Lucio phenomenon of leprosy LL type on pregnancy: a rare case.
Leprosy is a mycobacterial disease which manifestations, course, and prognosis are strictly associated with the patient's immune system. (1) Globally, the annual new case detection of leprosy continues to decline. (2) By 2012, Indonesia is the 3rd highest country for a new case of leprosy with East Java as the highest province for new cases of leprosy in Indonesia. (3) Lucio leprosy is manifested by ulcerative type of skin lesions occurring in diffusely infiltrative of lepromatous leprosy (LL) type. (4) This study reports a case of 7th month primigravida with Lucio Leprosy, without prior treatment using WHO-Multi Drug Therapy (MDT).
A 29 year old Indonesian female, 7th months primigravida presented to our emergency ward with a 4-month history of painful scarlet spots that darken and ulcerate on both of her hands and legs (Figures 1 and 2).
The patient was experiencing fever. The patient's eyebrows were lost (Figure 3) and her earlobes were thickened (Figure 4) 3 years ago.
No cardinal signs of leprosy such as hypopigmented or erythematous patches/plaques and sensory loss were found.
Slit-skin smear from earlobes revealed bacterial index (BI) 6+ with globi and morphological index (MI) of 7%. A skin biopsy from the leg's ulcer margin showed atrophic epidermis with Grenz zone, group of foam cells with some of lymphocyte cells, and vasculitis with endothelium occlusion of thrombus on the dermis. The subcutaneous fat showed lymphocyte cell infiltrations followed by proliferation of capillary blood vessels (Figure 5A). Acid-fast bacilli were seen prominently inside the blood vessels (endothelium) and on the subcutaneous fat in Fite-Farraco staining (Figure 5B). These findings concluded a Lucio phenomenon.
Polymerase Chain Reaction (PCR) method was performed to detect M. leprae DNA using the pF-LpR primer. Samples taken from the skin lesion and amniotic fluid showed a positive result (Figure 6).
Serological examination result of the patient's peripheral blood and umbilical cord blood using Enzyme Linked Immunosorbent Assay (ELISA) were taken during caesarean section as shown in Table 1.
WHO-MDT Multi Bacillary (MB) regimen was given along with dexamethasone 0-5 mg three times daily until the symptoms alleviate before it was changed to methylprednisolone 16 mg twice daily, paracetamol 500 mg three times daily, folic acid 1,000 mg once daily, and wound care. A caesarian section was performed on 40th week of pregnancy. Follow up serological examinations results using ELISA were collected from the patient after 7 months of WHO-MDT MB and the baby at 5 months and 1 year as shown in Table 1.
Leprosy in women indicates that it is not just access to health services that influences underreporting cases but also the illiteracy, low status, and other cultural factors. (4) LL may present as a diffuse variant, in which there are no circumscribed elements, i.e., nodules (lepromas), macules, or plaques, but instead characterised by diffuse and massive infiltration of the skin, known as diffuse LL or Lucio-Latapi leprosy (5) which were found in this patient.
Reports of serodiagnosis using PGL-1 have indicated that it is possible to identify 75-100% of MB patients. (6) Patients sometimes remain seropositive for many years after treatment, a possible cause of persistency is the continuing presence of dead or dormant bacteria inside the body. In leprosy patients with pregnancy, the large number of women who first presented with leprosy lesions in puerperium could be attributed to the increased visibility of leprosy lesions. The increase load of M. leprae is most frequently seen in the 3rd trimester of pregnancy. (7)
In this case, the anti-PGL-1 IgM and IgG titers of the patient's taken during caesarian section were highly over the cutoff limit, while the umbilical cord blood anti-PGL-1 IgM and IgG titers were below. Herein, placenta is considered a protective barrier toward fetomaternal transmission of M. leprae. The placenta presents multiple innate defenses against pathogens. Few pathogens can circumvent these barriers, and those that can do so at relatively low frequencies. (8)
M. leprae DNA was demonstrated in the amniotic fluid. Five percent of babies born from mothers with active leprosy had self-healing indeterminate leprosy under the age of 2 years, and also anti-M. leprae antibodies of class IgA, IgG, and IgM. The presence of IgA and IgM anti-M. leprae antibodies in the cord blood from newborns of leprosy mothers probably indicates intrauterine immunologic stimulation due to transplacental transmission of M. leprae antibodies. The levels of IgG anti-M. leprae antibodies showed a continuing and marked decrease at 3-6 months and 6-9 months and even 15-24 months after birth. (9,10) To support this theory, anti-PGL-1 IgM and IgG titers were reviewed again when the baby was 5 months old and 1 year old and we found that the titers were drastically decreasing, especially the anti-PGL-1 IgG titer. Based on these data, we assumed that the baby had the passive antibody to M. leprae from the mother's blood transmitted through the umbilical cord blood as showed by the presence of anti-PGL-1 IgG antibody.
(1) Thorat DM, Sharma P. Epidemiology. In: Kar HK, Kumar B (eds). Indian association of leprologist textbook of leprosy, New Delhi: Jaypee Brothers Medical Publishers Ltd, 2010, pp. 24-31.
(2) Smith WCS. Epidemiology of leprosy. In: Makino M, Matsuoka M, Goto M, Hatano K (eds). Leprosy: Science working towards dignity, Hadano: Tokai University Press, 2011, pp. 26-35.
(3) WHO. Global leprosy situation. Weekly Epidemiological Record, 2012; 87(34): 317-328.
(4) Bryceson A, Pfaltzgraff RE. Leprosy. 3rd edition, New York: Churchill Livingstone, 1990.
(5) Zenha EMR, Ferreira MAN, Foss NT. Use of anti-PGL-1 antibodies to monitor therapy regimes in leprosy patients. Bras J Med Biol Res, 2009; 42: 968-972.
(6) Lyde CB. Pregnancy in patients with Hansen's disease. Arch Dermatol, 1997; 133: 623 -627.
(7) Job CK, Sanchez RM, Hastings RC. Lepromatous placentitis and intrauterine fetal infection in lepromatous ninebanded armadillos (Dasypus novemcinctus). Lab Invest, 1987; 56: 44-48.
(8) Maurus JN. Hansen's disease in pregnancy. Obstet Gynecol, 1978; 52: 22-25.
(9) Melsom R, Harboe M, Duncan ME. IgA, IgM, and IgG anti-M. Leprae antibodies in babies of leprosy mothers during the first 2 years of life. Clin Exp Immunol, 1982; 49: 532-542.
(10) Khanna N. Leprosy and pregnancy. In: Kar HK, Kumar B (eds). Indian association of leprologist textbook of leprosy, New Delhi: Jaypee Brothers Medical Publishers Ltd, 2010, pp. 24-31.
CITA ROSITA SIGIT PRAKOESWA *, NANNY HERWANTO *, REGITTA INDIRA AGUSNI *, FRANSISKA RISMAULI NATALYA *, MUHAMMAD YULIANTO LISTIAWAN *, DINAR ADRIATY **, RATNA WAHYUNI **, ISWAHYUDI ISWAHYUDI ** & INDROPO AGUSNI *
* Faculty of Medicine, Universitas Airlangga, Dr. Soetomo General Hospital, Surabaya, East Java, Indonesia
** Institute of Tropical Disease, Universitas Airlangga, Dr. Soetomo General Hospital, Surabaya, East Java, Indonesia
Accepted for publication 5 October 2016
Correspondence to: Cita Rosita Sigit Prakoeswa, Faculty of Medicine, Universitas Airlangga, Dr. Soetomo General Hospital, Surabaya, East Java, Indonesia (e-mail: email@example.com)
Caption: Figure 1. Hyperpigmented macules and infiltrated plaques distributed bilaterally, symmetrical on both hands.
Caption: Figure 2. Hyperpigmented macules and infiltrated plaques distributed bilaterally, symmetrical on both legs.
Caption: Figure 3. Eyebrows loss.
Caption: Figure 4. Earlobes thickening.
Caption: Figure 5. (A) On the sub-cutaneous fat, there were lymphocyte cell infiltrations followed by proliferation of capillary blood vessels, Hematoxylin Eosin staining, 400X, (B) Acid-fast bacilli were seen prominently inside the blood vessels (endothelium) and on the subcutaneous fat, Fite-Farraco staining, 400X.
Caption: Figure 6. PCR examination; 1. Skin lesion, 2. Amniotic fluid, 3. Umbilical cord membrane, 4. Umbilical cord, 5. Negative Control, 6. Positive Control M. leprae Thai53, 7. DNA ladder.
Table 1. Results of serological examination using ELISA ELISA anti PGL-1 Cut off Subject IgM IgG IgM IgG Patient during the 4,854 U/mL 1,061 U/mL caesarian section Umbilical Cord Blood 0 U/mL 516 U/mL during the caesarian section Patient (after 7 1,912 U/mL 1,505 U/mL 605 U/mL 630U/mL months of therapy) Baby (5 month-old) 5 U/mL 1,724 U/mL Baby (1 year-old) 0 U/mL 3 U/mL
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|Title Annotation:||CASE REPORT|
|Author:||Prakoeswa, Cita Rosita Sigit; Herwanto, Nanny; Agusni, Regitta Indira; Natalya, Fransiska Rismauli;|
|Article Type:||Clinical report|
|Date:||Dec 1, 2016|
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