Porokeratosis Clinical Presentation

  • Author: Linda V Spencer, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: May 7, 2010
 

History

Classic porokeratosis (Mibelli)

During childhood, a small, asymptomatic or slightly pruritic lesion develops, expanding over a period of years. Less commonly, lesions may develop during adulthood and enlarge rapidly, usually in the clinical setting of immunosuppression. Occasionally, patients have a history of an antecedent trauma, such as a burn wound.

Disseminated superficial (actinic) porokeratosis

Multiple, brown, annular, keratotic lesions that develop predominantly on the extensor surfaces of the legs and the arms characterize DSAP. They are usually asymptomatic, but they may itch slightly. Sunless tanning lotions containing dihydroxyacetone cause the cornoid lamellae to darken and become more clinically prominent. Dermoscopy reveals a "white track" structure with brown pigmentation on the inside of the track that can be seen at the edge of an individual lesion, corresponding to the cornoid lamella. Centrally, a white area with red dots, globules, and lines is present that corresponds to capillary vessels; it is more easily observed through the atrophic epithelium.

Facial lesions are seen in approximately 15% of patients, but the face may be the only area of involvement. Patients are typically women in their third or fourth decade of life, with a history of excessive ultraviolet exposure. Patients may have a history of phototherapy for psoriasis.

Nonactinic forms may be seen following electron beam total skin irradiation, organ transplantation, hepatitis C virus–related hepatocellular carcinoma,[15] HIV infection, renal failure, or in association with other causes of immunosuppression.

Linear porokeratosis

Linear porokeratosis lesions typically develop during infancy or early childhood. They arise as reddish brown patches or linear keratotic papules and plaques typically distributed along Blaschko's lines, suggesting cutaneous mosaicism. Linear porokeratosis can be localized, blaschkoid, generalized, or systematized, and have a higher risk of malignant degeneration, possibly because of allelic loss.

Porokeratosis palmaris et plantaris disseminata

Small, relatively uniform lesions are first seen on the palms and the soles. They may then develop in a generalized distribution, including the mucosal membranes. The lesions may itch or sting, but they are usually asymptomatic. The onset is typically during adolescence or early adulthood, and males are affected twice as often as females.

Punctate porokeratosis

Multiple, asymptomatic, tiny, hyperkeratotic papules with thin, raised margins develop on the palms and the soles during adulthood.

Porokeratotic adnexal ostial nevus

Lesions usually develop during infancy or childhood, although late onset during adulthood has been reported. Asymptomatic hyperkeratotic papules and plaques, and occasionally punctate pits filled with a comedolike keratin plug, develop typically on the distal extremities, although proximal extremities, trunk, and face may be involved. During the neonatal period, the lesions may initially appear as erosions. Often, lesions are aligned along Blaschko lines and may extend into nailbeds. Note the image below.

A young boy with a linear lesion of porokeratotic A young boy with a linear lesion of porokeratotic eccrine ostial and dermal duct nevus extending onto the nailbed, causing pterygium formation.
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Physical

Classic porokeratosis (Mibelli)

The lesion develops as a small, light brown, keratotic papule that slowly expands to form an irregularly shaped, annular plaque with a raised, ridgelike border. This border may be hypertrophic or verrucous and is usually greater than 1 mm in height. A thin furrow is typically seen in the center of the ridge, causing a Great Wall of China effect. The lesion is slightly hypopigmented or hyperpigmented, minimally scaly, slightly atrophic, hairless, and anhidrotic. The size may vary from a few millimeters to several centimeters.

Lesions may be found anywhere, including the mucous membranes, although they most commonly occur on the extremities. Generally, few lesions are observed. Porokeratosis ptychotropica is a verrucous variant that is localized to the buttocks and clinically resembles psoriasis. Note the image below.

Porokeratosis of Mibelli on the lower leg in a renPorokeratosis of Mibelli on the lower leg in a renal transplant recipient.

Disseminated superficial (actinic) porokeratosis

Dozens of small, indistinct, light brown patches with a threadlike border are seen on the extensor surfaces of the arms and the legs.

Dermoscopy shows a "white track" structure at the periphery of the lesion, with a brownish pigmentation on the inner side and with a double white track in some parts of the lesion. This corresponds to the cornoid lamella. The center may show a white homogeneous area, corresponding to acanthotic epithelium, or red dots, globules, and lines corresponding to enlarged capillary vessels that can be seen because the epithelium is atrophic.[16]

Facial lesions are seen in approximately 15% of patients. Nonactinic DSP may have a generalized distribution, sparing the palms and the soles. Bullous and pruritic variants have been described. Note the images below.

Disseminated superficial actinic porokeratosis on Disseminated superficial actinic porokeratosis on the lower legs of a female patient. A 42-year-old woman with multiple lesions on the pA 42-year-old woman with multiple lesions on the pretibial aspects of the legs.

Linear porokeratosis

Grouped, linearly arranged, annular papules and plaques with the characteristic raised peripheral ridge are seen unilaterally on an extremity, the trunk, and/or the head and neck area. The lesions commonly follow a dermatomal distribution, and they may arise initially as reddish-brown patches.

Multiple linear groups may be seen in one patient, typically on the same side. They may be seen in association with other forms of porokeratosis. Individual lesions within the linear grouping have a well-developed border, often with a central furrow similar to that seen in classic PM.

Clinical changes consistent with the development of a basal or squamous cell carcinoma are more common in linear porokeratosis than in other forms of porokeratosis.

Porokeratosis palmaris et plantaris disseminata

The lesions are small, superficial, relatively uniform, with a slightly hyperpigmented, atrophic center and a minimally raised peripheral ridge. Mucosal lesions are small, annular or serpiginous, and pale. Squamous cell carcinoma has been reported to develop within lesions of PPPD.

Punctate porokeratosis

Dozens of discrete or grouped seedlike hyperkeratotic lesions with characteristic thin, raised ridgelike margins develop on the palms and the soles. Patients usually have other forms of porokeratosis as well, most commonly the linear or Mibelli types. Punctate porokeratosis may be clinically and histologically indistinguishable from punctate porokeratotic keratoderma, which is considered to be a cutaneous sign of an internal malignancy.

Porokeratotic adnexal ostial nevus

Asymptomatic hyperkeratotic papules and plaques, and occasionally punctate pits filled with a comedolike keratin plug, develop on the extremities and occasionally the trunk and/or face. If the lesions are present at birth, they may develop as erosions that evolve into darker brown, well-marginated linear plaques. Pterygium formation can occur if lesions extend into the nail bed. Lesions are often distributed along the lines of Blaschko.

Rare case reports describe other findings, including psoriasis, focal anhidrosis, developmental delay, seizures, scoliosis, hemiparesis, polyneuropathy, hyperthyroidism, hearing loss, and breast hypoplasia. Squamous cell carcinoma has been reported to develop within well-established lesions. Additionally, Late-onset lesions developing during adulthood may be clinically indistinguishable from PPPD.[17]

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Causes

Risk factors for porokeratosis include genetic inheritance, ultraviolet light exposure, and immunosuppression. One study found that approximately 10% of patients who had undergone renal transplantation developed porokeratosis.[18]

  • Classic porokeratosis (Mibelli): Autosomal dominant inheritance and immunosuppression are the usual causes. PM has been seen following radiation therapy, at burn wounds,[19] and at hemodialysis sites.
  • Disseminated superficial (actinic) porokeratosis: Sun exposure and/or artificial ultraviolet radiation exposure in a patient who is genetically predisposed causes DSAP. Exacerbations have been reported following prolonged sun exposure, repeated tanning bed exposure, electron beam radiation therapy, and therapeutic phototherapy or photochemotherapy for psoriasis. Drug-induced photosensitivity may play a role. Protection from ultraviolet radiation may lead to spontaneous resolution. Additionally, immunosuppression predisposes patients to both DSAP and nonactinic DSP. Because of this, a viral etiology has been hypothesized.
  • Linear porokeratosis: No definite inheritance pattern has been established. Loss of heterozygosity has been proposed as a genetic mechanism and may explain the higher risk of malignant degeneration seen in linear porokeratosis in comparison to other forms of porokeratosis.
  • Porokeratosis palmaris et plantaris disseminata: Familial PPPD is transmitted in an autosomal dominant mode with variable penetrance. Acquired PPPD may be caused by immunosuppression, or it may be a cutaneous marker of internal malignancy.
  • Punctate porokeratosis: This condition has no unique inheritance pattern and is usually associated with other forms of porokeratosis.
  • Porokeratotic adnexal ostial nevus: This is nonhereditary and sporadic, possibly the result of a mosaic lethal gene mutation.
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Contributor Information and Disclosures
Author

Linda V Spencer, MD  Consulting Staff, Department of Dermatology, St Clare Medical Center

Linda V Spencer, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, and Indiana State Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Marjan Garmyn, MD, PhD  Professor, Faculty of Medicine, Katholieke Universiteit Leuven, Belgium; Chair and Adjunct Head, Department of Dermatology, University of Leuven, Belgium

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD  Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Consulting fee Consulting; Celgene Honoraria Safety monitoring committee; GSK - Glaxo Smith Kline Consulting fee Consulting; TenXBioPharma Consulting fee Safety Monitoring committee

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

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Porokeratosis of Mibelli on the lower leg in a renal transplant recipient.
Disseminated superficial actinic porokeratosis on the lower legs of a female patient.
A 42-year-old woman with multiple lesions on the pretibial aspects of the legs.
A young boy with a linear lesion of porokeratotic eccrine ostial and dermal duct nevus extending onto the nailbed, causing pterygium formation.
 
 
 
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