New insights into leukotrichia in nonsegmental vitiligo: A cross-sectional study.

BACKGROUND: Leukotrichia has been considered a predictor of poor outcome in vitiligo. However, studies considering the different clinical aspects of leukotrichia in vitiligo patients are few. AIM: Our aim was to conduct a detailed clinical study to provide insights into the relevance and associations of leukotrichia in non-segmental vitiligo. METHODS: In this cross-sectional study, vitiligo patients attending the dermatology outpatient clinic and phototherapy unit at Cairo University Hospital over a period of 6 months (April-September 2016) were included. Family history, clinical details, the Vitiligo Global Issues Consensus Conference classification, the Dermatology Life Quality Index, Vitiligo Area and Severity Index, Vitiligo Extent Score, Vitiligo Disease Activity Score and Vellus Score were determined and these measurements were correlated to leukotrichia. RESULTS: Out of the 101 patients studied, leukotrichia was found in 47 (46.5%) patients, with vellus hair involved in 37 (78.7%), terminal hairs in 30 (63.8%) and both in 20 (42.5%) patients. Vellus hair involvement was significantly higher in generalized bilaterally symmetrical vitiligo than in acrofacial or unclassified vitiligo. The incidence of scalp leukotrichia also was higher in generalized symmetrical vitiligo than in acrofacial vitiligo. The Vellus Score showed significant associations with Vitiligo Area and Severity Index, Vitiligo Extent Score and the Dermatology Life Quality Index. LIMITATIONS: This was a short-term study with a small sample size. Prognostic and therapeutic correlations were not studied; prospective longitudinal studies are needed for further evaluation. CONCLUSION: Leukotrichia was found in almost half of the studied sample and its frequency varied among the different types of vitiligo.

The teratogenic effects of thalidomide on limbs.

Thalidomide remains notorious as a result of the damage it caused to children born to mothers who used it to treat morning sickness between 1957 and 1961. The re-emergence of the drug to treat a range of conditions including erythema nodosum leprosum (a complication of leprosy) has led to a new generation of thalidomide damaged children being born in Brazil. Although thalidomide affects most of the developing tissues and organs of the body, the damage to the limbs is striking. Indeed phocomelia, the severe reduction or loss of the proximal long bones with retention of the distal hand/foot plate remains the stereotypical image of thalidomide. This review focuses on the type and range of damage thalidomide caused to the limbs, reviews current understanding of the mechanisms underlying thalidomide-induced limb malformations and outlines some of the challenges remaining in elucidating its teratogenicity.

Clinico-pathological features of erythema nodosum leprosum: A case-control study at ALERT hospital, Ethiopia.

BACKGROUND: Leprosy reactions are a significant cause of morbidity in leprosy population. Erythema nodosum leprosum (ENL) is an immunological complication affecting approximately 50% of patients with lepromatous leprosy (LL) and 10% of borderline lepromatous (BL) leprosy. ENL is associated with clinical features such as skin lesions, neuritis, arthritis, dactylitis, eye inflammation, osteitis, orchitis, lymphadenitis and nephritis. ENL is treated mainly with corticosteroids and corticosteroids are often required for extended periods of time which may lead to serious adverse effects. High mortality rate and increased morbidity associated with corticosteroid treatment of ENL has been reported. For improved and evidence-based treatment of ENL, documenting the systems affected by ENL is important. We report here the clinical features of ENL in a cohort of patients with acute ENL who were recruited for a clinico-pathological study before and after prednisolone treatment. MATERIALS AND METHODS: A case-control study was performed at ALERT hospital, Ethiopia. Forty-six LL patients with ENL and 31 non-reactional LL matched controls were enrolled to the study and followed for 28 weeks. Clinical features were systematically documented at three visits (before, during and after predinsolone treatment of ENL cases) using a specifically designed form. Skin biopsy samples were obtained from each patient before and after treatment and used for histopathological investigations to supplement the clinical data. RESULTS: Pain was the most common symptom reported (98%) by patients with ENL. Eighty percent of them had reported skin pain and more than 70% had nerve and joint pain at enrolment. About 40% of the patients developed chronic ENL. Most individuals 95.7% had nodular skin lesions. Over half of patients with ENL had old nerve function impairment (NFI) while 13% had new NFI at enrolment. Facial and limb oedema were present in 60% patients. Regarding pathological findings before treatment, dermal neutrophilic infiltration was noted in 58.8% of patients with ENL compared to 14.3% in LL controls. Only 14.7% patients with ENL had evidence of vasculitis at enrolment. CONCLUSION: In our study, painful nodular skin lesions were present in all ENL patients. Only 58% patients had dermal polymorphonuclear cell infiltration showing that not all clinically confirmed ENL cases have neutrophilic infiltration in lesions. Very few patients had histological evidence of vasculitis. Many patients developed chronic ENL and these patients require inpatient corticosteroid treatment for extended periods which challenges the health service facility in resource poor settings, as well as the patient's quality of life.

Prevalence of leprous neuropathy determined by neurosensory testing in an endemic zone in Ecuador: Development of an algorithm to identify patients benefiting from early neurolysis.

The success of a microneurosurgical intervention in leprous neuropathy (LN) depends on the diagnosis of chronic compression before irreversible paralysis and digital loss occurs. In order to determine the effectiveness of a different approach for early identification of LN, neurosensory testing with the Pressure-Specified Sensory Device™ (PSSD), a validated and sensitive test, was performed in an endemic zone for leprosy. A cross-sectional study was conducted to analyze a patient sample meeting the World Health Organization (WHO) criteria for Hansen's disease. The prevalence of LN was based on the presence of ≥1 abnormal PSSD pressure threshold for a two-point static touch. A total of 312 upper and lower extremity nerves were evaluated in 39 patients. The PSSD found a 97.4% prevalence of LN. Tinel's sign was identified in 60% of these patients. An algorithm for early identification of patients with LN was proposed using PSSD testing based on the unilateral screening of the ulnar and deep peroneal nerves.

Pancytopenia due to lepromatous involvement of the bone marrow: successful treatment with multidrug therapy.

Leprosy is a chronic infectious disease with a wide spectrum of signs and symptoms depending on the ability of the host's immune system to resist the infection. The disease is frequently associated with sensory loss in skin lesions and damage in peripheral nerve trunks leading to nerve function impairment. In lepromatous leprosy, the immune system offers no protection against the multiplying bacilli and this results in heavy infiltration of the internal organs. We report a case of florid lepromatous leprosy with bone marrow suppression due to the disease, presenting with anemia, leukocytopenia and thrombocytopenia. The hematological abnormalities were fully reversed by mutidrug therapy for leprosy. We suggest that infiltration of the bone marrow by Mycobacterium leprae can cause pancytopenia, which can be cured by treatment of the leprosy alone.

Cutaneous vasculitis as a presenting feature of multiple myeloma: a report of 2 cases.

We report two male patients who presented with symmetrical, painful purpura that evolved into bullae and necrotic ulcers, predominantly on the extremities, over two months in spite of conventional therapy including oral steroids. Examination showed livedoid and purpuric patches with necrotic centers in starburst pattern over the extremities and buttocks. The first case also had similar lesions over the ears. The clinical presentation and the histopathological examination suggested a diagnosis of necrotizing leukocytoclastic vasculitis (LCV). Blood testing ruled out connective tissue disease, hepatitis B or C infection or streptococcal infection as underlying cause of vasculitis. Serum antinuclear factor, antineutrophilic cytoplasmic antibody and anticardiolipin anticoagulant were negative in both cases. Cryoglobulins were positive in case 2. An incidental finding was raised serum proteins and globulins in case 2. Further investigations revealed M band on electrophoresis and features of multiple myeloma on bone marrow biopsy in both cases. These cases emphasize the importance of simple investigations like serum proteins in the evaluation of LCV.