Porokeratosis

Diagnosis: Porokeratosis

Porokeratosis is a genodermatosis (autosomal dominant) Porokeratosis is typically seen as a keratotic plaque surrounded by a raised hyperkeratotic border Histologically, border corresponds to the cornoid lamella Differential diagnosis includes other entities that may have cornoid lamella such as seborrheic keratosis (SK), verruca vulgaris, and squamous cell carcinoma (SCC) Six clinical types of porokeratosis exist: [ ]

Clinical Presentation

Porokeratosis - Porokeratosis is a genodermatosis (autosomal dominant) - Porokeratosis is typically seen as a keratotic plaque surrounded by a raised hyperkeratotic border - Histologically, border corresponds to the cornoid lamella - Differential diagnosis includes other entities that may have cornoid lamella such as seborrheic keratosis (SK), verruca vulgaris, and squamous cell carcinoma (SCC) - Six clinical types of porokeratosis exist: - 1. Plaque-type porokeratosis ("Classic porokeratosis" and "Porokeratosis of Mibelli") is characterized by skin lesions starting as small, brownish papules that slowly enlarge to form irregular, annular, hyperkeratotic or verrucous plaques. Can grow up to 10 cm or more. Key fact = Squamous cell carcinomas have been reported to develop in Mibelli's type porokeratosis over partianal areas involving anal mucosa. This was the first report mentioning mucosal malignancy in any form of porokeratosis. - 2. Disseminated superficial porokeratosis is a mo

Clinical History

Annular plaque with raised keratotic border. Sun-exposed distribution for DSAP. Immunosuppression history. Monitor for malignant transformation.

Treatment

Topical 5-fluorouracil, imiquimod, topical retinoids, diclofenac gel. Cryotherapy. Photodynamic therapy. Sun protection. Regular surveillance for dysplasia.

Differential Diagnosis

• Actinic keratosis • Psoriasis • Tinea corporis • Granuloma annulare • Bowen disease • Lichen planus

Key Learnings

• Disorder of keratinization with characteristic cornoid lamella (column of parakeratotic cells) • Classic: annular plaque with raised, thread-like peripheral ridge • Subtypes: classic of Mibelli, disseminated superficial actinic (DSAP), linear, punctate, palmoplantar • DSAP is the most common variant — multiple small lesions on sun-exposed areas • Malignant transformation to SCC/BCC occurs in ~7-10% of long-standing lesions • Immunosuppression exacerbates disease and increases malignancy risk

Tags: case, dermatologist, dermatology, genodermatoses, genodermatosis, kodachrome, kodachromes, porokeratosis, skin lesion