Dermoscopy of porokeratosis: results from a multicentre study of the International Dermoscopy Society.

BACKGROUND: The diagnosis of porokeratosis can be challenging, and knowledge about its dermoscopic features is limited. OBJECTIVES: To describe the dermoscopic features of porokeratosis of Mibelli and disseminated superficial actinic porokeratosis (DSAP) and the frequency of these features in a larger case series. The interobserver concordance was also assessed. METHODS: In this retrospective cohort study, members of the International Dermoscopy Society contributed macroscopic and dermoscopic images of histopathologically verified cases of porokeratosis of Mibelli or DSAP. Three observers independently reviewed the collected images to identify the presence of predefined dermoscopic features. Following this, a consensus meeting was held to agree upon which dermoscopic features were present in each lesion. RESULTS: In total, 78 clinical and dermoscopic images of porokeratoses were collected. The most common dermoscopic feature was keratin rim, which was present in 74 lesions (92.3%). The most common vascular structures were dotted or glomerular vessels which were present in almost half of the cases (48.7%). Other relatively frequent dermoscopic findings were as follows: non-peripheral scales (44.9%), grey-brown dots or pigmentation along the keratin rim (38.5%), and light-brown pigmentation within the keratin rim (33.3%). Shiny white structures and blood spots or erosions along the keratin rim were findings never before described in porokeratosis and were detected in 16.7% and 17.9% of the lesions, respectively. Dermoscopic findings in porokeratosis of Mibelli and DSAP were similar except for fewer blood spots or erosions along the keratin rim and more light-brown pigmentation within the keratin rim in DSAP. The interobserver concordance ranged from 0.44 (moderate) to 0.84 (almost perfect). CONCLUSIONS: The dermoscopic hallmark of porokeratosis is the keratin rim, a finding also allowing for almost perfect interobserver agreement. Pigmentation or erosions along the keratin rim, vascular structures, as well as scales, pigmentation or shiny white structures within the keratin rim are additional dermoscopic clues.

A rare case of multiple variants of porokeratosis in the same patient: The clue of dermoscopy for diagnosis and therapeutical update.

Porokeratosis (PK) consists of abnormal keratinization of the epidermis of uncertain etiology and was first described by Mibelli in 1893. Multiple clinical variants of porokeratosis are recognized. The following is a case of a young male who presented more than one form of PK simultaneously. The hallmark of PK is the cornoid lamella, which can be identified in histopathology, and sometimes, as in our case, dermoscopy examination is the clue for diagnosis. This condition is often misdiagnosed and, therefore, not appropriately treated. Several treatment options are available and each clinical form may respond better to a specific therapy. However, consistency in treatment guidelines is still lacking.

Hyperkeratotic porokeratosis ptychotropica with satellite lesions: a rare presentation of an unusual variant of porokeratosis.

Since its description in 1995, porokeratosis ptychotropica (PP) has remained a less-recognized variant of porokeratosis (PK). The term porokeratosis ptychotropica was coined in reference to its characteristic of affecting body folds. It mimics many other dermatological diseases and is therefore often misdiagnosed. We report a patient with multiple hyperkeratotic, warty lesions across the buttocks that mimicked cutaneous tuberculosis (CTB), but histological examination confirmed the correct diagnosis of PP.

Craniosynostosis, delayed closure of the fontanelle, anal, genitourinary, and skin abnormalities (CDAGS syndrome): first report in a Mexican patient and review of the literature.

INTRODUCTION: Craniosynostosis and clavicular hypoplasia, delayed closure of the fontanelle, cranial defects, anal and genitourinary abnormalities, and skin (CDAGS), is an infrequent autosomal recessive entity with only 10 cases reported; no associated gene has been identified so far. CASE REPORT: The proband is a 2-year-old Mexican female with brachycephaly, cleft palate, anal malformation with rectovestibular fistula, and clinodactyly of the third toe overlapping the second. At 4 months of age, she developed a disseminated dermatosis with erythematous scaly nummular plaques, elevated keratotic sharp borders with thin to broad flaking, hematic crusts, and keratotic surface in others. The lesions were slightly pruritic and began at the lower limbs with posterior dissemination to the upper limbs, head, and trunk; palms and soles were unaffected. A skin biopsy showed hyperkeratosis, parakeratosis, acanthosis, and perivascular inflammatory infiltration in the upper reticular dermis among other alterations. She also presented mild bilateral neurosensory hypoacusia and enamel dysplasia. Her karyotype was normal. Treatment with topical hydrating creams partially improved the skin lesions at their center, while the sharply keratotic borders remained, giving a clinical resemblance to porokeratosis. DISCUSSION: The patient suffers from CDAGS syndrome but has normal development, and feet abnormality was described in only one other patient. The treatment with topical hydrating creams improved the skin lesions at their center, while porokeratotic characteristics persisted. CDAGS remains a diagnostic challenge; a comparison with previously reported cases is discussed. The timely detection of the syndrome will allow early treatment that may improve the condition of the patients.

Dermoscopy of Disseminated Superficial Actinic Porokeratosis.

We present a series of 6 cases of disseminated superficial actinic porokeratosis and describe their dermoscopic features. Dermoscopy is a noninvasive in vivo technique that is useful as a tool for the diagnosis and follow-up of porokeratosis. This condition has specific dermoscopic features that were observed in our series of cases and that are consistent with reports in the international literature.

Porokeratosis simulating Bowen's disease on dermoscopy

Abstract Porokeratosis is a disorder of epidermal keratinization characterized by the presence of annular hyperkeratotic plaques. Its etiopathogenesis is not yet fully understood, but a relationship with immunosuppression has been reported. Dermoscopic examination revealed a classic yellowish-white ring-like structure that resembled “volcanic crater contour” – the so-called cornoid lamella. We describe a case of porokeratosis in a female patient with chronic lymphedema, which was similar to Bowen’s disease due to the many glomerular vessels seen on clinical examination and dermoscopy.

Porokeratosis simulating Bowen's disease on dermoscopy.

Porokeratosis is a disorder of epidermal keratinization characterized by the presence of annular hyperkeratotic plaques. Its etiopathogenesis is not yet fully understood, but a relationship with immunosuppression has been reported. Dermoscopic examination revealed a classic yellowish-white ring-like structure that resembled "volcanic crater contour" - the so-called cornoid lamella. We describe a case of porokeratosis in a female patient with chronic lymphedema, which was similar to Bowen's disease due to the many glomerular vessels seen on clinical examination and dermoscopy.