Facial lesions resembling leprosy
To the Editor:
Leprosy is a chronic granulomatous disease which leaves deformities if not treated early and adequately. The manifestations of early lepromatous leprosy and its late complication of nasal deformity are well-described (4). However, the diagnosis of leprosy should not be established when convincing evidence is not found because leprosy still carries significant stigma in the society, despite years of health education campaigns. Two patients, one with presenile sebaceous gland hyperplasia and the other with late yaws, who were believed to have leprosy, are reported.
CASE REPORTS
Case 1. A 61 -year-old Chinese male was referred to the skin clinic as a case of lepromatous leprosy with "leonine facies." He had bright, erythematous rashes on the face, especially on the cheeks, forehead and earlobes (Fig. 1). The face was "oily" with loss of hair on the lateral aspects of the eyebrows. There was thickening and furrowing of the forehead. Sensory and motor functions of the face were intact. There wereneither cutaneous lesions in other parts of the body nor any tender or enlarged peripheral nerves on palpation. A slit-skin smear from the usual sites and a repeat one from the earlobes and the cheeks were negative for acid-fast bacilli (AFB). A skin biopsy showed a greatly enlarged sebaceous gland consisting of numerous lobules grouped around a wide sebaceous duct. There was dermal infiltration of a few mononuclear inflammatory cells and no AFB on Fite stain. He was diagnosed as a case of sebaceous gland hyperplasia. He was given isotretinoin 20 mg daily which produced dramatic improvement in his disorder; it was reduced and stopped within 5 months and he is on follow up.
Fig. 1. Erythematous rashes on the face. Note thickening, of the earlobe and furrowing of the foreheal.
Case 2. A 71-year-old Malay female was referred as a case of "burnt-out" leprosy with saddle nose deformity. There was no history of leprosy or syphilis, but she grew up in a village where patients had yaws which is locally called "penyait puru." However, she could not remember whether she had symptoms of yaws while she was young. She presented with gross destruction of the nose and the palate resulting in gangosa and also with bilateral bony swelling on the sides of the nose known as goundou (Figs. 2 and 3). She had a few thin scars on the legs but no other obvious abnormalities. A venerai disease research laboratory (VDRL) test was reactive in a 1-8 dilution and the Treponema pallidum hemagglutination (TPHA) test was positive. She was given a single dose of intramuscular benzathine penicillin. She did not go for checkup at the ENT clinic and screening of the family could not be done.
Fig 2. Gangosa and gondou.
Fig 3. Lateral view of the face.
There are several conditions which resemble leprosy (2). These two patients presented with features suspicious of leprosy. The lirst one had partial madarosis and swelling of the earlobe; thickening and hollowing of the forehead which mimicked leonine fades. There was no nerve deficit since even in lepromatous leprosy the manifestation of nerve damage is a late phenomenon. The significant features of this case was the evidence of sebaceous gland hyperactivity and the absence of AFB in the smear as well as in the tissue. Presenile sebaceous gland hyperplasia is a progressive disease, has an early onset at puberty and occurs sporadically or in families on the face, especially on the forehead and cheeks, resulting in the skin thickened and furrowed but sparing the periorbital and perioral regions (6). It is seen more in males and associated with excessive sebum secretion, suggesting a probable androgen activity. Isotretinoin, a synthetic retinoid, is effective even in a low dose, as in the present case. It inhibits sebaceous gland differentiation and shrinks them (1).
In the second case, the thin scars on the legs indicate that she might have contracted yaws at an early age, since she is from an area which was endemic for yaws during the Japanese occupation of the then Malaya in the 1940s (3). Yaws is rare now since the mass penicillin campaign by the World Health Organization in 1950s (3) but late cases are seen sporadically (4). Gangosa or mutilating rhinopharyngitis and goundou or subperiosteal deposition of new bone over the nasal processes of the maxilla are features of late yaws (4). The serological tests for syphilis are positive since yaws is caused by Treponema pertenue which is similar to Treponema pallidum. The three granulomatous disorders which affect the bones and cartilages producing especially the destruction of the nasal septum, resulting in saddle nose deformity, are leprosy, syphilis (congenital) and yaws. These patients highlight the importance of establishing the diagnosis of leprosy before starting treatment and also to be aware of yaws, although it is eradicated.
-Kader B.Mohamed, M.B.B.S.,
Dip.Derm.. Dip. Ven.
Department of Dermatology
General Hospital
10990 Penang, Malaysia
REFERENCES
1. Grekin, R. C. and Ellis. C. N. Isotretinoin for the treatment of sebaceous hyperplasia. Cutis 34(1984)90-92.
2. Mohamed, K. B. Dermatologieal disorders resembling leprosy. Sing. Med. J. 30(1989)265-268.
3. Mohamed, K. B. Imported yaws in Johore, Malaysia. Ann. Trop. Paediatr. 8(1988)222-224.
4. Mohamed, K. B. Late yaws and optic atrophy. Ann. Trop. Med. Parasitol. 84(1990)637-639.
5. Pealtzgraff, R. E. and Bryceson, A. Clinical leprosy. In: Leprosy. Hastings, R. C.. ed. Edinburgh: Churchill Livingstone, 1985, pp. 134-176.
6. Waranya. B. and Vichit. L. Familial presenile sebaceous gland hyperplasia. J. Am. Acad. Dermatol. 36(1997)120-122.