Itakura Hideyo

Itakura, Hideyo

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Environmental Pathology in the Tropics : Diseases of Uncertain Etiology and Environmental Diseases (Part I)

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Trop．Med・，35（3），125－129，September，1993　　　　　　　　　　　　　　　　　　　　　125Review ArticleEnvironmental Pathology in the Tropics: Diseases ofUncertain Etiology and Environmental Diseases (Part I )Hideyo ITAKURADepartment of Pathology, Institute of Tropical Medicine,Nagasaki University, Nagasaki 852, JapanSome endemic diseases in the tropics suggest that environmental factors, ethnicalpredisposing factors, and human ecological factors exert an influence upon disease manifesta-tions. This paper is a preliminary trial of a new geopathological approach to diseases ofuncertain etiology, and to environmental diseases in the tropics.Familial Mediterranean Fever and Related Conditions:Also called benign paroxysmal peritonitis, periodic peritonitis, periodic arthrosis,familial recurrent polyserositis, and periodic or recurrent polyserositis. The disease ischaracterized by severe recurrent attacks of fever with acute abdominal pain (Siegal, 1945;Andrade, 1952; Sohar et al., 1967). Pain in the chest or joints and erythema resemblingerysipelas are occasionally seen. It is sometimes complicated by certain forms of primaryamyloidosis on a genetic basis, in which the deposits of fibrillar protein are of the AA type.In some cases, the attack resulted in the formation of residual fibrous adhesions which lateroccasionally caused further intra-abdominal complications. The disease occurs in certainfamilies and is thought to be transmitted by a single recessive autosomal gene. Clear cutgeographical distribution of familial Mediterranean fever and amyloid peripheral neuropathyin the Portuguese have been recognized. It is a hereditary disease usually occurring in Arme-nians and Sephardic Jews. It very rarely affects Arabs and Maltese. Most of the affectedSephardic Jews had formerly been resident in the North African littoral or in Iraq, and itdoes not affect Jews of Yemeni or Persian origin.These facts suggest that familial Mediterranean fever is an endemic disease andmanifestations of the disease are influenced by ethnical predisposing factors and ecologicalfactors.Tropical Ulcer:Also called tropical phagedenic ulcer, ulcus tropicum, tropical phagedena, tropicalsloughing phagedena, Aden ulcer, Malabar ulcer, Cochin sore, and Naga (Nagana) sore.Various native names but only geographical significance have been given to the tropical ulcer(Basset, 1969; O'Brien, 1976). The tropical ulcer is a chronic, often progressive, sloughingulcer, usually occurring on the lower extremities. The pathologic change in the ulcer is essen-tially a necrosis of the skin and subcutaneous tissues. It may extend deeply, with destructionReceived for Publication October 12, 1993Contribution No.2876 from the Institute of Tropical Medicine, Nagasaki University126of underlying muscles, tendons, periosteum and bone. Squamous cell carcinoma may arise asa rare complication. It is necessary to take into account in the differential diagnosis of othervarious chronic ulcerations of tropical countries: framboesia (yaws), tuberculosis,leishmaniasis, histoplasmosis, and Buruli ulcer caused by Mycobacterium ulcerance. The cor-rect diagnosis depends upon early recognition of the characteristic features in thehistopathological picture. The etiology is unknown but spirochetes, fusiform bacilli, and otherbacteria are often present in the affected lesion. Protein and vitamin deficiency with loweredresistance to infection may be important in the etiology. It is widespread throughout thetropical areas and is particularly prevalent in malnourished children in the wet tropics.Ainhum:Also called dactylolysis spontanea ("to saw" in African). Ainhum is a condition markedby linear constriction of one or both of the little toes and may ultimately result inpathological amputations. A linear constriction around the affected digit leads to spontaneousamputation of the distal part of the digit. Very rarely the 5th finger, index finger or thumbmaybe affected (Findlay, 1951). The condition was reviewed by Browne (1961). It is prac-tically confined to the darker skinned people in tropical countries, mainly in Africa, Northand South America and the West Indies, though it has also been reported occasionally amongnon-negro races in India and Polynesia. It occurs more frequently in males and chiefly inadult life. It is rare to see the condition in persons who habitually wear shoes. Life styleseems a more important to consideration in the pathogenesis. Other examples of disease ofdactylolysis spontanea are also seen in leprosy.Tropical Eosinophilia:Also called tropical pulmonary eosinophilia, pseudotuberculosis of the lung withmassive eosinophilia, eosinophilic lung, pulmonary eosinophilia, benign eosinophilic leukemia,eosinophilia with pulmonary disease, tropical eosinophilic asthma, Frimodt-Moller and Bartondisease, and Weingarten's disease or syndrome. The condition is characterized clinically byleukocytosis with an absolute increase in eosinophils, by spasmodic bronchitis with coughing,anorexia and malaise. Routine blood counts of patients show high eosinophilia varying from20 to 90%. It is characterized by diffuse eosinophilic infiltrations of the lung, and episodicnocturnal wheezing and coughing. The etiology is possibly a subacute or chronic form of oc-cult filariasis, usually involving Brugia malayi or Wuchereria bancrofti, occurring in thetropics (Webb et al.t 1960; Donohugh, 1963; Pacheco and Danaraj, 1963; Neva and Ottensen,1978; Ottensen et al., 1979). Microfilariae are seldom detected in peripheral blood films sincethe parasites are confined primarily to the lungs. Tropical eosinophilia is a disease occurringin certain parts of India, Sri Lanka, certain parts of Africa, China, the Philippines, Samoa,West and East Malaysia, the West Indies, and a few other areas. In India, it affects chieflyAsiatic Indians, in whom it may represent a genetic predisposition.Idiopathic Elephantiasis:Also called non-filarial elephantiasis (Oomen, 1969a, b; Price, 1972) and lymphostaticverrucosis (Clark, 1948). Elephantiasis is a chronic disease characterized by inflammation andobstruction of the lymphatics followed by remarkable fibrosis of the skin and subcutaneous127tissues. Verrucosis is a condition marked by the presence of multiple warts, or verrucaewhich are lobulated hyperplastic epidermal lesions with a horny surface. The foot is principal-ly affected, with enlargement, thickening, discoloration, and fissuring of the skin, and occa-sionally a formation of ulcers. Lymphoedema of the foot or leg is a common occurrence inmanyparts of the tropics. The term 'mossy foot' is often used to describe the late results oflymphoedema which are characterized by hyperkeratosis of the overlying epithelium givingrise to a mossy appearance. Idiopathic elephantiasis is a disease without any obviousetiology. However, such a picture is seen in the lymphoedema of filariasis, in mycetoma, andoccasionally in Kaposi's sarcoma.Desert Sore:Synonyms are veld sore, Barcoo rot, Gallipoli sore, and Umballa sore. Also called byvarious native names and by names having only geographical significance (O'Brien, 1972).This is a form of ulcer resembling the varicose ulcer and appearing on the face, back ofhands and forearms, or on the lower extremities, especially on the knees and shins. Thisphagedenic ulcer is characterized initially by the development of papulovesicular lesions thatrupture and form painful crusted purulent ulcers. The condition probably represents an in-fected ulcer from some secondarily antecedent neglected lesion. Etiology is uncertain. It oc-curs in various desert regions of Africa, Australia and the Near East. Its relationship totropical ulcer is not established.Tumoral Caldnosis:Also called exudative calcifying fasciitis and calcium gout. The disease is a conditionmarked by the deposition of calcium salts in nodules in various tissues of the body, especiallyin the subcutaneous tissues, muscles, tendons, and nerves. The lesions are characterized by adevelopment of periarticular masses, usually present as slowly growing tumors over pressurepoints such as the shoulder, elbows, buttocks or hips. They may be multiple and vary from 1to 8 cm in diameter. Involvement of underlying muscle is not uncommon.Features oftumoral calcinosis are frequently multilocular cysts of fluid containing hydroxyapatite [3Ca3(P04)2-Ca(OH)2] crystal clusters with marked calcification surrounded by a fibrous capsuleand giant cells (Thomson and Path, 1966; James et al, 1976). McClatchie and Bremner(1969) described some of these larger calcifying foci as arising in degenerative bundles of col-lagen. The occurrence of benign calcified lesions in the subcutaneous tissues around the hips,buttocks and shoulder girdle was described by Inclan (1943). The disease is marked by symp-toms such as sciatica due to pressure on adjacent nerves. Some cases have been diagnosedby routine radiography (Palmer, 1966). The pathogenesis of these lesions is unknown, butmust be related to local factors, as there is no disturbance of calcium metabolism. It is ofunknownetiology, with the onset usually in the first or second decade of life. Palmer (1966),and McClatchie and Bremner (1969) reported a number of cases from different parts ofAfrica. The majority of cases occurs in African people, but other ethnic groups may be af-fected. In the tropics it is important to differentiate this condition from calcification of a deadwormin the subcutaneous tissue. A calcified degenerative Onchocerca and calcification in bur-sae may simulate this lesion (Maathuis and Koten, 1969). A different case but with similar128manifestations has been reported in Japan (Kubo et al., 1992). Ethnical factors and life style,especially food habits are important to find out the pathogenesis.Eclampsia:Also called toxemia of pregnancy. Toward the end of pregnancy, salt retention, edema,albuminuria, and hypertension develop in about 6% of women.This syndrome is called tox-emia of pregnancy or preeclampsia. The most severe manifestation of eclampsia is accom-panied by convulsions and coma, but rarely coma alone, occurring in a pregnant or puerperalwoman,associated with hypertension, edema, and proteinuria. There are extensive in-travascular coagulations with fibrin thrombi and focal necroses in the liver (Arias andJimenez, 1976) and other organs, kidneys, and brain, and jaundice (Iber, 1965).Histopathological findings of the liver in eclampsia are fundamentally edematous change;sinusoidal dilatation and inflammatory exudate with Kupffer cell mobilization. Liver cellcords are atrophic and show irregular structures and pleomorphism of liver cell nuclei.Degenerative or necrotic liver parenchyma are observed in intermediate and outer zones ofhepatic lobules, while centrilobular areas remain intact. Eclampsia is a condition occurring inmalnourished mothers in the tropics. Not only genetic predisposition but also socioeconomicconditions are important to obtain the pathogenesis.ACKNOWLEDGMENTSThe author is grateful to Dr. A. Makatta and Dr. M. Rahaman for helpful commentsand advice on the manuscript in English.REFERENCES1 ) Andrade, C. (1952): A peculiar form of peripheral neuropathy. Familiar atypical generalizedamyloidosis with special involvement of the peripheral nerves. Brain, 75, 408-427.2 ) Arias, F. & Jimenez, R.M. (1976): Hepatic fibrinogen deposits in pre-eclampsia. N. Engl. J. Med.,295, 578-582.3 ) Basset, A. (1969): Tropical phagedenic ulcer, pp. 25-33. In Simons, R.D.G.Ph. & Marshall, J. (ed.).Essays on Tropical Dermatology. Vol. I. Excerpta Medica. Amsterdam.4 ) Browne, S.G. (1961): Ainhum. A clinical and etiological study of 83 cases. Ann. Trop. Med. Parasit,55, 314-320.5 ) Clark, M. (1948): Lymphostatic verrucosis in the Fort Hall district of Kenya. Trans. Roy. Soc. Trop.Med. Hyg., 42, 287-290.6 ) Donohugh, D.L. (1963): Tropical eosinophilia. An etiologic inquiry. N. Engl. J. Med., 269,1357-1364.7 ) Findlay, G.H. (1951): Ainhum with special reference to involvement of fingers. Trans. Roy. Soc.Trop. Med. Hyg., 44, 747-754.8 ) Iber, F.L. (1965): Jaundice in pregnancy-a review. Am. J. Obst. & Gynec., 91, 721-753.9 ) Inclan, A. (1943): Tumoural calcinosis. J. Am. Med. Ass., 121, 490-495.12910) James, W., Harkess, M.B., & Ch, B. (1976): Tumoral calcinosis: A report of six cases. J. Bone JointSurg., 49 (A), 721-731.ll) Kubo, K., Manabe, J., Matsumoto, S., Kawaguchi, T., & Kitagawa, T. (1992): Unusual huge in-tramuscular granuloma with calcium phosphate crystal deposition in the buttock. Acta Pathol. Jpn.,42,508-511.12) Maathuis, J.B. & Koten, J.W. (1969): Kikuyu-bursa and tumoral calcinosis. Trop. Geogr. Med., 21,389-396.13) McClatchie, S. & Bremner, A.D. (1969): Tumoural calcinosis - an unrecognised diasease. Brit. Med.J., I, 153-161.14) Neva, F.A. & Ottensen, E.A. (1978): Tropical filarial eosinophilia. N. Engl. J. Med., 298,1129-1131.15) O'Brien, J.P. (1972): Desert Sore, pp.610-611. In Hunter, G.W., HI, Frye, W.W. & Swartzwelder,J.C. (ed.). A Manual of Tropical Medicine. W.B. Saunders Co., Philadelphia.16) O'Brien, J.P. (1976): Tropical ulcer, pp.666-667. In G.W. Hunter, IE, J.C. Swartzwelder & D.F.Clyde (ed.). Tropical Medicine. 5th ed. W.B. Saunders Co., Philadelphia.17) Oomen, A.P. (1969a): A reconsideration of the problem of elephantiasis. Trop. Geogr. Med., 21,225-235.18) Oomen, A.P. (1969b): Studies on elephantiasis of the legs in Ethiopia. Trop. Geogr. Med., 21,236-253.19) Ottensen, E.A., Neva, F.A., Paranjape, R.S., Tripathy, S.P., Thiruvengadam, K.V. & Beaven, M.A.(1979): Specific allergic sensitization to filarial antigens in tropical eosinophilia syndrome. Lancet, I,1158-1161.20) Pacheco, G. & Danaraj, TJ. (1963): Ethanol extracts of various helminths in a complement fixationtest for eosinophilic lung (tropical eosinophilia). Am. J. Trop. Med. Hyg. 12: 745-747.21) Palmer, P.E.S. (1966): Tumoral calcinosis. Brit. J. Radiol., 39, 518-525.22) Price, E.W. (1972): The pathology of non-filarial elephantiasis of the lower legs. Trans. Roy. Soc.Trop. Med. Hyg., 66, 150-159.23) Siegal, S. (1945): Benign paroxysmal peritonitis. Ann. Intern. Med., 23, 1-21.24) Sohar, E., Gafni, J., Pras, M. & Heller, H. (1967): Familial Mediterranean fever: a survey of 470cases and review of the literature. Am. J. Med., 43, 227-253.25) Thomson J.G. & Path, F.C. (1966): Calcifying collagenolysis (tumoural calcinosis). Br. J. Radiol., 39,526-532.26) Webb, J.K.G., Job, C.K. & Gault. E.W. (1960): Tropical eosinophilia. Demonstration of microfilariaein lung, liver, and lymph nodes. Lancet, 1, 835-842.

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Environmental Pathology in the Tropics : Diseases of Uncertain Etiology and Environmental Diseases (Part I)

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