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On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.79 no.2 Rio de Janeiro Mar./Apr. 2004
The Lucio's phenomenon (necrotizing erythema) in pregnancy*
Karin Adriane HelmerI; Isabela FleischfresserII; Luciana D. Kucharski-EsmanhotoIII; José Fillus NetoIV; Jesus Rodriguez SantamariaV
IIResident M.D., 3rd year of Dermatology
IVAdjunct professor of Pathology - Federal University of Parana
VAssistant professor of Dermatology - Federal University of Parana
The Lucio's phenomenon, a type 2 reactional condition in leprosy probably mediated by immune complexes, is a severe necrotizing skin reaction that occurs mainly in patients with non-nodular lepromatous leprosy. This report presents a 27-year-old woman, in her 32nd week of pregnancy, with a one-week history of painful skin lesions in extremities, reddish-purple, sharply delineated, confluent, with bullae and occasional necrosis and ulceration. The patient also referred fever. Baciloscopy showed acid-fast bacilli and globi, and the histopathologic findings of a skin biopsy were consistent with lepromatous leprosy and Lucio's phenomenon. Prednisone and multidrug therapy with rifampin, clofazimine and dapsone were given, with remission. Pregnancy has been associated with a high incidence of first diagnosis of leprosy or with an exacerbation of symptoms in patients with the established disease because hormonal alterations cause immunological imbalance, particularly between the last three months of pregnancy and the first three months of lactation, when immunosuppression is higher. Despite the recommendation not to take drugs during pregnancy, the multidrug therapy regimen must be used, since the benefits achieved with the treatment surpass the risks.
Keywords: erythema; pregnancy; leprosy.
Leprosy is a chronic infectious disease caused by the acid-fast bacillus Mycobacterium leprae, transmitted by inhalation following exposure of a person with an untreated bacilliform infection, usually during intimate and prolonged contact. Depending on the patient's immunity, the infection may develop toward recovery or the clinical forms of the disease: indeterminate, tuberculoid, dimorphic or lepromatous, each of which may course with episodes of acute inflammation called "reaction or reactional states". Type 1 reactions are mediated by cellular immunity and clinically present neuritis or a reverse reaction. Those of type 2 involve the immunocomplex and cytokine production, such as tumoral necrosis factor alpha,1 and include erythema nodosum leprosum, multiform erythema, Lucio's leprosy phenomenon and neuritis.2
Lucio's leprosy phenomenon was described by Lucio and Alvarado in 1852 in Mexico, and was so named in 1948 by Latapi and Zamora.3 It represents a variant of lepromatous reaction type 2 and histopathologically is characterized by acute necrotic vasculitis, being synonymous with the expression "necrotizing erythema".2 This can occur in Lucio's leprosy (where it forms a pure and primitive lepromatosis) and in other forms of lepromatous leprosy.4,5
The exacerbation of leprosy at the end of gestation and in the puerperium was described for the first time by Ryrie, in 1938, who declared: "Leprosy does not present any effects on the course of the pregnancy, however, the pregnancy exercises an important effect on the course of the leprosy".6 Since then, gestation has been associated with the high incidence of onset of the first signs or aggravation of leprosy.
This work presents the case of a 27-year-old pregnant woman, in the thirty-second week of gestation, with ulcerous necrotic lesions in the extremities, diagnosed as diffused non-nodular leprosy with vasculitis of Lucio's leprosy phenomenon.
The authors herein present a 27 year old female patient, 32 weeks pregnant, who had reported a burn with scalding water on the left leg seven months previously, resulting in a lesion of difficult cicatrization. Two weeks earlier, a secondary infection had presented itself in that area, there had been no improvement despite having used cefazolin, heparin and symptomatic medication. It developed with the appearance of purpuric lesions in the inferior and superior members and in the abdomen, accompanied by pain and fever.
Regarding personal history, this was her second gestation and she did not refer any problems during the first. She denied smoking, alcoholism or drug addiction. When questioned about family antecedents, she affirmed that she was not aware of similar occurrences.
At admission she was in a regular general state, with stable vital signs and afebrile. A dermatological exam showed infiltrated facies with telangiectasia and madarosis (Figure 1), edema and extensive erythema in the members, with disseminated and confluent erythematous-purpuric lesions, topped by blisters with well-defined borders, some with ulcerated and necrotic areas (Figures 2 and 3) and bilateral inguinal enlarged lymph nodes.
Laboratory exams showed hypochromic microcytic anemia, leukocytosis with deviation to the left, increase in the erythrocyte sedimentation rate, anti-HIV, VDRL and non-reagent anticardiolipin antibodies.
Bacilloscopy of the lymph revealed acid-fast bacilli with the formation of globi, bacilloscobic index = 1, with 5% complete bacilli, and 95% granular bacilli. Examination of the biopsy of apparently healthy skin but with lesions using hematoxylin and eosin stain showed leukocytoclastic vasculitis with fibrinoid necrosis (Figure 4), foamy histiocytes occupying the lobular portion of the hypodermis (Figure 5) and presence of numerous granular bacilli determined by Fite-Faraco (Figure 6). This pattern is compatible with Virchow's leprosy and Lucio's leprosy phenomenon.
Multibacillary polychemotherapy was initiated with rifampicin 600mg/month, clofazimine 300mg/month and 50mg/day and dapsone 100mg/day administered with prednisone 60mg/day, which was reduced progressively over an eight-week period, with good clinical results (Figure 7).
The gestation developed to term for a normal childbirth, without concurrent disease. The newborn was of appropriate weight for gestational age and without malformations.
Lucio's leprosy phenomenon represents a serious cutaneous necrotizing reaction that can occur in Lucio's leprosy and also in other forms of lepromatous leprosy.4,5
Lucio's leprosy is most common in Mexico and Central America and little found in other ethnic groups,2,7 it is characterized by diffuse infiltration of the skin, madarosis and loss of eyelashes, giving "a swollen, edematous look" to the skin.4 Generally, three to four years after onset of the disease3 and usually in untreated patients,7 Lucio's leprosy phenomenon establishes itself with the emergence of painful erythematous discoloration that courses into necrosis and ulceration.3,8 This development is ascendant (inferior members, superiors, buttocks, trunk and, with lesser frequency, the face)9 and resolves in a period that varies from two to four weeks, leaving atrophic scars.
In other forms of lepromatous leprosy, Lucio's leprosy phenomenon is characterized by necrosis in lesions of nodular or polymorphic erythema that appear in the course of a lepromatous reaction in patients with nodular or non-nodular lepromatous leprosy.4,5 Although Brazil occupies second place in the world according to the number of cases of leprosy, reports of Lucio's leprosy phenomenon are rare.10
Laboratory findings always present anemia, leukocytosis and deviation to the left. Examination of the lymph generally presents a high bacilloscobic index4,8 (not found in the described case, probably due to a technical flaw while performing the tests). In the histology, infiltrated inflammation with foamy histiocytes are observed together with vascular damage due to direct invasion by M. leprae into endothelial cells7 causing vasculitis and thrombosis of the superficial and deeper vessels with consequent hemorrhage and infarction of the skin. Acid-fast bacilli can be detected with Ziehl or Fite-Faraco stains.3,4,10 These findings are observed as much in the clinically altered skin as in the apparently healthy skin.2
Treatment consists of polychemotherapy for multibacilli and sometimes systemic corticoids for control of the reactions. Unlike erythematous nodular leprosy, Lucio's leprosy phenomenon when it occurs in Lucio's leprosy does not present a good response to thalidomide.9,11
Pregnancy has been associated with a high incidence of onset of the first signs or aggravation of leprosy. The critical period is considered to be between the last quarter of gestation and the first three months of nursing due to hormonal, metabolic and immune system alterations. In gestation cellular immunity is relatively suppressed, mainly in the third quarter, leading to the unchaining or worsening of type 2 reactions, as in the described case. There is also the risk of recurrence of the disease.12 In the puerperium there is a relative suppression of the humoral immunity, with a greater risk for the development of type 1 reactions beginning between the third and sixteenth week of postpartum.6
Newborn whose mothers presented leprosy during pregnancy tend to weigh less than the mean for the general population12 and present a higher incidence of respiratory problems,13 due to placental inadequacy and consequent intra-uterine growth retardation. Nevertheless there is no conclusive controlled study that provides evidence of complications in a gestation as a result of having been treated with polychemotherapy.6 Although the newborn may present intercurrent disease, such as foleate dermatitis in the first hours of life due to the sulfone and impregnation of clofazimine in the skin, which presents a brownish coloration.12
Women should be encouraged to treat leprosy with polychemotherapy before considering a future pregnancy.13 In a recent publication, the Ministry of Health (Ordinance 1073-26 Sept. 2000),14 recommended the treatment of pregnant women with leprostatics (rifampicin, clofazimine and dapsone) in the standard regimen, despite the usual recommendation of restricting the ingestion of drugs in the first quarter of pregnancy. It was considered that the benefits of the treatment outweigh the risks.
The clinical picture, laboratory exams and histopathology in the described case confirm the diagnosis of non-nodular lepromatous leprosy with Lucio's leprosy phenomenon. The gestation being associated with the diagnosis of infection and to the virulent reactional state type 2, which was probably related to the physiologic immunosuppression of this period. q
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Karin Adriane Helmer
R. Padre Anchieta, 1721 - apto. 102
80730-000 Curitiba PR
Tel.: (41) 336-0161
in August, 17th of 2001
Approved by the Consultive Council and accepted for publication in October, 28th of 2002
* Work done at "Hospital de Clínicas da Universidade Federal do Paraná".