Nerve biopsies in patients with peripheral neuropathy: A prospective evaluation.

Background: In approximately 25% of peripheral neuropathy cases, diagnosis remains obscure. In India, leprosy continues to remain one of the most frequent causes of peripheral neuropathy. We, in this prospective evaluation, performed nerve biopsies in patients with peripheral neuropathy for early confirmation of the diagnosis. Materials and Methods: A total of 55 consecutive cases of peripheral neuropathy were included in this study. All patients were subjected to clinical and electrophysiological evaluation. Sural nerve biopsies were performed in all the patients. Result: After a nerve biopsy in 29 cases, we were able to identify the underlying cause of peripheral neuropathy. In 26 cases, the diagnosis remained obscure. The most frequent histopathological diagnosis was leprosy, which was seen in 20 cases. Other diagnoses were chronic demyelinating neuropathy (four cases), vasculitis (two cases), and amyloidosis in one case. In two biopsies, the findings were consistent with hereditary neuropathies. The demonstration of lepra bacilli was the most distinctive feature. In addition, foamy macrophages (100%) and granuloma (100%) formation, epineurial (83.3%) and endoneurial infiltration (69%) along with epineurial (87.5%) and perineurial thickening (77.3%) were also noted more frequently in leprosy-associated neuropathy. Conclusion: The nerve biopsies revealed that leprosy was the most common etiology in patients with peripheral neuropathy. In approximately 47% of the cases, even nerve biopsies failed to establish a confirmed diagnosis.

Central nervous system, spinal root ganglion and brachial plexus involvement in leprosy: A prospective study.

Background: Leprosy is primarily a disease of peripheral nerves. Some isolated case reports and case series have communicated imaging changes in the central nervous system (CNS) and brachial plexus in patients with leprosy. Objectives: To study the neuroimaging abnormalities in patients with lepra bacilli-positive neuropathy in the context of CNS, spinal root ganglion, and brachial plexus. Design: Prospective observational study. Methods: We screened newly-diagnosed patients with multibacillary leprosy presenting with neuropathy. Patients with bacilli-positive sural nerve biopsies were included in the study and subjected to magnetic resonance imaging (MRI) of the brain and spinal cord. Results: A total of 54 patients with bacteriologically confirmed multibacillary leprosy were screened; Mycobacterium leprae was demonstrated in the sural nerve biopsies of 29 patients. Five patients (5/29; 17.24%) had MRI abnormalities in CNS, spinal root ganglion, and/or brachial plexus. Three patients had MRI changes suggestive of either myelitis or ganglionitis. One patient had T2/FLAIR hyperintensity in the middle cerebellar peduncle while 1 had T2/FLAIR hyperintensity in the brachial plexus. Conclusion: CNS, spinal root ganglion, and brachial plexus are involved in patients with leprous neuropathy. Immunological reaction against M leprae antigen might be a plausible pathogenetic mechanism for brachial plexus and CNS imaging abnormalities.

A rare case of scrub typhus vasculitis presenting as acute coronary syndrome diagnosed by skin manifestations.

A 48-year-old diabetic man presented with complaints of acute onset chest heaviness with palpitations, anxiety and headache. He had raised troponin-T level and electrocardiogram showed ST elevation myocardial infarction. There was a prior history of fever of 4 days duration with associated abdominal pain. He later developed skin rash and neurological symptoms following admission to the hospital. Dermatological examination revealed purpura and a livedo-like rash. Investigations revealed deranged liver and renal function tests and positive serological tests for scrub typhus. Coronary angiography revealed no evidence of atherosclerosis or any other pathology. He was therefore diagnosed as a case of scrub typhus-induced vasculitis with coronary manifestations and was managed with oral doxycycline. Scrub typhus presenting like an acute coronary syndrome has been reported very rarely previously. In addition, patient had gastrointestinal, central nervous system and hematological involvement which added to the rarity of the case.

Pathological, ultrasonographic, and electrophysiological characterization of clinically diagnosed cases of pure neuritic leprosy.

A subset of neuritic form of leprosy, called pure neuritic leprosy (PNL), seen in a minority of leprosy patients, is characterized by peripheral neuropathy without skin lesions and an absence of acid-fast bacilli on skin smears. Patients with PNL are often started on drug therapy without confirmation of diagnosis. We, therefore, did a prospective study of clinically diagnosed PNL patients with correlation of ultrasonographic and biopsy findings. A total of 100 consecutive patients with PNL, diagnosed according to the consensus case definition, were included in the study. All patients underwent nerve conduction study, peripheral nerve ultrasonography, and sural nerve biopsy. Multiple mononeuropathies were present in 75% of cases, mononeuropathy in 18%, and polyneuropathy in the remaining 7%. Compared to clinical examination, ultrasonographic assessment of the peripheral nerves was not only better at the detection of thickening but also helped in characterization of their fascicular architecture, echogenicity, and vascularity. A total of 32 cases were confirmed on nerve biopsy, out of which 75% had demonstrable lepra bacilli. Cranial nerve involvement, presence of trophic ulcers, and bilateral thickening of the great auricular nerve were significantly associated with the positivity of lepra bacilli. A significant improvement in the disability score happened after multidrug therapy. A comprehensive electrophysiologic, ultrasonographic, and histological evaluation may be helpful in establishing a diagnosis of PNL with greater confidence, while ruling out other non-leprosy diagnoses.

Livedoid vasculopathy: A review of pathogenesis and principles of management.

Livedoid vasculopathy is a rare cutaneous disease manifesting as recurrent ulcers on the lower extremities. The ulceration results in atrophic, porcelain white scars termed as atrophie blanche. The pathogenesis is yet to be understood with the main mechanism being hypercoagulability and inflammation playing a secondary role. The important procoagulant factors include protein C and S deficiency, factor V Leiden mutation, antithrombin III deficiency, prothrombin gene mutation and hyperhomocysteinemia. Histopathology of livedoid vasculopathy is characterized by intraluminal thrombosis, proliferation of the endothelium and segmental hyalinization of dermal vessels. The treatment is multipronged with anti-thrombotic measures such as anti-platelet drugs, systemic anticoagulants and fibrinolytic therapy taking precedence over anti-inflammatory agents. Colchicine, hydroxychloroquine, vasodilators, intravenous immunoglobulin, folic acid, immunosuppressive therapy and supportive measures are also of some benefit. A multidisciplinary approach would go a long way in the management of these patients resulting in relief from pain and physical as well as psychological scarring.

Greater auricular nerve masquerading as lymph node.

Hansen's disease is on the verge of being eliminated from India and often missed by clinicians due to low index of suspicion. We present an unusual case in which greater auricular nerve thickening masqueraded as enlarged lymph node in the neck. The patient was referred for fine needle aspiration cytology, which revealed epithelioid cell granulomas suggestive of Hansen's disease. Further clinical examination and investigations including the skin biopsy confirmed the disease, highlighting the role of pathologist in the management of such unusual presentation of a common disease.

Bullous erythema nodosum leprosum manifesting in the post partum period with unusual features.

A 30 year old woman who presented with multiple numb patches on the body was initially diagnosed as borderline lepromatous leprosy and started on multidrug therapy for leprosy. She had an episode of Type 1 reaction during the fifth month of pregnancy. After delivery, she stopped therapy fearing harm to her child and developed an episode of Type 2 reaction. The reaction was unusual in that bullous lesions developed over previous leprosy patches which had initially become indurated, with associated neuritis. Histopathology revealed bullae with intense neutrophilic reaction and strong positivity for acid fast bacilli. There was no response to steroid therapy which was started for the reaction. Thalidomide had to be prescribed after stopping lactation by medical means. She responded dramatically to Thalidomide with regression of cutaneous lesions and neuritis. This patient is being reported as a very unusual manifestation of bullous erythema nodosum leprosum in the postpartum period responding dramatically to thalidomide.

Rare atypical presentations in Type 2 lepra reaction: a case series.

OBJECTIVES: Type 2 lepra reaction is a Th2-mediated type III hypersensitivity reaction in leprosy, with a characteristic cutaneous manifestation in the form of erythema nodosum leprosum (ENL). We describe unusual presentations of Type 2 lepra reaction in five patients. METHODS: Patient data and dermatological findings were analyzed in three men and two women diagnosed with Hansen's disease. RESULTS: Findings included multiple tender, polycyclic, necrotic lesions distributed over the face in one patient, and painful, fluid-filled lesions on both arms and lower limbs in another. The third patient showed erythematous, tender nodules, bullae, and necrotic ulcers over the back and upper and lower limbs. The fourth showed erythematous tender nodules over the face, neck, back, and extremities, predominantly in sun-exposed areas. The fifth revealed multiple erythematous, severely tender nodules and urticarial plaques mimicking those of Sweet's syndrome. Diagnosis of borderline or lepromatous leprosy with atypical Type 2 reaction were made in all cases. CONCLUSIONS: Type 2 lepra reactions are antigen antibody-mediated immune complex reactions that present with constitutional symptoms and ENL characterized by tender, erythematous, evanescent nodules mainly on the face, arms, and legs. Over 50% of lepromatous leprosy patients and 25% of borderline lepromatous leprosy patients experienced type 2 lepra reactions prior to the advent of multi-drug therapy. Thalidomide is the drug of choice for severe atypical lepra reactions because of its anti-tumor necrosis factor-α action. Awareness of these atypical variants and prompt diagnosis and treatment are essential to prevent mortality and morbidity in potentially treatable patients.

Cyclosporine in Stevens Johnson syndrome and toxic epidermal necrolysis and retrospective comparison with systemic corticosteroid.

BACKGROUND: Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening dermatological emergencies. Many immunosuppressive modalities have been tried with variable results. AIMS: To determine the efficacy of cyclosporine in cases of SJS and TEN and compare the efficacy with systemic corticosteroid in the same condition. METHODS: Study was conducted at a tertiary hospital during 01 July 2011 to 30 June 2012. SCORTEN was assessed at the time of admission. Total body surface area (TBSA) assessment was like any burn patients. Cyclosporine was administered in the dose of 3 mg/kg body weight in three divided dosage for 07 days and then tapered over another 07 days. Data were compared to a historical series of SJS/TEN patients, managed by systemic steroids a year ago. RESULTS: A total of 11 consecutive patients with a mean age of 32.09 and standard deviation (SD 16.17) were enrolled in to cyclosporine group, which were retrospectively compared to 6 patients with a mean age of 27.87 (SD 13.97) years in the corticosteroid group. The mean duration of re-epithelialization was 14.54 (SD 4.08) and 23 days (SD 6.68) in cyclosporine and corticosteroid group respectively (P = 0.009956). Mean hospital stay was 18.09 (SD 5.02) and 26 (SD 6.48) days in cyclosporine and corticosteroid group respectively (P = 0.02597). A total of 1.11 and 0.51 patients were expected to die against no death and two deaths in cyclosporine and corticosteroid group respectively (Standardized mortality ratio = 3.92) (P = 0.04321). CONCLUSION: This study definitely suggests that cyclosporine has encouraging role in the management of uncomplicated cases of SJS, SJS-TEN overlap or TEN.

Mononeuritis multiplex and painful ulcers as the initial manifestation of hepatitis B infection.

Hepatitis B virus infection leads to multisystem manifestations owing to involvement of kidney, skin, vasculature, haematopoietic and nervous system. The hepatitis B infection can cause neuropathy either to vasculitis associated with polyarteritis nodosa or immune-mediated neural damage. In this submission, we report a young woman, who presented with mononeuritis multiplex and painful ulcerations as the first manifestation of chronic hepatitis B virus infection. The antiviral therapy along with steroids led to remarkable recovery. The clinical settings of hepatitis B virus infection should not be ignored in the presentation of mononeuritis multiplex with ulcers, although the commonest cause is leprosy in the Indian sub-continent.