Presentations of Cutaneous Disease in Various Skin Pigmentations: Porokeratosis.

Description Porokeratosis was first described in 1893. It is a relatively rare disorder with over 9 subtypes. Lesions are clinically characterized as well-demarcated, erythematous papules (raised, <1 cm) or plaques (raised, >1 cm), with an atrophic center, and raised scaly border. Porokeratosis is an important diagnosis to identify because it may undergo malignant transformation and mimics many commonly encountered diagnoses. These commonly mimicked diagnoses include squamous cell carcinoma, tinea corporis, nummular dermatitis, and psoriasis vulgaris, to name a few. The clinical images in this review focus on identifying porokeratosis along the full spectrum of skin tones.

Challenges in the Diagnosis and Management of Giant Porokeratosis: A Case Report.

Porokeratosis encompasses a diverse group of dermatoses, both acquired and genetic, marked by a keratinization disorder. Porokeratosis of Mibelli (PKM) presents as solitary plaques or multiple papules/macules with central atrophy and raised hyperkeratotic borders. Here, we present a case of giant porokeratosis (GPK), a rare form often considered a morphological variant of PKM, with unique clinical and histopathological aspects. Our case involves a 29-year-old patient with a 15 × 10 cm irregular plaque on the dorsal aspect of the right hand. The patient was previously prescribed various topical treatments (retinoids, calcineurin inhibitors, and combinations of corticosteroids with vitamin D3 analogs) and systemic retinoids without improvement before presenting to our department. Due to the high risk of neoplastic transformation and the unavailability of imiquimod, the patient was recommended topical 5-fluorouracil treatment. The trajectory of the lesion under treatment revealed a favorable evolution, and the patient was subjected to regular monitoring every three months to assess the ongoing progress. Recognizing GPK as a high-risk variant is crucial for dermatologists, and it requires a personalized approach. Regular monitoring is advised to detect potential malignant transformations promptly. Future research holds promise for diagnostic advancements, refined treatment modalities, and a deeper understanding of the molecular mechanisms underlying malignancy in porokeratosis.

Giant Porokeratosis: Report of Three Cases.

Giant porokeratosis (PK) is a rare entity. Many consider it as a variant of PK of Mibelli, while others describe it as a separate variant. The diameter may range between 10 and 12 cm and the wall of hyperkeratotic ridge may be upto 1 cm. High tropical temperature and long duration of outdoor activities without adequate clothing are known influencing factors of PK. To the best of our knowledge, only five cases of solitary giant PK and a case of disseminated giant PK have been documented from India. We report three cases of giant PK for their rarity.

Porokeratosis: A Review of Its Pathophysiology, Clinical Manifestations, Diagnosis, and Treatment. / Poroqueratosis. Revisión de su etiopatogenia, manifestaciones clínicas, diagnóstico y tratamiento.

Porokeratosis comprises a group of heterogeneous and uncommon acquired or congenital skin diseases of unknown origin characterized by a keratinization disorder resulting from abnormal clonal expansion of keratinocytes. Numerous genetic mutations are thought to be involved. These conditions are characterized histologically by the presence of a cornoid lamella. Clinical manifestations are variable, with localized, disseminated, and even eruptive forms. Porokeratosis has been associated with immunosuppression, ultraviolet radiation, and systemic, infectious, and neoplastic diseases. Many authors consider it to be a premalignant condition because of the potential for malignant transformation to squamous cell or basal cell carcinoma. Therefore, long-term follow-up is a key component of treatment, which is usually complex and often unsatisfactory. We review the latest advances in our understanding of the pathogenesis, diagnosis, and treatment and propose a treatment algorithm.

[Circumscribed palmar hypokeratosis (CPM): The diagnostic value of dermoscopy]. / Hypokératose acrale circonscrite (HAC) : utilité diagnostique de la dermoscopie.

BACKGROUND: Circumscribed palmar hypokeratosis (CPH) is a rare skin disease, first described in 2002, associated with sudden localized reduction of the corneal layer. In most cases, it presents as an isolated rounded erythematous palmar lesion on the thenar eminence. We describe the dermoscopic semiology of CPH in 3 cases. PATIENTS AND METHODS: Three patients between the ages of 59 and 72 presented very limited erythematous lesions suggestive of CPH. Dermoscopic examination of these lesions provided similar findings. Biopsy, which was performed in one patient, confirmed the diagnosis of CPH. RESULTS: Two dermoscopic elements of CPH are characteristic: (1) the sides of the lesion have a "stair step" or "geological strata" type of configuration, and the thickness of the different strata varies; (2) the centre of the lesion showed a homogeneous erythematous area with a vascular pattern composed of dotted vessels of the superficial dermis and sometime vascular loops. DISCUSSION: These dermoscopic aspects are characteristic and enable CPH to be differentiated from Bowen's disease or porokeratosis of Mibelli. In Bowen's disease, there is no stair step like aspect to the sides of lesions; further, the centre of the lesion shows glomerular vessels (coiled vessels) and/or globular vessels (small red clods). In porokeratosis, peripheral keratotic "white track" structures comprise a single pigmented channel or a double white line. There is no "stair step" or central vascular pattern. CONCLUSION: The dermoscopic semiology of CPH is highly characteristic and enables differentiation from Bowen's disease and porokeratosis.

Coexistence of Porokeratosis of Mibelli and Disseminated Superficial Actinic Porokeratosis / 대한피부과학회지

No abstract available.

Recalcitrant Digital Porokeratosis of Mibelli: A Successful Surgical Treatment.

CONTEXT: Porokeratosis of Mibelli (PM) is a rare, benign, asymptomatic, epidermal hyperkeratinization dermatitis that is characterized by annular plaque that expands through the edges and leaves an atrophic center. Many therapies have been attempted for the treatment of PM, but none of these have given satisfactory results. The efficacies of treatment options are limited, and currently there is no gold standard. CASE REPORT: This paper reports the case of a 22-year-old female with 3-years history of PM, who had not responded to routine therapies like topical corticosteroids, topical tretinoin, topical salicylic acid, and various emollients and keratolytic agent. Cryosurgery and laser ablation did not have acceptable response. We used surgical treatment with successful cosmetic outcome. CONCLUSION: The treatment of PM should be individualized considering the aesthetic and functionality, and the patient's preferences. Complete surgical excision for isolated digital PM had good results.

Cutaneous sarcoidosis simulating porokeratosis of Mibelli.

We report a skin localization of systemic sarcoidosis, which presented with lesions that resemble porokeratosis of Mibelli. Skin biopsy showed non-caseating sarcoidal granuloma. Whereas cutaneous sarcoidosis is present in up to one-third of cases and may present with a wide variety of lesions, our presentation is uncommon. Partial remission was obtained with hydroxychloroquine and prednisone.

A Rare Case of Porokeratosis of Mibelli on the Scrotum / 대한피부과학회지

No abstract available.

Poroceratose de Mibelli com lesões gigantes: relato de caso / Porokeratosis of Mibelli with huge injuries: case report

Poroceratose de Mibelli é uma genodermatose disceratósicade forma crônica, progressiva e rara, comrisco de evolução para malignidade. É de transmissãoautossômica dominante e a patogenia é ainda desconhecida.O caso clínico mostra um paciente com 78anos de idade, com 75 anos de evolução de Poroceratosede Mibelli com lesões gigantes, exuberantes ehistória médica familiar da genodermatose. As lesõesda poroceratose de Mibelli são assintomáticas e maiscomumente encontradas nas extremidades, preferencialmentedorso das mãos e pés. Pode apresentar progressãoe regressão espontânea e ocasionar cicatrizeslevemente atróficas. O diagnóstico é clínico-histológico,apresentando a lamela coróide no histológico. A policeratosede Mibelli deve ser tratada para evitar a transformaçãoem células escamosas, doença de Bowen oucarcinoma basocelular.

Porokeratosis of Mibelli is a chronic, progressiveand rare genodermatosis disceratosica, that has therisk to become a malignant tumor. Its transmissionis mainly autosomal and the pathogenesis is stillunknown. The clinic case shows a patient with 78years, 75 years of those with evolving Porokeratosisof Mibelli, huge injuries, and familiar historic ofgenodermatosis. The injuries from the Porokeratosisof Mibelli are asymptomatic and generally found inthe ends of the body, preferably in the back of thehand or feet. The injuries can increase or diminishspontaneously and lead to scars a little atrophic.The diagnosis is clinical-histological, and it is representedby a choroid lamella in the histological. ThePorokeratosis of Mibelli may be treated to avoid thetransformation into squamous cells, Bowen diseaseor basal cell carcinoma.

Porokeratosis of Mibelli with Amyloid Deposits in Dermis / 대한피부과학회지

Porokeratosis of Mibelli, a chronic heritable disorder of the skin, presents with characteristic histopathological features including cornoid lamella as a result of faulty keratinization. Amyloid deposits are characterized by the accumulation of ultrastructually fibrillar material and have been observed in inflammatory and tumoral skin diseases. Some cases of amyloid deposits in the upper dermis in various subtypes of porokeratosis have been reported, but no porokeratosis of Mibelli reports are available in Korea. We report a case of a 26-year-old woman with three verrucous hyperkeratotic plaque lesions on her right popliteal fossa with histological feature of hyperkeratosis, cornoid lamella, and dermal amyloid deposits.

Coexistence of porokeratosis of Mibelli with Gardner's syndrome: A rare case report.

Porokeratosis represents a heterogeneous group of disorders characterized clinically by a distinctive ridge-like border and histologically by cornoid lamellae. Gardner's syndrome, a variant of familial adenomatous polyposis (FAP), is an autosomal dominant disease characterized by colorectal polyps, osteomas, epidermoid cysts and soft tissue tumors. Here we report a case of 18 yr old female who presented with porokeratosis of Mibelli with osteoma, multiple epidermoid cytsts, and solitary rectal polyp.

Three Cases of Porokeratosis of Mibelli Treated with 5% Imiquimod Cream / 대한피부과학회지

Porokeratosis is a disorder of epidermal keratinization that is histologically characterized by the presence of cornoid lamella. Many treatment modalities have been used, such as, surgical excision, cryosurgery, CO2 laser, oral etretinate, topical 5-fluorouracil and keratolytics. However, universal treatment guidelines have never been established. We tried 5% imiquimod cream on three patients who have porokeratosis of Mibelli which are intractable to other previous treatment modalities. Two of them showed a marked and moderate improvement, respectively. But the third patient exhibited only slight response. Herein we report our treatment experience and review the related literature.

A Case of Porokeratosis of Mibelli in the Glans Penis / 대한피부과학회지

We report a case of a 44 year-old man with porokeratosis of Mibelli, who presented with a 10 year history of a 1 cm diameter, well-circumscribed, erythematous, scaly patch with an elevated border on his glans penis. Histologic examination revealed a typical cornoid lamella and absence of an underlying granular layer. The localized type of porokeratosis of Mibelli is most commonly found on the limbs: however, the face, lips, mucosal surfaces, or genital area may instead be involved. We report a case of porokeratosis of Mibelli confined to the glans penis, a very rare site.

Coexistence of Porokeratosis of Mibelli and Disseminated Superficial Actinic Porokeratosis(DSAP)

There have been several reports of more than one type of porokeratosis occurring in the same family or the same individual. We hope to support the view of different phenotypic expressions of a common genetic aberration by describing an additional case of porokeratosis of Mibelli on the perianal area and DSAP on the face, forearms occurring in a 45-year-old man.

Squamous Cell Carcinoma Arising in Porokeratosis of Mibelli

We report a case of squamous cell carcinoma arising in porokeratosis of Mibelli in a 32-year-old male who presented with a large, slowly extending, erythematous patch with central ulceration on the left lateral side of the neck. Histopathologic examination of the periphery of the patch and central tumor showed porokeratosis and squamous cell carcinoma, respectively.