ECTOPIC CUTANEOUS SCHISTOSOMIASIS: REPORT OF TWO CASES AND A REVIEW OF THE LITERATURE

ECTOPIC CUTANEOUS SCHISTOSOMIASIS:

REPORT OF TWO CASES AND A REVIEW OF THE LITERATURE

José de Souza ANDRADE FILHO(¹), Maria Sueli S. N. LOPES (²),

Antônio A. CORGOZINHO FILHO (³) & Gil P. M. PENA (⁴)

SUMMARY

Two cases of ectopic cutaneous schistosomiasis are described. Both patients presented with abdominal papular skin lesions, which on biopsy were found to contain granulomas with Schistosoma mansoni eggs. Twenty-five other cases were retrieved from the literature. Most patients were female, mean age 24.9 year, with a predominance of the white race. The most common localization was anterior thorax and abdomen. Usually, the lesions were asymptomatic. In few cases, however, severe clinical syndromes due to the parasite coexisted, such as transverse myelitis or the acute-toxemic form of the disease. Intestinal infection was not frequently demonstrated in these patients. The importance of the recognition of these cutaneous lesions may rest on the opportunity to provide an etiological diagnosis in these difficult cases.

KEYWORDS: Schistosomiasis; Skin diseases.

INTRODUCTION

Adult Schistosoma mansoni worms are usually lodged in venules of the mucosa and submucosa of the colon and rectum. By virtue of this localization, ova are frequently found in the colon and liver. After the development of portal hypertension and the establishment of a portacaval shunt, they can also be found in the lungs.

Despite high endemicity of this parasitosis in several parts of the world, the finding of schistosome eggs or worms in other organs constitutes a rare phenomenon, sometimes with serious clinical complications, usually reported in the literature as pathological curiosities. The possible implications that these ectopic localizations may have on the comprehension of the parasite behavior within host environment has been given little attention.

We have had the opportunity to observe two cases of cutaneous ectopic localization of Schistosoma mansoni infection. We stress out the clinical aspects of this rare dermatologic affection, as well as possible implications these observations may have on the understanding of the parasite behavior within host.

CASE REPORT

Case 1

A 14-year-old white boy with history of intermittent fever, diarrhoea (10 evacuations/day), weight loss (about 12 kg in two months), was seen at the hematology section, in March, 1994, because of intense leukocytosis with eosinophilia. He also presented papular cutaneous lesions in periumbilical region bilaterally (Fig. 1), which appeared about 20 days earlier.

Fig. 1
- Clinical aspect of ectopic cutaneous schistosomiais (case 1). Randomly distributed papular skin lesions in periumbilical area, bilaterally.

In January, 1994, during a bath in Paraopeba river, in the city of Esmeraldas, State of Minas Gerais, he noted cutaneous pruritic lesions in the left foot. In retrospect, these lesions were presumed to represent cercarial dermatitis.

Laboratory studies revealed high erythrocyte sedimentation rate (89 mm/60 min), leukocytosis (WBC = 34100/mm³), with eosinophilia (25516 eosinophils/mm³), slight elevation of hepatic enzymes (SGOT = 50 UI/L and SGPT = 58 UI/L - normal range 8-25 UI/L; alkaline phosphatase = 182 UI/L - normal range 12-43 UI/L; GGT = 76 UI/L - normal range 19-24 UI/L).

Stool samples were repeatedly negative for Schistosoma eggs. An intradermal test was performed, also with negative result.

An abdominal echography showed hepatosplenomegaly and periaortic and portacaval lymphadenectasis.

Histopathological examination of periumbilical skin lesions revealed granulomas with Schistosoma mansoni eggs. (Fig. 2)

Fig. 2
- Schistosoma mansoni egg, with characteristic lateral spine in dermis from histopathological examination of periumbilical skin lesions (case 1).

Case 2

A 32-year-old white man presented with dermatologic lesions characterized as small, non-pruritic papules in left abdomen, extending from around umbilicus to left hypocondrium, which appeared three months before (Fig. 3). Any other symptom was denied. No history of previous contact with possibly infected waters could be assured. The patient reports a travel to a river shore, three years before, but denies any direct contact with the water. The lesions were suspected clinically to represent micronodular sarcoidosis, lichen planus or ectopic cutaneous schistosomiasis.

Fig. 3
- Clinical aspect of ectopic cutaneous schistosomiais (case 2). Papular skin lesions in left periumbilical area. Lesions are sometimes confluent with a slightly erythematous halo.

Histopathological picture was characterized by dermal granulomas containing schistosomal eggs, some of them calcified. Stool examination or rectal biopsy was refused by the patient.

COMMENT

In a review of the literature, 25 cases of ectopic cutaneous schistosomiasis mansoni could be retrieved (Table 1). Main demographic variables and other relevant characteristics are presented in Table 2. The majority of the patients were female with a predominance of the white race.

The majority of lesions presented in the anterior trunk (14/25). This occurrence is anatomically explained by the connections of portal and systemic venous circulation territories. On an analysis of the distribution of trunk lesions, one can easily recognize the patterns of distribution of engorged veins in collateral circulation in cases of portal hypertension. This may indicate that worms and eggs might have reached the cutaneous veins through naturally existing connections between systemic and portal venous territories. This distribution favours a previous passage of worms through portal territory before adult worms reach their ectopic oviposition place. In four cases, the lesions were located in the head^1,3,8,10. In one case, with associated transverse myelitis, the cutaneous lesion was located in the foot sole¹¹. In another case, also with associated transverse myelitis, the lesions were distributed in paravertebral area¹⁵. In three other cases, lesions were also located in posterior trunk^1,12. In one case, lesions coexisted in both anterior and posterior thorax¹².

The cutaneous lesions were described as papules. Papules were variously characterized as erythematous, clear, firm, slightly keratotic, skin-colored, hyperchromic or shiny. As a rule, the papules tended to coalesce, forming plaques. In one instance, the lesion was characterized as a hypopigmented plaque, with no discernible papule elements⁸.

Intestinal infection could only be detected in about one third of the cases, by means of stool examinations or rectal biopsy. However, rectal biopsy was performed in only four patients, and was positive in one, with previous negative stool examinations. Since rectal biopsy is considered more sensitive than stool examinations, a somewhat higher proportion of intestinal infection might be expected.

Incomplete data were available in respect to eosinophil counts of reported cases. In 15 cases, hematological status was stated; in six cases, eosinophilia was reported by the authors^1,4,6,7,14. In none of them, there were indications of an acute-toxemic form of the disease. The case 1, presented here, seems to be the first one to present the association of these two rare forms of the disease. The dramatic clinical course, together with negative intradermal and stool examinations, has led to the diagnostic hypothesis of a rare form of eosinophilic leukaemia. The skin biopsy, however, provided the opportunity for a prompt diagnosis.

A very interesting aspect of ectopic schistosomiasis is the apparent tendency of these patients to present ectopic localizations in other organs. This association was observed in three cases, in which cutaneous lesions presented in more unusual localizations. In two cases, in which ectopic cutaneous schistosomiasis was associated with medullary involvement, the cutaneous lesions were present in the foot sole¹¹ and paravertebral area¹⁵. In another case, the cutaneous lesion, located in the face, was associated with inflammatory ocular schistosomal disease¹⁰. A presumptive case with egg embolism to the brain was also reported⁴.

Other important aspect is the observation of a migratory pattern of cutaneous lesions observed in some cases^2,7. This may represent an evidence of the migratory activity of adult worms within host. Probably, this migratory activity also occur in its usual habitat, the colon venules. An abnormal migration related to incomplete or unsuccessful treatment does not seem to play a major role in the pathogenesis of ectopic cutaneous schistosomiasis. A history of previous treatment could be retrieved in only two cases^1,11.

RESUMO

Esquistossomose cutânea ectópica: relato de dois casos e revisão da literatura

Dois casos de esquistossomose cutânea ectópica são relatados. Ambos os pacientes apresentavam lesões papulares no abdômen que, ao exame histopatológico, mostraram presença de granulomas contendo ovos de Schistosoma mansoni. Vinte e cinco casos de esquistossomose cutânea ectópica foram encontrados descritos na literatura. A maioria dos pacientes era do sexo feminino, com idade média de 24,9 anos e predominância da raça branca. A localização mais comum foi o tórax anterior e abdômen. Freqüentemente, as lesões eram assintomáticas. Em alguns casos, no entanto, coexistiam alterações sistêmicas graves, relacionadas ao parasito, como mielite transversa e a forma aguda toxêmica da doença. Na maior parte dos casos, a infecção intestinal não pôde ser demonstrada. O reconhecimento destas lesões cutâneas pode ser importante por proporcionar oportunidade para um diagnostico etiológico nestes casos.

Received: 02 February 1998

Accepted: 11 May 1998

• 1. BITTENCOURT, A.; PINHO, O.; LENZI, H. L. & COSTA, I. M. C. - Extragenital cutaneous lesions of Schistosomiasis mansoni. Report of two cases. Amer. J. trop. Med. Hyg., 28: 84-86, 1979.
• 2. CONVIT, J. & REYES, O. - Ectopic skin lesions produced by Schistosoma mansoni Amer. J. trop. Med. Hyg., 22: 482-484, 1973.
• 3. COSTA, I. M. C.; MOREIRA, R. R. & MORAIS, M. A. P. - Esquistossomose cutânea ectópica. An. bras. Derm., 64: 183-184, 1989.
• 4. FINDLAY, F. H. & WHITING, D. A. - Disseminated and zosteriform cutaneous schistosomiasis. Brit. J. Derm., 85 (suppl. 7): 98-101, 1971.
• 5. FURTADO, T.; VIEGAS, A. C. & CUNHA, A. S. - Esquistossomose cutânea ectópica por Schistosoma mansoni In: REUNIĂO ANUAL DOS DERMATÓLOGOS LATINO-AMERICANOS, 5.. Buenos Aires, 1980. apud VALE, E. C. S. & FURTADO, T. - Esquistossomose cutânea ectópica por Schistosoma mansoni Med. cut. ibero lat.-amer., 15: 233-237, 1987.
• 6. GUIMARĂES, N. S. & SOUZA, A. P. B - Esquistossomose cutânea ectópica. Tręs novos casos. An. bras. Derm., 62: 101-103, 1987.
• 7. GUIMARĂES, N. S.; SOUZA, A. P. B.; DINIZ, M. R. & GUIMARĂES, N. A. - Esquistossomose cutânea ectópica. An. bras. Derm., 60 (supl. 1): 257-261, 1985.
• 8. JACYK, W. K.; LAWANDE, R. V. & TULPULE, S. S. - Unusual presentation of extragenital cutaneous schistosomiasis mansoni. Brit. J. Derm., 103: 205-208, 1980.
• 9. MACDONALD, D. M. & MORRISON, J. G. L. - Cutaneous ectopic schistosomiasis. Brit. med. J., 2: 619-620, 1976.
• 10. MILLIGAN, A. & BURNS, D. A. - Ectopic cutaneous schistosomiasis and schistosomal ocular inflammatory disease. Brit. J. Derm., 119: 793-798, 1988.
• 11. PATRUS, O. A. R. - Esquistossomose cutânea ectópica Belo Horizonte, 1974. (Dissertaçăo de Mestrado - Faculdade de Medicina da Universidade Federal de Minas Gerais).
• 12. PIVA, N.; CORREA, M. C. & DINIZ, V. - Lesőes dermatológicas na esquistossomose mansoni. In: CONGRESSO DA SOCIEDADE BRASILEIRA DE MEDICINA TROPICAL, 14., Joăo Pessoa, 1978. Resumo p. 116.
• 13. TORRES, V. M. - Dermatologic manifestations of schistosomiasis mansoni. Arch. Derm., 112: 1539-1542, 1976.
• 14. VALE, E. C. S. & FURTADO, T. - Esquistossomose cutânea ectópica por Schistosoma mansoni Med. cut. ibero lat.-amer., 15: 233-237, 1987.
• 15. WOOD, M. G.; SROLOVITZ, H. & SCHETMAN, D. - Schistosomiasis. Paraplegia and ectopic skin lesions as admission symptoms. Arch. Derm., 112: 690-695, 1976.

Publication Dates

• Publication in this collection
  20 Jan 1999
• Date of issue
  July 1998

History

• Accepted
  11 May 1998
• Received
  02 Feb 1998