VOL. 26, No.3, MARCH, 1980

For the invasive type, which patho­physiologically acts like Shigella, an absorbable antibiotic might seem more appropriate.

(c ) Specific anti-diarrhoeals Paragoric and diphenoxylate with atropine (Lomotil) have Ibeen reported to prolong

fever, duration of diarrhoea and bacterial excretion in Shigella dysentery. Decreas­ing peristalsis increases the time availa'ble for organism proliferat;on, toxin produc­tion, and mucosal ,invasion. Recent evalua­tions of Kaopectate, and other kaolin -containing m;xtures, show them to be no more effective than a placebo in reducing frequency and water content of stools (Portnoy et al, 1967).

A summary of the suggested use of antibiotics in AGE is given in Fig. 2.

ACKNOWLEDGEMENTS

I am indebted to Mrs. S. Efstratiou for Fig. 1, and to Mrs. M. Sapseid and Miss C. Mash­anyare for typing the script.

REFERENCES

Brunser, 0., Reid, A., Monekeberg, F., Maeoioni, A. and Contreras, T. (1966), Pediatrics, 38, 605.

Creamer, B. (966), Postgraduate Gastro-enterology. Eds. Thomson, T. J. and Gillespie, T. E. Balliere, Tindall and Cassell, p. 16.

Deo, M. G. and RamaJ.ingaswami, V. (1965), Gastro­enterology, 49, 150.

Kent, T., Formal, S. and Labree, E. (1966), Arch. Path., 81, 501.

Klipstein, F. A. and Engert, R. F. (1967), In/ec. Imm., 13, 1307.

McNeill, L. L. and Hamilton, J. R. (1971), Pedia­trics, 47, 65.

Middleton, P. J., Szymanski, M. T. and Petrie, J. (1977), Am. I. Dis. Child., 121, 733.

Nelson, J. (1971), Pediatrics, 48, 248. Pensoert, M., Haelterman, F. 0., Burnstein, T.

(1970), Arch. Gesampte Virus forsch., 31, 321. Pmtnoy, B. L., Du Pont, H. L., Pruitt, D., Abdo, J.

A. and Rodr-lguez, J. T J. A. med. Assoc., 236, 884. Rodr.iguez, W. J., Kim, H. W. Arrobio, J. O. Brandt,

C. D., Chanoek, R. M., Kapikian, A. Z., Wya1Jt, R. G. and Parrott, R. H. (1977), I. Pediatrics, 91, 188.

Sakazeki, R., Tamura, K. and Saito, M. (1967), lap. med. Sci. Bio., 20, 387.

Taylor, J. and Bettelheim, K. A. (1956), j. Gen. Microbiology, 42, 309.

59

Tuft CENTRAL ArnlCAN JOVRN.U. OF MEDICJNK

Connective Tissue Disease in

Nigeria with Emphasis on

Scleroderma BY

A. O. SOMORIN,* F.R.C.P., F.M.C.P., F.W.A.C.P., D.T.M. II< H.,

D. DERM (LOND.). Section of Allergy and Immunology Clinic,

Head 0/ the Dermatology Unit, College 0/ Medicine, University 0/ Lagos,

AND

V. L N. MORDI, M.D., DTM & H, (HAMBURG),

F. PATH (GERMANY). Department of Morbid Anatomy,

Lagos University Teaching Hospital,

INTRODUCTION

Although inf¢etive dermatological diseases had been described in Africa (Verhagen et al 1959) information is scanty regarding the incidence of connective tissue diseases (C.T.D.) in Nigeria. It is therefore appropriate to review the pattern of these collagen diseases in an African population. Scleroderma, a member of C.T.D. is character­ised by connective tissue collagenisation either locally or systemically. However, Rodnan and Benedek ( 1962) had alluded to the higher incidence c4' systemic scleroderma among whites compared with black Americans.

In view of the reports on connective tissue diseases in Africans, a retrospective review of patients with collagenoses, attending the Al­lergy Clinic of the University Teaching Hrs­pital Lagos, Nigeria was undertaken. This paper:describes four of -the cases of scleroderma in this environment and emphasises the inci­dence and differential diagnosis of the disease. The first patient showed systemic type while the other patients demonstrated the localised va­riety.

CASE 1: History:

A. J., a fifty-six year old Nigerian house­wife was admitted on 30-3-73 with progressive body stiffness, weakness, painful peripheral joints, progressive scarring alopecia involving

* Communications to:-Dr. A. O. Somorin, Dermatology Unit, College 0/ Medicine, University of Lagos, P.M.B. 12003, Lagos, Nigeria.

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MARCH, 1980 CONNECTIVE TISSLTE DISEASES IN NIGERIA THE CENTRAL AFKlC.Uf

JOUflN.L Of' MWrCINI:

the scalp and pubic area, occasional palpita­tions, dysphagia for solids and Raynaud's phe­nomenon of the extremities.

Examination:

She was an ill-looking lady with alopecia of the sites mentioned above, mask-like, and cari­cature appearance of the face with pouting of the mouth. The skin of the face, upper chest, hands, forearms and feet was smooth, waxy and tethered down, and punctuated at various areas with pigmentary changes. There were painful, necrotic, ulcers on the small fingers and the lateral aspect of the (R) ankle, B.P. was 120/ 80.

Fig. 1. - The patient demonstrated dotted but dif­fu se hypopigmentary areas on the scalp, and the upper chest, while the alopeoia is also well depicted.

Other Investigations: Electrocardiagram : Sinus tachycardia, T. wave inversion in

III, V2, V3, Erythrocyte sedimentation rate: 54 mm/ hr; barium swallow and "follow through" showed normal oesophagus and small intestinal radiological appearances, Hb = 63%, WBC = 7,100 cells/ cm. Sickling: positive: Genotype AS, blood electrolytes, urea and urin­alysis were normal. Rheumatoid factor: posi­tive, LE cells: negative: antinuclear factor and tissue immunofluorescence were not done: fast­ing blood sugar: 74 mg%.

Physiotherapeutic joint management with short course of daily oral prednisolone and general nursing were given. Despite all efforts, she died six weeks after admission.

Skin Biopsy: "Oedema of the dermis with numerous thick hyal ine bands and dermal de­position of fibrin".

60

CASE 2: S.A., thirty-seven year old epileptic Nigerian

house-wife, with an unstable social background, maintained on Mysoline, and phenobarbitone for 5 years, progressively developed hyperpig­men ted, smooth, waxy areas on the face, back of trunk, thighs and arms, recurrent folliculi­tis, diffuse non-scarring alopec:a, dermatitis artefacta and recurrent wrist and (L) shoulder arthritis.

Fig. 2 . - Case 2. Showing multiple hypopigmented areas on the face and on the scalp.

Investigations:

ESR = 54 mm/hr, Hb = 12%, WBC = 2,500 celljmm, eosinophils = 15%, bilirubin =- 3 mg%, conjugated = 1.8 mg%, uncon­jugated = 1.2 mg%, skull, chest, barium meal and follow through X-rays were normal. Stool examination : E . histolytica; WR and Khan tests were negative. There were no L.E. cells in the peripheral blood, urinalysis: proteinuria, pus cells in the peripheral and granular casts + . Dental X-rays showed widening of the period­ontal membrance and dissolution of the lamina dura.

Skin Biopsy: "Organised dense irregular folds of coIlagen fibre depos:t in the dermis".

CASE 3:

A. 0., fifty-four year old Nigerian male trad­er, has been attending the Dermatological Out­patient Clinic since 1968, with a ten-year his­tory of hypop:gmented spots on the face, scalp and body.

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MARCH, 1980 CONNECTIVE TISSUE DISEASES IN NIGERIA THE CENTRAL AFRICAN JOURNAL 09 Mr.»lCINS

Examination: He was pale and depressed. There were

multiple, ivory, silvery macular areas on the hairy scalp, face, abdomen and back. There were no other abnormal signs. There was slight loss of pain sensation over the facial macular spots.

Investigations: Hb 77%, WBC = 7,900 cell/mm, eosinophilis 22%, Fasting blood sugar: 71 mg%, Cholesterol 250 mg%.

Skin Biopsy: "shows an epidermal layer of varying thickness and with melanin pigmenta­tions of the basal cells as well as some cells of the stratum spinosum while the collagen fibres of the corium shows dense irregular folds which in some areas have been separated by oedema. The corium contained a few sweat glands".

Progress and Treatment: He was treated with oral dapsone since the

provisional diagnosis then was "leprosy". The condition was reviewed in 1973 when there was no improvement and he was maintained on placebo drugs including cosmetic disguising solution (Dy-O-Derm solution Owens Ltd., Texas U.S.A.).

CASE 4: A twenty-one year old Nigerian male factory

worker was seen with a one-year history of scattered, hypopigmented areas on the face. Examination: He was pale looking and de­pressed. There were multiple ill-defined, waxy, ivory macular areas on the face, forehead, scalp and a few on the trunk.

Investigations: Heaf test: Grade II positive, Haemogram: Hb = 70%, WBC and ESR were within normal limits.

Skin Biopsy: "Showed a thick, epidermal layer and melanin deposit in the basal layer. There are increased dermal coHagen fibres with sparse lymphocytic infiltration. The sebaceous and sweat glands were m:nimaIIy present in the section".

RESULT

It will be observed that of the four Nigerians, three rf the patients presented with morphoea. Table 1, shows that of the 30 ind;viduals studied, 10 presented with localised scleroder­ma, 2 with progressive systemic scleroderma 7 with discoid lupus erythematous, 3 with sys­temic lupus erythematflsus and 8 patients form­ed the miscellaneous group (incIud;ng rheuma­toid arthritis, polyarteritis nodosa). This table also shows that morphoea is very common in males unlike patients with discoid lupus erythe­matsus.

61

I.

2.

3.

4.

Table I . Incidence of Connective Tissue Disease os Seen at Lagos University Teaching Hospital

. (1968-1971)

Disease No. of cases Percentage

Localised 10 Sclerodenna (·All Males) 33.3

Systemic 2 Scleroderma Females 6.7

Miscellaneous: Rheumatoid, Arthritis 8 Polyateritis Nodosa Females Collagenosis 3 Males 26.7

Systemic Lupus 3 Erythematosus Females 10.0

5. Discoid Lupus 7 Erythematosus Females 23.3

.. Connective Tissue Diseases form 1 % of the skin diseases seen during this period.

Table 2' shows that of the patients attending the hospital during this period only 1 % of der­matological consultations presented with connec­tive tissue diseases while 50% presented with bacterial fungal infections.

Table II Incidence of Skin Diseases

Seen at Lagos University Teaching Hospital (1968-1971)

Diseases Observed Incidence ----"'-'-'----

Bacterial Infections Fungal Infections Eczema/Dermatitis Psoriasis Lichen Planus Acne Vulgaris Connective Tissue Diseases Malignant Tumors

DISCUSSION

50% 28.5% 12% 0.2% 0.7% 1% 1% 0.1%

Sclerosing skin diseases are of common ocur­rence in Africans (Omodare 1972) even though, certain dermatoses are commoner in temper­ate areas than in tropical environment (Mar­shal 1959). Diseases of the connecfve tissue are not as commrn as infective dermatoses in a tropical environment (Verhagen et al 1969, Somorin 1973).

Even though the aetiology of these collagen­oses is unknown, certain hypotheses may ex­plain their rarity in this environment. Lupus erythematosus cells, anti-nuclear factor, (A.N. F.), are often seen in systemic scleroderma (Piper and Helwig 1955). Malaria antibody

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MARCH, 1980 CONNECTIVE TISSUE DISEASES IN· NIGERIA THE CENTRAL AFRTCAN JOUR ..... AL OF MEDICINE

and A.N.F., commonly present in the blood of certain Africans, probably prevent expression of L.E. cell phenomena in indigenous individ­uals from malaria endemic areas. No .antibodies against D.N.A. or R.N.A., had ever been found in renal glomeruli in nephrotic malarials (Al­lison and Houba 1974). Furthermore, malaria infection has an immunosuppressive ability (Greenwood & Greenwood 1971, Greenwood et al 1973). Finally the therapeutic role of chloroquine in systemic lupus -erythematosus (Goetz 1945, Harvey 1954, et al1962) may also explain the rarity of these diseases in malaria endemic areas.

Table 1 shows that localised scleroderma (morphoea is commoner than systemic form in Nigerian environment. It further shows that the localised form is the commonest connective tissue disease in th:s area. Table 2, reveals the apparent rarity of collagen diseases among the study population.

However, certain conditions in this locality may mimick scleroderma and confuse the prac­tising physician, thus creating clinical diagnostic problem:

1. Hansen's Disease (Indeterminate Type) : This is often the commonest condition sim­

ulating localised· scleroderma in tropical areas but the features of leprosy (e.g. nasal nodules, granulomatous histology and acid fast rods of Mycobacterium leprae in the skin scrapings) may confirm this imitator.

2. Lichen sclerosus et Antrophicus: This condition can be differentiated histolo­

gically by its homogenization of collagen fibres in the upper derm:s and a mid-dermal inflam­matory infiltration.

3. Vitiligo: This is often difficult to differentiate from

localised scleroderma but can be excluded by the very white skin with definite well-defined edges with or without white hairs of the latter. Histologically, there is no disturbance of the collagen fibres with epidermal and dermal lymphocytic infiltrations, the melanocytes . may be absent or present but with reduced tyrosinase activity.

4. Addison's Disease: This is rare in the tropics where it may be

due to tuberculosis. The presence of systemic manifestations of this disease; coupled wi1:h its hyper-and hypopigmentary changes differentiate it from scleroderma. The histology is like that of vitiligo.

62

5. Pinta: This is a treponema infection due to T.

carateum although it is supposed to be rare in this locality. It is characterised in its late stage, by multiple hypopigmented, vitiliginous, white uncollagen!sed lesions.

6. Scleroderma Adultorum of Buschke: . This is a rare acute condition, featured by

browny, waxy, non-pitting oedema of the skin, malaise, and myalgia. It can be differentiated, histologically by the accummulation of dermal mucopolysacchar'des with moderate perivascular mononuclear infiltrate and clinically by absent p:gmentary or atropic changes.

7. Lupus Erythematosus: In system:c lupus erythematosus (S.L.E.)

when there are acrosclerotic changes, mani­fested by aI'thralgia, acral ulcers, nail bed vas­cular changes, the condition may simulate sys­temic scleroderma_ Histologically there is fibn­noid necrosis, collagen sclerosis, vascular en­dothelial thickening and basophilic inclusion bodies.

In discoid lupus erythematosus there are sclerotic depigmented scarred areas with loss of ha:r. The lesions are often peripheral with or without a but-terflyappearance. Histolo­gically there is hyalinization of the collagen fibres, and degeneration of the basal layer.

8. Dermatomyositis: This is often difficult to differentiate from

systemic scleroderma clinically and histologi­cally, especially in advanced stages_ However the patient often presents with a heliotropic maculo-papular rash on the face, and around the eyelids with nail bed changes. Both derma­tomysitis and systemic scleroderma may mani­fest with dysphagia and joint involvement.

9. Pseudo-scleromatous Rheumatoid Arthritis: In ,the terminal phase of severe rheumatoid

arthritis, acrosclerotic involvement of the fingers and toes may occur but the necrobiotic histo­logy of the skin, differentiates this disease from progressive systemic sclerosis.

10. Onchocerciasis: This common disease may present with sago­

grain mottled pre-tibial hyper-and 'hypo-pig­mented sk;n changes, localised severe itching, with or without regional lymphadenopathy and dermal microfilaria. Histologically unlike scler­oderma, there is eosinophilic infiltrate in the dermis which may be perivascular. or perimi­crofilarial.

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CONNECTIVE TISSUE DISEASES IN NIGERIA THE CINntAL AFRICAN JOIJRNAL OF M£DICINE

The poor response of scleroderma to che­motherapeutic management is a measure of the lack of well defined aetiology of the disease. However, certain anti-connective tissue agents had been tried with little success (Zarafonetis 1962, Wells 1963, Monoyhan 1973), while corticosteroids chemotherapy had also been tried with moderate success in systemic sclero­derma (Rodnan & Fennell 1962). This study did show that symptomatic management is the hall-mark of treatment in localised scleroderma.

The prognosis of localised scleroderma is very good, since it rarely progresses to the systemic type, while in systemic scleroderma the future is grim (Rowell 1972). In an envi­ronment where leprosy -is very common, the localised disease can easily be misdiagnosed (Batchvarov & Jantjie 1971) leading to socio­logical and psychological consequences of lep­rophobia.

SUMMARY

The apparent rarity of systemic connective tissue diseases as shown in 'the present retro­spective four-year study (1972-1976) among patients attending the University Teaching Hospital, Lagos, Nigeria, is presented. Of the 30 collagenoses patients seen, there were 12 with scleroderma, 10 with lupus erythematsus and 8 with the miscellaneous type. In partic­ular, it was observed that localised scleroderma (morphoea) was more common among this s~udy population during this period. The diag­nostic similarity of certain tropical dermatoses to this collagenoses in clinical hospital practice _ forms the high-light of this report.

ACKNOWLEDGEMENT

The technical assistance of the Medical Il­lustration Department of the College of Me­dicine is gratefully acknowledged. The typo­graphical assistance of Mrs. T. Shittu is highly appreciated.

REFERENCES

Allison, A. C., Houba, V. (1976). In Textbook of Immunology of Parasitic Infections. Edit. Cohen, S., Sad un, E. Pub. Blackwell Scientific Publications, London, p. 445.

Batchvarov and Jantjie V.: Personal Communication. Goetz, R. H. (1945). GUn. Proc. (Cape Town),

4, 337. Greenwood, B. B. & Greenwood, A. M. (1971). Trans.

Roy. Soc. trop. Hyg. 65, 581. Greenwood, B. B., Bradley-Moore, A. M., Palh, A.,

Bryceson, A. M. M. (1973). Lancet, 169. Greenwood, B. M., & Voller, A. (1970). GUn. Exp.

Immunol. 7, 793. Marshall, J. (1959). Diseases of the Skin, Edit. J.

Marshall, Printed by E. & S. Livingstone Limited., Edinburgh.

Harvey, A. M. et al (1954). Medicine, 33, 291. Omodare, P. (1972). Thesis (University of Binning­

ham) "Keloids in West Africa". Piper, W. N. & Helwig, E. B. (1955). Arch. Derm.

72, 735. Rodnan, G. P., Fennell, R. H., Jr.: (1962). J. Amer.

med. Assoc., 180, 665. Rodnan, G. P.·&, Benedek, T. G. (1962). Ann. into

Med., 57, 305. Rowell, N. R. (1972). On scleroderma in Textbook

0/ Dermatology, Vol. 2. Ed. Rook Wilkhson & Ebling, P. 1097.

Somorin, A. O. (1973). Int. ]. trop. Derm., 12,332. Verhagen, R. R. H. B., Maniar, S. H. & Vanbre­

ughem (1969). Trans. Roy. Soc. trop. Med. and Hyg. 63, 2: 275.

Wells, R. S. (I963). Trans. Rep. St. John's Hosp. Denn. Soc. Lond., 49, 148.

Zarafonites, C. J. D. (1962). Ann. J. Med. Sci., 243, 147.

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