Profile of Rheumatological Manifestations in Leprosy in a Tertiary Care Hospital from Himachal Pradesh.

The rheumatological manifestations of leprosy occur singly or in varying combinations, particularly during lepra reactions. Despite being third most common, these remain under diagnosed and under reported. This study has been carried out to study the spectrum of rheumatological manifestations in leprosy patients. One hundred consecutive patients of leprosy presenting during January to December 2013 were studied for rheumatological manifestations. Complete hemogram, serum biochemistry, urinalysis, rheumatoid factor, ASO titer, C-reactive protein, ANA, and x-rays for hands, feet, chest and involved joints were performed. These 100 (M:F 66:34) patients aged between 16-80 years had indeterminate (2 patients), TT (4 patients), BT (26 patients), BB (2 patients), and LL leprosy (32 patients). 27 patients had rheumatological manifestations; arthritis involving large or small joints in 23 patients being the commonest. 7 of 24 patients in type-1 lepra reaction had enthesitis in 3 patients and oligoarthritis in 4 patients. Rheumatoid arthritis-like polyarthritis was noted in 19 patients with type-2 reaction. Tenosynovitis, dactylitis, bony changes were also noted. Except for one case, these features were present in patients having lepra reactions. Rheumatoid factor in 14, ANA in 15, C-reactive protein in 45 cases was positive. ASO was positive 34 cases. Symmetrical polyarthritis involving small joints of hands and feet, oligoarthritis, enthesitis and dactylitis are common in leprosy particularly with borderline leprosy, type-2 lepra reaction especially in the presence of positive RAfactor.

Type II lepra reaction: an unusual presentation.

Ulcers with maculo-papular rash are an unusual presenting feature of leprosy. They occur as result of neuropathy, type-2 lepra reaction or Lucio's phenomenon. The hall mark of type-2 reaction is erythema nodosum. Very rarely it manifests as ulcerative skin lesions. We describe one such unusual case of a young male who presented with multiple ulcers and maculo-papular rash over the legs, chest and abdomen. In addition to this, he had fever, heart murmur, pulmonary infiltrates, neuropathy, and deranged liver function. A clinical differential diagnosis of infective endocarditis and systemic nectrozing vasculitis was made. Skin biopsy showed dense inflammation with lepra bacilli consistent with type-2 lepra reaction.

Immunoregulation of cytokines in infectious diseases (leprosy), future strategies.

Leprosy is a dynamic disease model in which distinct Mycobacterium leprae-responsive T cell subsets play an important role in host defense and control clinical and immunological spectrum. Th1 cells are associated with tuberculoid leprosy patients that have strong M. leprae-specific CMI-DTH responses. Th2 cells are expressed in lepromatous leprosy patients that are characterized by strong humoral immune responses and lack of T cell responses. Recently cytokines are thought to play immunoregulatory role in both the protection and immunopathogenesis of the host. Recombinant cytokines for immunotherapy have been used for controlling mycobacterial infections including leprosy. The diversity of T-cell subsets contributing to Th1 and Th2 cell derived cytokines, other major cytokines of the immune system, their sources, modes of action and possible therapeutic potentials are discussed.

The possible role of interleukin (IL)-12 and interferon-gamma-inducing factor/IL-18 in protection against experimental Mycobacterium leprae infection in mice.

Cell-mediated immunity participates in host defense against mycobacterial infection. Both interleukin 12 (IL-12) and interferon-gamma-inducing factor (IGIF/IL-18), produced mainly by macrophages, play a critical role in expression of cell-mediated immunity. To investigate the role of IL-12 and IGIF/IL-18 in vivo, we examined cytokine profile, bacterial growth, and the potential benefit of cytokine therapy in susceptible and resistant mice infected with Mycobacterium leprae. The early expression of IL-12 p40 and IGIF/IL-18 at the site of inoculation was found in resistant mice 3-72 h after the infection, but not in susceptible mice. Both strains of mice did not show expression of IFN-gamma and IL-4. IL-12 administration resulted in a significant reduction of bacterial counts in mice with established M. leprae infection. The results imply that susceptible mice exhibit decreased expression of type 1 helper T (Th1) response without reciprocal increased Th2 response and show responsiveness to exogenous IL-12. IL-12 therapy may be a possible rationale for treatment of M. leprae infection.

Normal variants of skin in neonates.

2221 consecutive live births taking place between March 1994 and February 1995 were evaluated for a minimum period of 5 days to note for the occurrence of various normal anatomical variants specially those of skin. Birth weight, gestational age, maternal age, socio-economic status and consanguinity were carefully recorded in all the cases. Mongolian spots (72%), Epstein pearls (43.8%), Milia (26.2%) and Erythema toxicum (25.2%), were the common dermatological variants noted. Maturity of the babies and possibly genetic factors (consanguinity) are important factors in their causation as ordered in our study.

Numerous giant mollusca contagiosa and kaposi's sarcomas with HIV disease.

A 50-year old man with a history of multiple contacts with commercial sex workers and two episodes of blood transfusions in Bombay, presented with giant mollusca contagiosa, Kaposi's sarcomas and refractory pulmonary tuberculosis. Patient was confirmed to be infected with HIV by ELISA and Western blot method.

Naevus comedonicus associated with epidermoid cyst.

Naevus comedonicus associated with epidermoid cyst is a rare association. Two such case reports are presented.

Psoriasis in identical twins: simultaneous occurrence on same sites.

A pair of 12-year-old identical male twins presented to us with dull erythematous plaques with coarse scaling and deep fissuring over soles of both feet starting simultaneously 18 months ago. The diagnosis of psoriasis was confirmed by histopathological examination of the biopsy specimen.

Unilateral darier's disease.

Various variations of Darier's disease have been mentioned in the literature. Here we describe a young male with unilateral involvement with clinical and histopathological features typical of Darier's disease.

Gottron's papules with raised CPK level without other manifestations of dermatomyositis.

Dermatomyositis without myositis has been reported rarely and has been given several names such as dermatomyositis sine myositis and amyopathic dermatomyositis. We report a 19-year-old male patient with Gottron's papules and raised CPK level. The patient has been followed for 10 months and has not developed other features of myositis.

Comparative study of short term results in two multidrug regimens in multibacillary leprosy.

Thirty lepromatous and Borderline lepromatous leprosy patients were treated with multidrug therapy in an open trial. Fifteen of them received the standard WHO multidrug regimen ie., rifampicin 600 mg and clofazimine 300 mg monthly, supervised, and dapsone 100 mg daily and clofazimine 100 mg on alternate days as self administered; the other 15 received a modified multidrug therapy regimen comprising of rifampicin 600 mg, clofazimine 100 mg and dapsone 100 mg daily for 21 days as suggested by the Indian Association of Leprologists, followed by the standard WHO regimen. The observation period was six months. Clinical, bacteriological, histological and immunological parameters were studied. The fall in morphological index was much faster in patients receiving modified multidrug therapy regimen compared to those receiving the standard WHO regimen. Otherwise, there was no difference between the two groups of patients. Five patients developed type I (upgrading) reaction with one developing ulnar nerve paralysis. No untoward effects of drugs were noted in the study subjects except for darkening of skin colour of all the patients.

Sarcoidosis masquerading as leprosy, pulmonary tuberculosis and urolithiasis.

A middle aged man presented as a recalcitrant case of borderline leprosy with concomitant pulmonary tuberculosis and urolithiasis which continued to progress relentlessly despite adequate multiduring antileprosy and antitubercular treatment. After detailed and relevant workup, the diagnosis of sarcoidosis was made. Rapid clinical improvement occurred with steroid therapy.