresthesias in the ulnar edge and left ulnar claw. After evaluation, the diagnosis was changed to borderline tuberculoid leprosy accompanied with neuritis of the left ulnar nerve. Early neurolysis resulted in rapid reduction of the pain and recovery of motor function.

Leprosy Reactions in Patients Coinfected with HIV: Clinical Aspects and Outcomes in Two Comparative Cohorts in the Amazon Region, Brazil.

BACKGROUND: Leprosy, caused by Mycobacterium leprae, can lead to scarring and deformities. Human immunodeficiency virus (HIV), a lymphotropic virus with high rates of replication, leads to cell death in various stages of infection. These diseases have major social and quality of life costs, and although the relevance of their comorbidity is recognized, several aspects are still not fully understood. METHODOLOGY/PRINCIPAL FINDINGS: Two cohorts of patients with leprosy in an endemic region of the Amazon were observed. We compared 40 patients with leprosy and HIV (Group 1) and 107 leprosy patients with no comorbidity (Group 2) for a minimum of 2 years. Group 1 predominantly experienced the paucibacillary classification, accounting for 70% of cases, whereas Group 2 primarily experienced the multibacillary classification (80.4% of cases). There was no significant difference in the prevalence of leprosy reactions among the two groups (37.5% for Group 1 vs. 56.1% for Group 2), and the most frequent reaction was Type 1. The appearance of Group 1 patients' reversal reaction skin lesions was consistent with each clinical form: typically erythematous and infiltrated, with similar progression as those patients without HIV, which responded to prednisone. Patients in both groups primarily experienced a single episode (73.3% in Group 1 and 75% in Group 2), and Group 1 had shorter reaction periods (≤3 months; 93.3%), moderate severity (80%), with 93.3% of the patients in the state of acquired immune deficiency syndrome, and 46.7% presenting the reaction at the time of the immune reconstitution inflammatory syndrome. CONCLUSIONS/SIGNIFICANCE: This study used a large sample and makes a significant contribution to the clinical outcomes of patients in the reactive state with comorbid HIV and leprosy. The data indicate that these diseases, although concurrent, have independent courses.

Leprosy profiles in post-elimination stage: a tertiary care hospital experience.

BACKGROUND: Most countries that were previously highly endemic for leprosy have achieved elimination at the national level. OBJECTIVES: To find out the pattern, prevalence, and trends of leprosy in the post-elimination stage. METHODS: A descriptive retrospective cross-sectional study was carried out using the registered records of patients attending the leprosy clinic in Chittagong Medical College Hospital between the periods 2001 and 2011. RESULTS: The new case detection rate was declining. The prevalence rate was 0.75 at the end of 2011. Of a total of 789 patients, males (74%) outnumbered the females (26%). The age of the patients in the study group ranged from 6 years to 87 years with mean age 35.58 years ± 0.05 SEM. Paucibacillary (PB) and multibacillary (MB) cases were 314 (39.80%) and 475 (60.20%), respectively. MB percentage was increasing more among new cases in the study period, and 119 (15.08%) patients presented with grade 2 deformities. Smear positive cases were 141 (17.87%). Leprosy reaction comprised of 193 (24.46%) type 1, 68 (8.62%) type 2, and 97 (12.29%) neuritis cases. LIMITATIONS: This is a retrospective study from a single tertiary health center. CONCLUSION: MB cases with grade 2 deformities are in an upward trend, and rates of children are declining. Developing reinforced new therapies to curb reactions, deformities are very important and contact tracing, especially of children, is essential. Domiciliary treatment needs to be made available to ensure early diagnosis.

Menstrual irregularities, fertility status, and ovarian function in female patients with leprosy in India.

BACKGROUND: Gonadal involvement in males in lepromatous leprosy is not uncommon, but there is a paucity of literature on the involvement of gonads in female patients with leprosy. This study was undertaken to determine if there is any menstrual dysfunction, alteration in fertility status and circulating luteinizing hormone (LH), follicle-stimulating hormone (FSH), prolactin, and estradiol in female patients with paucibacillary (PB) and multibacillary (MB) leprosy. MATERIALS AND METHODS: In a cross-sectional study, 229 patients with leprosy (79 with PB leprosy and 150 with MB leprosy) and 100 age-matched non-leprosy controls were evaluated for menstrual function, fertility status, and circulating sex hormones. RESULTS: Twenty percent of patients with MB leprosy had menstrual irregularities post-dating the onset of leprosy in comparison to 6.3% patients with PB leprosy, and this difference was statistically significant (P < 0.001). However, the fertility profile of patients with PB leprosy was comparable to that of patients with MB leprosy (P > 0.05). A significantly higher number of patients with MB leprosy (9.3%) had elevation of circulating FSH, LH, and prolactin vis-à-vis patients with PB leprosy (1.3%), and this difference was statistically significant (P < 0.05). Similarly, the mean levels of LH, FSH, and prolactin were significantly elevated in patients with MB leprosy vis-à-vis patients with PB leprosy and controls (P < 0.05). CONCLUSIONS: Multibacillary leprosy may be associated with menstrual irregularities and elevation of gonadotropin hormones, indicating an ovarian dysfunction.

Renal dysfunction in leprosy: a historical cohort of 923 patients in Brazil.

We investigated the factors associated with renal dysfunction in leprosy patients from Brazil. We report on a historical cohort of leprosy patients followed in two hospitals in Fortaleza City in northeastern Brazil. The factors associated with renal dysfunction were investigated. A total of 923 patients were included, with a mean age of 41.5 ± 19.1 years, and 53.3% were male. Renal dysfunction was found in 35 cases (3.8%). Proteinuria was found in 4.8% of cases, haematuria in 6.8% and leukocyturia in 10.4%. Factors associated with renal dysfunction by multivariate analysis were: reaction episode (odds ratio [OR] = 3.9, P = 0.03), multibacillary classification (OR = 3.5, P = 0.02) and advanced age (OR = 1.04, P = 0.01). Four patients (0.4%) died. Leprosy is associated with renal dysfunction, especially in older patients and those presenting with reaction episode and multibacillary classification.

Surgical treatment of type I neuritis in a teenage boy with borderline tuberculoid leprosy

Exacerbation of the immune response against Mycobacterium leprae can lead to neuritis, which is commonly treated via immunosuppression with corticosteroids. Early neurolysis may be performed concurrently, especially in young patients with a risk of functional sequelae. We report the case of a young patient experienced intense pain in the left elbow one year after the treatment of tuberculoid-tuberculoid leprosy. The pain was associated with paresthesias in the ulnar edge and left ulnar claw. After evaluation, the diagnosis was changed to borderline tuberculoid leprosy accompanied with neuritis of the left ulnar nerve. Early neurolysis resulted in rapid reduction of the pain and recovery of motor function.