|CASE REPORT |
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|Year : 1995 | Volume : 61 | Issue : 1 | Page : 48-49 |
Porokeratosis plantaris palmaris et disseminata
MPS Sawheny, V Mahatachar, YS Bisht
A 65-year-old man presented with typical lesions of porokeratosis palmaris et disseminata clinically and histopathologically. Although this disease has autosomal dominant mode of inheritance, no family history of similar illness was seen in our case.
|How to cite this article: |
Sawheny M, Mahatachar V, Bisht Y S. Porokeratosis plantaris palmaris et disseminata. Indian J Dermatol Venereol Leprol 1995;61:48-9
|How to cite this URL: |
Sawheny M, Mahatachar V, Bisht Y S. Porokeratosis plantaris palmaris et disseminata. Indian J Dermatol Venereol Leprol [serial online] 1995 [cited 2014 Mar 5];61:48-9. Available from: http://www.ijdvl.com/text.asp?1995/61/1/48/4130
| Introduction || || |
Porokeratosis has been classified variously by different authors., Porokeratosis plantaris palmaris et disseminata (PPPD) is the least common of all. Its seperate identity with an autosomal dominant mode of inheritance was established when Marschalko and Somali described a fourth family affected by this disorder. We describe here an isolated case of PPPD without any family history of similar disorder.
| Case Report || || |
A 67-year-old man presented with mildly itchy, hyperpigmented lesions over trunk for last 6 months. The number of skin lesions had increased tremendously for last 2 months. In addition, patient had numerous keratotic scaly lesions over both palms and soles for the last few years. There was no history of similar illness in any member of his family. Examination revealed numerous hyperpigmented annular plaques, 0.5-1.0 cm in diameter with central atrophy and raised edges. There was no furrow around the lesions. The lesions were distributed over scalp, forehead, neck, trunk and proximal parts of upper extremities [Figure - 1]. Patient also had multiple depressed hyperkeratotic lesions over both palms and soles, 1 to 3 mm in diameter with filiform keratinous raised margins.
Routine physical examination, haemogram and blood sugar estimation were normal.
Histopathological examination from a typical lesion at the back showed shallow keratin filled furrows and infiltration of few mononuclear cells in the underlying dermis.
| Discussion || || |
Porokeratosis is a chronic and progressive disorder of keratinisation. The following five clinical types have been described : Porokeratosis of Mibelli, also called classical porokeratosis (CP), linear porokeratosis, punctate porokeratosis, disseminated superficial actinic porokeratosis (DSAP) and porokeratosis plantaris palmaris et disseminata (PPPD).,
Guss et al were the first to describe PPPD as an entity distinct from DSAP with small papular and annular lesions 0.5-1.0cm in size over both the sun exposed as well as the covered areas of the body alongwith the involvement of palms and soles in a family. Subsequently four more families with atuosomal dominant mode of inheritance have been described by Brasch et al, Shaw and White, and Marschalko and Somali and Pavithran. Kaur and Singh have described an isolated case of PPPD variant with features of both PPPD and porokeratois of Mibelli in the Indian literature. We have described here another isolated case of PPPD in a male. This may be explained either by mutation or by variable penetrance of the family may be having forme fruste lesions of PPPD over palms and soles, since none of the other family members were available for examination.
Superficial disseminated porokeratosis described by Lever and Lever and classified separately from PPPD by Vineet Kaur and Gurpreet Singh and Pavithran is nothing but PPPD described initially by Guss et al.
| References || || |
|1. ||Congenital Diseases (Genodermatoses) : Porokeratosis. In : Histopathology of the Skin (Lever WF and Schamberg-Lever G, eds), 6th edn, Philadelphia : JB Lippincott, 1983;62-4. |
|2. ||Chernosky ME. Porokeratosis. Arch Dermatol 1986;122:869-70. [PUBMED] |
|3. ||Marschalko M, Somali B. Porokeratosis plantaris palmaris et disseminata. Arch Dermatol 1986;122:890-1. |
|4. ||Guss SB, Osbourn RA, Lutzner MA. Porokeratosis plantaris, palmaris et disseminata : A third type of porokeratosis. Arch Dermatol 1971;104:366-73. [PUBMED] |
|5. ||Brasch J, Scheuer B, Christopher E. Porokeratosis plantaris et disseminata. Hautarzt 1985;36:459-61. |
|6. ||Shaw JC, White CR. Porokeratosis plantaris palmaris et disseminata. J Am Acad Dermatol 1984;11:454-60. |
|7. ||Pavithran K. Superficial disseminated porokeratosis with diabetes mellitus. Ind J Dermatol Venereol leprol 1991;57:36-7. |
|8. ||Kaur V, Singh G. Porokeratosis palmaris et plantaris disseminata. Ind J Dermatol Venereol Leprol 1993;59:130-1. |
Figures [Figure - 1]