Disseminated superficial and warty type of porokeratosis: a rare coexistence.

A 60-year-old male presented with hyperpigmented annular plaques with raised keratotic wall and central groove along with warty plaque involving the trunk and the extremities, respectively. A provisional diagnosis of porokeratosis was made which was confirmed histopathologically. Herein, we report a case of rare coexistence of disseminated superficial and warty porokeratosis.

Introduction

Mibelli described classical porokeratosis in 1893.[1] Different clinical types have been reported: superficial disseminated, disseminated actinic, porokeratosis of Mibelli, punctate and hypertrophic and porokeratosis palmaris et plantaris disseminata. There are also reports of atypical presentation like hypertrophic, warty and nodular variety.[2] Mucous membrane, palms, soles, axillary vault and inguinal folds are not commonly involved.[1]

Case Report

A 60-year-old male presented with multiple itchy, hyperpigmented annular plaques over trunk, extremities, scalp, oral mucosa of 40 years duration and warty lesions on lower extremities of 10 years duration. To begin with, the lesions were small keratotic hyperpigmented papules which gradually enlarged to form a ring-like plaque with central atrophy surrounded by discrete ridge. On family screening, it was found that two of his four children had similar lesions.

Cutaneous examinations revealed multiple well-defined erythematous and hyperpigmented annular scaly plaques of size ranging from 1.5 to 5 cm, distributed on the scalp, face, axillae, trunk and extensor surface of the extremities [Figure 1]. Scalp lesions were associated with cicatricial alopecia. Buccal mucosa lesions showed maceration [Figure 2]. Some lesions had slightly raised keratotic wall with central groove that became accentuated by application of gentian violet. Hyperkeratotic warty plaques with minute ulcerations and post-inflammatory hypopigmentation were seen on extensor aspect of lower extremities.

Figure 1

Hyperpigmented keratotic scaly annular plaques and warty plaques with minute ulcerations on extensor aspect on lower extremities

Figure 2

Maceration seen over buccal mucosa

A provisional diagnosis of disseminated superficial porokeratosis along with warty type was made, which was confirmed histopathologically [Figure 3]. Routine blood investigations and biochemistry were within normal limits. Blood for HIV was negative.

Figure 3

Hyperkeratosis, parakeratosis, cornoid lamella, and absence of granular layer below the cornoid lamella (H and E staining, ×40)

Discussion

Disseminated superficial form was first described by Respighi.[3] It usually starts in third and fourth decades and is progressive over the years. Females are more commonly affected than males.[13] Areas such as cornea, oral mucosa and perianal region have been associated with porokeratosis.[4-6]

Atypical forms like warty porokeratosis have been described in literature.[7] There are also reports of disseminated porokeratosis with linear type and porokeratosis of Mibelli.[8-10]

The interesting feature of this case report is simultaneous occurrence of disseminated superficial porokeratosis and warty porokeratosis.To the best of our knowledge; this is the second case report of rare coexistence of disseminated superficial porokeratosis and warty porokeratosis.[11]