Porokeratosis: An enigma beginning to unravel

Porokeratosis is a keratinization disorder with unclear etiopathogenesis, varied clinical presentation and characteristic histopathology, and is usually unresponsive to current therapeutic options. Until now, it was considered to be a clonal disorder with immunity, ultra violet radiation and other factors playing important roles in etiopathogenesis. It is now known that abnormalities in the mevalonate pathway are responsible for this clonal keratinization abnormality. New variants of porokeratosis like eruptive bullous, pruriginous, lichen planus like, follicular variants and porokeratoma have been described. While the cornoid lamella is the classical histopathologic feature, dermoscopy and reflectance confocal microscopy make the diagnosis clearer. Development of malignancy in a few variants is a concern. Linear, disseminated superficial actinic and giant lesions are most prone to developing malignancies. Bowen’s disease, squamous cell carcinoma, basal cell carcinoma and even melanoma have been reported in cases of long-standing porokeratosis. Newer modalities of therapy such as photodynamic therapy, ingenol mebutate and HMGCoA inhibitors may play a role in the future

Erythematous Nodules on Face: A Dermatologist’s Dilemma.

Granuloma faciale (GF) is a chronic inflammatory disease of unknown origin characterized by nodular or granulomatous lesions on the face in adults. It is now regarded as a variant of vasculitis in which eosinophils are particularly numerous. It remains a benign, though chronic condition, not associated with systemic lesions like other forms of vasculitis. We report a case of 24-year-old male who presented with multiple erythematous nodules on photoexposed areas since past 2 years. The patient had been previously treated for Hansen’s disease for 3 months but with no relief. The patient responded wonderfully to intralesional triamcinolone and topical tacrolimus after two initial failed attempts with intralesional cryotherapy.

Spectrum of purpura fulminans: Report of three classical prototypes and review of management strategies.

Purpura fulminans is a rare syndrome of intravascular thrombosis and hemorrhagic infarction of the skin that is rapidly progressive and is accompanied by vascular collapse and disseminated intravascular coagulation. It usually occurs in children, but this syndrome has also been noted in adults. The three forms of this disease are classified by the triggering mechanisms. We describe three classical cases of purpura fulminans of the three classical prototypes treated at our center and their varied clinical outcomes. We also describe a case of acute infectious purpura fulminans secondary to systemic leptospirosis which to our best knowledge is the first reported case in world literature. The various treatment options for purpura fulminans have also been reviewed.