|Year : 1990 | Volume
| Issue : 3 | Page : 211-212
Porokeratosis of mibelli
AS Prasad, Gurmohan Singh
A S Prasad
Source of Support: None, Conflict of Interest: None
Twenty two cases of porokeratosis of Mibelli belonging to two separate families having 18 and 4 cases respectively were studied. Their pedigress traced in three generations followed an autosomal dominant pattern.
Keywords: Porokeratosis of Mibilli, Family pedigree
|How to cite this article:|
Prasad A S, Singh G. Porokeratosis of mibelli. Indian J Dermatol Venereol Leprol 1990;56:211-2
Porokeratosis of Mibelli is a rare genodermatosis with an autosomal dominant inheritance. Males are affected more than females. A number of cases have been reported,, but the occurrence of this disease in three generations has not been reported so far from our country. This prompted us to report the present series.
| Material and Methods|| |
Fifty three persons from two different families were studied. A detailed record of their history and clinical examination was made. Clinical diagnosis is corroborated by two independent observers and further confirmed histopathologically. The pedigrees were traced in both families.
| Results|| |
The two families comprised of 42 and 11 members. There were 18 cases of porokeratosis of Mibelli in family A [Figure - 1], while family B had 4 cases [Figure - 2]. Most of the patients had lesions on extremities, face and genitalia. The lesions in all the cases were noticed in early childhood. No congenital abnormality or growth disturbances or muscle wasting was present in any of the cases.
| Comments|| |
The cases under review showed autosomal dominant pattern of inheritance and males were affected more than females.
In family A, case number 3 was married with case number 8. All their male offsprings (No.9 to 17) suffered from porokeratosis of Mibelli. Interestingly, all the members of this family took it as their familial ancestral mark and not a disease.
The family B comprised of 11 members, but only 4 of them suffered in three generations. Here also males were seen to out-number females.
The above observations indicate that since this disease is genetic and transmitted as autosomal dominant, the double dosing leads to the occurence of the disease in all the offsprings. In none of them it was severe.
| References|| |
|1.||Lever WF and Schaumberg Lever G Histopathology of Skin, 6th Ed, JB Lippincot Company Philadelphia, 1983; p 62. |
|2.||Rook A and Ebling FJG : Porokeratosis of Mibelli, Text book of Dermatology, Vol II, 3rd Ed, Blackwell Scientific Publications, Oxford, 1979; p 1311. |
|3.||Dey NC : Porokeratosis of Mibelli, Ind J Dermatol, 1945; 1:43. |
|4.||Venkatesan TV Porokeratosis of Mibelli and Ind J Dermatol, 1965; 10:53. |
|5.||Girgla HS and Bhattacharya SK : Clinical study of Porokeratosis : report of 10 cases, Ind J Dermatol, 1976; 15:43. |
[Figure - 1], [Figure - 2]