|Year : 1991 | Volume
| Issue : 1 | Page : 36-37
Superficial disseminated porokeratosis with diabetes mellitus
Source of Support: None, Conflict of Interest: None
A case of superficial disseminated porokeratosis is described in a middle-aged male. He had associated maturity-onset diabetes mellitus. Patient's father and elder brother also had skin lesions typical of porokeratosis and they too had associated diabetes.
Keywords: Porokeratosis, Diabetes mellitus, Disseminate porokeratosis
|How to cite this article:|
Pavithran K. Superficial disseminated porokeratosis with diabetes mellitus. Indian J Dermatol Venereol Leprol 1991;57:36-7
|How to cite this URL:|
Pavithran K. Superficial disseminated porokeratosis with diabetes mellitus. Indian J Dermatol Venereol Leprol [serial online] 1991 [cited 2020 May 31];57:36-7. Available from: http://www.ijdvl.com/text.asp?1991/57/1/36/3617
Porokeratosis of Mibelli is a rare genodermatosis characterized' by development of asymptomatic, circular, oval or polycyclic plaques with well-defined, keratotic raised border and central pigmented, atrophic areas. The border at its summit shows the characteristic gutter-like groove filled with loose keratin. Several variants of porokeratosis have been reported. They may be generalised or localised. The generalised types include 1. generalised eruptive type,  2. disseminated superficial actinic porokeratosis, 3. porokeratosis palmaris et plantaris disseminata and 4. superficial disseminate porokeratosis. 2 Here, we report superficial disseminate porokeratosis in a middle-aged male, who had associated maturity-onset diabetes mellitus. His father and elder brother also were found to have porokeratosis of similar type and they were also diabetics.
| Case Report|| |
A 46-year-old male developed generalised, asymptomatic, tiny, atrophic skin lesions since the age of 12. He was a known diabetic since 15 years. The skin lesions first appeared on the abdomen and thighs and gradually spread to the other parts of the body. Examination revealed numerous discrete, circular to oval shaped papules of size varying from 4 to 8 mm diameter, distributed bilaterally on the trunk, arms, thighs and legs. Each lesion had a slightly raised, well-defined, keratotic border and hyperpigmented, atrophic central area [Figure - 1]. On the summit of the keratotic border, a gutter like groove filled with scaly material was seen when examined with a hand lens. All other systems were clinically normal.
Routine laboratory tests on blood, urine and stools were normal except for pr 'sense of sugar in urine (0.5 gm%). Fasting blood sugar level was 140 mg% and postprandials 200 mg%. Blood VDRL was negative. Histopathological study of the biopsy specimen taken from the left forearm skin revealed hyperkeratosis with invagination of parakeratotic cell mass (coronoid lamella) into a depression in the epidermis. Below this the granular layer was thin. There was scanty lympho-histiocytic infiltration in the upper dermis [Figure - 2].
Patient's brother aged 48 and father aged 68, also were examined. Both of them were diabetics and they had skin lesions typical of superficial disseminate porokeratosis. Application of 0.1% retinoic acid cream for 2 months did not show appreciable therapeutic response in the patient.
| Comments|| |
The clinical picture of porokeratosis is so characteristic that elaborate laboratory tests are seldom required for its diagnosis. The characteristic well-defined, keratotic border of the lesion with presence of gutter-like groove on its summit, observed in our patient made the diagnosis easy. The presence of coronoid lamella in the histopathological study further supported this diagnosis. Bilateral symmetrical distribution, and superficial nature of the lesions suggested that it was superficial disseminate type of porokeratosis. Absence of lesions on sun-exposed areas like face and neck, easily differentiated it from superficial disseminate actinic porokeratosis. The palms and soles, which are characteristically involved in porokeratosis palmaris et plantaris disseminate, were spared in our patient.
Though different types of porokeratosis have been reported from India, only one case of superficial disseminate porokeratosis has been reported previously. Among 5 cases seen by Gharpuray et al, a 9 year old girl had superficial disseminate type of the disease.  Occurrence of porokeratosis in the patient's brother and father suggested autosomal dominant mode of transmission. Extensive medical workups have so far failed to disclose any clinical or metabolic concomitants.  But our patient and two other members in his family affected by porokeratosis had associated maturity-onset type of diabetes mellitus. The exact significance of this association is not known. Diabetes as well as porokeratosis is a dominantly inherited disorder. So their association as observed in our patient, is more than fortuitous.
| References|| |
|1.||Eng AM and Kolton B : Generalised eruptive porokeratosis of Mibelli with associated psoriasis, J Cut Pathol, 1975; 2 : 203-213. |
|2.||Lever WF and Schamberg - Lever G Histopathology of the Skin, 6th ed, JB Lippincott, Philadelphia, 1983; 62-64. |
|3.||Gharpuray MB, Deshpande SG, Kulkarni V et al Porokeratosis : Report of five cases, Ind J Dermatol Venereol Leprol, 1988; 54 : 255-257. |
|4.||Shelley WB : Consultations in Dermatology II with Walter B Shelly, WB Saunders Company, Philadelphia, 1974; 202-206. |
[Figure - 1], [Figure - 2]