Juvenile dermatomyositis in Thai children: Retrospective review of 30 cases from a tertiary care center.

BACKGROUND: Juvenile dermatomyositis is a rare condition, but it is the most common idiopathic inflammatory myopathy in pediatric patients. AIM: To study the clinical manifestations, investigations, treatment, clinical course, and outcomes of juvenile dermatomyositis in Thai children. METHOD: This retrospective study included juvenile dermatomyositis patients treated at Siriraj Hospital, a 2,300-bed national tertiary referral center in Bangkok, Thailand, from 1994 to 2019. RESULTS: Thirty patients (22 females and 8 males) were included with a female to male ratio of 2.7:1. Median age at diagnosis was 5.1 years (range, 2.6-14.8 years). Median duration of illness before diagnosis was 6.5 months (range, 0.3-84.0 months). Acute and subacute onset occurred in the majority of patients. Presenting symptoms included muscle weakness in 27/30 (90%), skin rash in 26/30 (86.7%), muscle pain in 17/26 (65.4%), and arthralgia in 4/18 (22.2%) of patients. Dermatologic examination revealed Gottron's rash, heliotrope rash, and periungual telangiectasia in 25/30 (83.3%), 21/30 (70.0%), and 15/24 (62.5%) of patients, respectively. Interestingly, scalp dermatitis was found in 8/21 (38.1%) of patients. The most commonly used treatment regimen in this series was a combination of prednisolone and methotrexate. During the median follow-up of 3.1 years (range, 0.0-18.5 years), only one-third of patients were seen to have monocyclic disease. Extraskeletal osteosarcoma at a previous lesion of calcinosis cutis was observed in one patient at 12 years after juvenile dermatomyositis onset. LIMITATIONS: This was a retrospective single-center study, and our results may not be generalizable to other healthcare settings. Prospective multicenter studies are needed to confirm the findings of this study. CONCLUSION: juvenile dermatomyositis usually poses a diagnostic and therapeutic challenge, which can be compounded by the ethnic variations in the clinical presentation, as observed in this study. Asian patients tend to present with acute or subacute onset of disease, and arthralgia and/or arthritis are less common than in Caucasian patients. Scalp dermatitis is not uncommon in pediatric juvenile dermatomyositis patients. An association between juvenile dermatomyositis and malignancy, though rare, can occur.

Pure neuritic leprosy presenting as ulnar nerve neuropathy: a case report of electrodiagnostic, radiographic, and histopathological findings.

Hansen's disease, or leprosy, is a chronic infectious disease with many manifestations. Though still a major health concern and leading cause of peripheral neuropathy in the developing world, it is rare in the United States, with only about 150 cases reported each year. Nevertheless, it is imperative that neurosurgeons consider it in the differential diagnosis of neuropathy. The causative organism is Mycobacterium leprae, which infects and damages Schwann cells in the peripheral nervous system, leading first to sensory and then to motor deficits. A rare presentation of Hansen's disease is pure neuritic leprosy. It is characterized by nerve involvement without the characteristic cutaneous stigmata. The authors of this report describe a case of pure neuritic leprosy presenting as ulnar nerve neuropathy with corresponding radiographic, electrodiagnostic, and histopathological data. This 11-year-old, otherwise healthy male presented with progressive right-hand weakness and numbness with no cutaneous abnormalities. Physical examination and electrodiagnostic testing revealed findings consistent with a severe ulnar neuropathy at the elbow. Magnetic resonance imaging revealed diffuse thickening and enhancement of the ulnar nerve and narrowing at the cubital tunnel. The patient underwent ulnar nerve decompression with biopsy. Pathology revealed acid-fast organisms within the nerve, which was pathognomonic for Hansen's disease. He was started on antibiotic therapy, and on follow-up he had improved strength and sensation in the ulnar nerve distribution. Pure neuritic leprosy, though rare in the United States, should be considered in the differential diagnosis of those presenting with peripheral neuropathy and a history of travel to leprosy-endemic areas. The long incubation period of M. leprae, the ability of leprosy to mimic other conditions, and the low sensitivity of serological tests make clinical, electrodiagnostic, and radiographic evaluation necessary for diagnosis. Prompt diagnosis and treatment is imperative to prevent permanent neurological injury.

Granulomatous mycosis fungoides with large cell transformation misdiagnosed as leprosy.

BACKGROUND: Granulomatous mycosis fungoides is an unusual histopathological variant of cutaneous T-cell lymphoma without clinical distinction from classic mycosis fungoides. Symptoms associated with peripheral nerve involvement have rarely been reported in the literature. CASE REPORT: The authors described a case of granulomatous MF stage IIB with large cell transformation who initially presented with leprosy-like condition and chronic left peroneal neuropathy The patient received six courses ofgemcitabine with greater than 90% improvement of skin lesions. The rest of the lesions were successfully treated with local electron beam radiation. CONCLUSION: Granulomatous MF with neuropathy can be clinically misdiagnosed if there is no histopathological and immunohistochemical finding to support the diagnosis of lymphoma.

[Neuropathy of leprosy: characteristics of cases in 1962-1995]. / Neuropatía leprosa: caracterización de la casuística entre 1962 y 1995.

INTRODUCTION: Neuropathy due to leprosy is the most frequent cause of peripheral nervous system disorders due to an infective agent, one of the commonest aetiologies of peripheral neuropathy, and also one of the few peripheral neuropathies which is curable. Hansen's disease, initially and predominantly involves the skin, so it is not usually seen by a neurologist; a neurologist sees cases which are difficult to diagnose and often after the condition has been ruled out by other doctors from other medical specialties. PATIENTS AND METHODS: In the National Institute of Neurology and Neurosurgery of La Habana, Cuba, 18 nerve biopsies were studied (during its history), that had been diagnosed as leprous neuropathy. We reviewed the clinical histories of all the patients admitted to this centre. The sample was characterized by: age, sex, neurological clinical picture, nerves most affected, skin lesions, electrophysiological studies and a description of the anatomopathological findings. RESULTS AND DISCUSSION: Most patients were male and all (100%) were adults of 'working age' (19-65 years). The most frequent neuropathic pattern was multiple mono-neuropathy (78%) and the quality, purely sensitive or mixed was predominantly sensitive (100%). The nerves most affected were the ulnar (10 patients) and median (6 patients). The most frequent skin lesions were anesthetic maculae. Anatomo-pathological study showed inflammatory infiltration in all patients and the bacillus was absent in only one case.

Intertester reliability of manual muscle strength testing in leprosy patients.

This study reports the results of a study on the intertester reliability of manual muscle strength testing in leprosy patients with confirmed motor function loss of at least one nerve. Three testers graded the muscle strength of 72 patients in random order. Both hands and feet were graded. Strength was graded on a modified Medical Research Council Scale (9 points, 5, 4+, 4, 3+, 3, 2+, 2, 1, 0). The following movements were tested for strength: little finger and index finger abduction, intrinsic position of all four fingers, thumb abduction and opposition, foot dorsiflexion and eversion and extension of the big toe. The weighted kappa statistic was used to calculate the chance-corrected percentage of agreement between observers. Overall agreement for each of the 11 tests appeared to be good or very good (0.61-1.00). However, when data for hands or feet with normal strength or complete paralysis were excluded from the analysis, the reliability of the remaining mid-range scale was not acceptable (kappa 0.55-0.88, direct agreement range 11-41%). While the reliability of this scale could possibly be improved by special training, we feel that, for the evaluation of nerve function for leprosy patients with (suspected) nerve function loss, the extended 9-point VMT scale should only be used when direct intra- or intertester agreement is more than 80%.

Evaluation of results of surgical decompression of median nerve in leprosy in relation to sensory--motor functions.

Median nerve decompression was performed in 29 leprosy patients of which 20 were followed up for varying periods. It has been observed that the decompression was beneficial, sensory recovery was seen in 90% cases and in 45% cases the muscle strength improved and the process of deterioration was arrested in another 25% cases.

Disabilities in multibacillary leprosy patients: before, during and after multidrug therapy.

One hundred fifty-one patients (125 males and 26 females) of multibacillary leprosy (LL 88, BL 40, BB 23), registered during 1986-1992 for multidrug therapy (MDT), were analysed with reference to their disabilities before, during and after MDT. At induction 48 (31.7%) had no disability (Gr 0), 59 (39.0%) had only peripheral anaesthesia (Gr 1) and 44 (29.1%) had Gr 2 and 3 deformities with or without anaesthesia. The parallel analysis of the three groups, with nearly equal duration of symptoms, revealed that new deformities developed in only a few cases during and after MDT, least in the Gr 0 group. The crude fresh deformity incidence was 59.2 per 1,000 person years of observation. The rate of recovery from anaesthesia was higher (64%) in Gr 1 group than that (44%) in group with Gr 2, 3 deformities. No significant difference was observed between the incidence of Gr 2 deformities developed before, during and after MDT (incidence of claw-hands 9.2% before and 7.9% during and after MDT, trophic ulcers 13.9% before and 17.8% during and after MDT). Out of 19 cases which developed motor weakness during MDT and follow-up, 10 (52.6%) were instances of quite nerve paralysis. Occupational factors influenced the development of deformities but not the sex and bacterial load. Generally, the lower the Grade of disability at induction of patient for MDT, the lower the chances of new disability development and higher the chances of recovery from sensory impairments.