A Case Of Pregnancy With Coexisting Systemic Sclerosis And Paucibacillary Leprosy

Author Information
Nasare P*, Prasad M**, Gupta AS***
(* Third year Resident, ** Assistant Professor, *** Professor, Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai, India.)

The clinical characteristics of women who conceive during leprosy and systemic sclerosis and the association between it are not well known. A case of coexisting systemic sclerosis in which paucibacillary lepromatous leprosy developed secondarily, and its successful management in pregnancy is described here. To the best of our search of available literature, no cases of pregnancy with coexisting systemic sclerosis and paucibacillary leprosy were found. This appears to be the 1st case of pregnancy with coexisting systemic sclerosis and paucibacillary leprosy that is being reported.
Systemic sclerosis is an autoimmune disorder with a wide variety of presentation, of which dermatological manifestation in just one component. Paucibacillary leprosy is an infective disorder caused by Mycobacterium leprae with a predominantly dermatological presentation. The occurrence of leprosy has drastically reduced in India. While the occurrence as an individual disease itself is low, its occurrence along with an autoimmune disorder like systemic sclerosis is even rarer. Successfully management of such a case encountered in pregnancy is described here.
Case Report

 A 28 year old married woman gravida 2 para 1 living 1 presented to our outpatient department at 36 weeks of gestation. She had registered elsewhere in the end of first trimester and was on regular antenatal follow-up, compliant to hematinics/ calcium and other medications. Her first pregnancy was a spontaneous conception with a full term LSCS performed for transverse lie and she delivered a healthy 3 kg male child. At that time, she did not have any medical disorders. One year after completion of her first pregnancy, she noticed tightening of skin over arms, an approximately 3*4 centimeter plaque on right cheek, increased sensitivity to light, redness of skin, decreased opening of mouth following which she consulted a dermatologist. Investigations were done, of which ANA was positive and ANA blot showed Scl-70 positivity with a titer of 1:320 and a diagnosis of systemic sclerosis was reached. X-ray of bilateral hands had been done which was suggestive of acro-osteolysis. Spirometry was suggestive of mild restrictive lung disease. She was started on tablet prednisolone (initially 15 mg once a day tapered and maintained at 10 mg), tablet hydroxychloroquine 200 mg once a day and moisturizing ointments for local application.  After 6 months of diagnosis she was started on tablet nifedipine 10 mg once a day for sclerodermic ulceration of digits and tablet pentoxyfylline 400 mg thrice a day was added.
Around five months prior to the current pregnancy, a skin biopsy was taken from the right cheek circular lesion as it was not resolving with ongoing treatment. It showed paucibacillary lepromatous leprosy. She was started on multidrug treatment regimen which included tablet dapsone (100 mg OD), rifampicin (600 mg OD), clofazimine (300 mg OD). Tablet prednisolone was stopped and tablet azathioprine and nifedipine were continued.
She came to know about pregnancy status at around 12 weeks of gestation and registered at the same time in outside hospital. She stopped antimycobacterial treatment on her own but continued sclerosis medication. Ultrasonography for fetal malformation was normal. Blood pressure was always within normal limits.
In our hospital she registered at 36 weeks of gestation. She was advised admission for further management but she did not get admitted. As per dermatologists’ advise, she was restarted on multidrug treatment for paucibacillary leprosy. Cardiologists opinion was taken which showed normal echocardiography and pulmonologists suggested chest physiotherapy.
She was admitted at 41 weeks of gestation for induction of labor. On examination general condition was fair, afebrile, pulse was 80 per minute and blood pressure recorded was 120/80 millimeter of mercury. Cardiovascular and respiratory examination was within normal limits. Abdominal examination showed full term relaxed gravid uterus in vertex presentation with regular fetal heart sounds. Cervical internal os was closed and uneffaced. Preinduction cervical ripening was done with Foley’s catheter, following which labor was augmented.
She developed fetal distress and an emergency LSCS was done during first stage of induced labor. She delivered a healthy male child of 3.2 kilogram birth weight with Apgar score of 9/10. Post-delivery breast feeding was prohibited as per advice given by neonatologist.

Hansen's disease (HD) or Leprosy is a chronic granulomatous infectious disease caused by Mycobacterium leprae affecting the skin, mucous membranes and peripheral nerves. The worldwide prevalence rate of HD has decreased gradually over the years. The clinical manifestations of it are extensive which can mimic symptoms of many rheumatic diseases. It is rarely reported during pregnancy but can be exacerbated during pregnancy. It is important to treat leprosy during pregnancy as without treatment it can permanently damage the skin, nerves and eyes.[1]
Systemic sclerosis is a multi-systemic autoimmune disorder affecting predominantly the skin, lungs, gut and kidneys. Pregnancy in women diagnosed with systemic sclerosis is not a common condition but it has a favorable outcome according to the most recent studies.[2]
Our patient initially presented with gradual sclerotic skin change that is thickening of skin over forearms and sclerodactyly diagnosed on radiological imaging which is frequently observed in systemic sclerosis. Diagnosis was confirmed on doing immunological blood test. Later on a skin biopsy with acid-fast stain was done for a non-resolving skin lesion over face which confirmed lepromatous leprosy.
Systemic sclerosis in pregnancy can have complications like dyspnea, hypertension, gastroesophageal reflux disease and renal complications. The most common maternal complication is worsening of gastroesophageal reflux, and the most severe complications are renal crisis and flaring of arterial pulmonary hypertension.[3] But in our patient, none of the above has occurred during antenatal period. Obstetric complications include placental insufficiency, and these patients may benefit with aspirin, low dose anticoagulants or even nitric oxide donors. Our patient was started on aspirin and tablet nifedipine was continued.
The most important and interesting part of our case was the co-existence of systemic sclerosis and lepromatous leprosy.
Immunodeficient state is a risk factor for leprosy in pregnancy.[4] Our patient was on steroids and autoimmune medications, which is in an immunodeficient state.
Lepromatous leprosy, in general, has been reported to be a condition which is commonly misdiagnosed.  Systemic sclerosis is a condition with which it is commonly confused.[5,6] Salvi et al have reported a case series of leprosy cases occurring in a rheumatological settings, and had termed it at a challenging mimic, which is difficult to expose.[7]
Despite a thorough literature search, we have not been able to find any case report of a parturient suffering from a combination of systemic sclerosis and leprosy. We believe this may be the first such case report, and is hence reported here.
The patient in our case had an otherwise uneventful postpartum period. Breastfeeding is not allowed for those on anti-mycobacterial medications like dapsone.[4]
Though the neonate in our case had no problems, rare case reports exists which have recorded neonatal methemoglobinemia due to transplacental transfer of dapsone.[8]


The occurrence of lepromatous leprosy in pregnancy is rare. The occurrence of this in combination with systemic sclerosis is even rare. One such case is presented here.

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Nasare P, Prasad M, Gupta AS. A Case Of Pregnancy With Coexisting Systemic Sclerosis And Paucibacillary Leprosy. JPGO 2018. Volume 5 No.6. Available from: http://www.jpgo.org/2018/06/a-case-of-pregnancy-with-coexisting.html