Simultaneous development and parallel course of disseminated superficial porokeratosis and ovarian cancer: Coincidental association or true paraneoplastic syndrome?

doi:10.1016/j.jaad.2007.12.030

Journal of the American Academy of Dermatology

Volume 58, Issue 4, April 2008, Pages 657-660

https://doi.org/10.1016/j.jaad.2007.12.030 Get rights and content

Disseminated superficial porokeratosis has been described in the setting of immunosuppressive conditions, including organ transplantation, infections, and hematopoietic malignancies. The outbreak of disseminated superficial porokeratosis during the development of solid organ malignancies has been rarely reported in the literature in patients affected by hepatitis C virus–related hepatocellular carcinoma or by cholangiocarcinoma, which suggests a paraneoplastic nature of the cutaneous disease. We report an unusual case of disseminated superficial porokeratosis in a patient affected by ovarian cancer, characterized by simultaneous onset and a parallel course of the two pathologies; there was good clinical response to chemotherapy, accompanied by a successful stop of disseminated superficial porokeratosis progression and gradual clearing of the keratotic lesions.

Section snippets

Case report

A 54-year-old woman with a history of essential hypertension was admitted to the hospital because of the sudden onset of abdominal pain and swelling. On physical examination, abdominal tenderness and signs of ascites were found. Ultrasonography and a computed tomographic scan of the abdomen and pelvis disclosed ascites and an 8-cm mass in the right ovary. Chest computed tomography, gastroscopy, colonoscopy, and mammography findings were normal. Serum cancer antigen-125 was 2884 U/mL (reference

Discussion

Paraneoplastic dermatoses are skin changes with no intrinsically neoplastic nature that are caused by a malignancy; the recognition of the cutaneous disease and the consequent screening to detect the cancer may be of great importance for the prognosis because they might indicate the presence of the tumor, thereby allowing prompt therapeutic intervention. The diagnosis of paraneoplastic syndrome can be relatively simple in the case of peculiar disorders that are almost universally associated

References (11)

T. Kono et al.
Synchronous development of disseminated superficial porokeratosis and hepatitis C-virus related hepatocellular carcinoma
J Am Acad Dermatol
(2000)
M.A. de Roos et al.
p53 overexpression is a predictor of local recurrence after treatment for both in situ and invasive ductal carcinoma of the breast
J Surg Res
(2007)
P.L. Bencini et al.
Porokeratosis and immunosuppression
Br J Dermatol
(1995)
J. Kanitakis et al.
Porokeratosis and immunosuppression
Eur J Dermatol
(1998)
H.W. Lee et al.
Disseminated superficial porokeratosis in a patient with cholangiocarcinoma
J Am Acad Dermatol
(2006)

There are more references available in the full text version of this article.

Cited by (21)

Disseminated eruptive porokeratosis associated with hepatitis A
2022, Piel
Porokeratosis: A Review of Its Pathophysiology, Clinical Manifestations, Diagnosis, and Treatment
2020, Actas Dermo-Sifiliograficas
Las poroqueratosis son un grupo heterogéneo e infrecuente de dermatosis adquiridas o heredadas de etiología desconocida, caracterizadas por un trastorno de la queratinización secundario a una expansión clonal anormal de los queratinocitos. Se han descrito múltiples mutaciones genéticas potencialmente implicadas. Histológicamente, se caracterizan por la presencia de la lamela cornoide. Su presentación clínica es variable con formas localizadas, diseminadas e incluso eruptivas. Las poroqueratosis se han asociado con inmunosupresión, radiación ultravioleta, enfermedades sistémicas, infecciosas y neoplásicas. Muchos autores las consideran como entidades premalignas dada su potencial degeneración neoplásica a carcinoma escamoso o basocelular. Por ello, el seguimiento a largo plazo es uno de los pilares de su tratamiento, el que suele ser complejo y a menudo insatisfactorio. En la presente revisión se discuten los últimos avances en su etiopatogenia, diagnóstico y terapéutica, y se propone un algoritmo de 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.
Cutaneous alerts in systemic malignancy: Part 1
2013, Actas Dermo-Sifiliograficas
Citation Excerpt :
There have also been anecdotal reports of other erythematosquamous lesions occurring in association with malignant tumors; these lesions include small plaque parapsoriasis (***), described in a liposarcoma,52 and pityriasis rubra pilaris (***), described in Merkel cell carcinoma cutaneous squamous cell carcinoma, hepatocellular carcinoma, breast carcinoma, and lung carcinoma.53 Some authors have suggested that disseminated superficial porokeratosis (***) might be a paraneoplastic presentation in hematologic malignancies, hepatocellular carcinoma, cholangiocarcinoma, and esophageal and ovarian carcinoma.54–57 Palmoplantar keratoderma (**) is seen in benign conditions, such as psoriasis, pityriasis rubra pilaris, climacteric, or as mentioned earlier, Bazex syndrome, acanthosis nigricans (tripe palms) erythema gyratum repens, and others.
The aim of this review is to familiarize dermatologists and clinicians in general with cutaneous signs and symptoms that can help lead to an early diagnosis of an underlying malignancy. Because the skin is one of the most accessible organs, it should never be overlooked in systemic disease. Examination of the skin has the advantage of revealing important information about the patient's condition without requiring the use of invasive techniques.
In the literature, most discussions of cutaneous manifestations of internal malignancy refer to classic paraneoplastic syndromes, but a wide variety of skin conditions, while not strictly paraneoplastic, can, in certain contexts, indicate the presence of malignancy or an increased risk of developing cancer later in life.
In this review, various skin conditions that can signal malignancy or increased cancer risk are presented in randomly ordered groups based on clinical morphology.
Conditions with multiple signs and symptoms have been classified on the basis of their most characteristic feature.
El objetivo de esta revisión es alertar al dermatólogo y a los clínicos en general de los signos y síntomas cutáneos que pueden contribuir al diagnóstico precoz de una neoplasia subyacente. Puesto que la piel es uno de los órganos más accesible, nunca debe ser ignorada en las enfermedades sistémicas. Posee la ventaja de que su exploración no requiere técnicas agresivas y nos revela datos importantes de la situación del paciente.
Habitualmente se abordan los síndromes paraneoplásicos clásicos, pero hay una gran variedad de procesos cutáneos no estrictamente paraneoplásicos, que en un determinado contexto sugieren la presencia de una malignidad o bien tienen más riesgo de desarrollarla a lo largo de la vida.
Se ha optado por enumerar las distintas dermatosis agrupándolas por su morfología clínica, siguiendo un orden aleatorio.
Varios de los procesos comparten signos y síntomas múltiples por lo que se ha recurrido al más notorio para incluirlo en un determinado grupo.
Disorders of epidermal maturation and keratinization
2009, Weedon's Skin Pathology: Third Edition
Porokeratoses—A Comprehensive Review on the Genetics and Metabolomics, Imaging Methods and Management of Common Clinical Variants
2023, Metabolites
Paraneoplastic syndromes in cholangiocarcinoma
2020, World Journal of Hepatology

View all citing articles on Scopus

: Funding sources: None.

: Conflicts of interest: None declared.

View full text

Case reportSimultaneous development and parallel course of disseminated superficial porokeratosis and ovarian cancer: Coincidental association or true paraneoplastic syndrome?

Section snippets

Case report

Discussion

J Am Acad Dermatol

J Surg Res

Porokeratosis and immunosuppression

Br J Dermatol

Porokeratosis and immunosuppression

Eur J Dermatol

Disseminated superficial porokeratosis in a patient with cholangiocarcinoma

J Am Acad Dermatol

Case report
Simultaneous development and parallel course of disseminated superficial porokeratosis and ovarian cancer: Coincidental association or true paraneoplastic syndrome?