Continuing Medical Education
Clinical and pathologic findings of paraneoplastic dermatoses

https://doi.org/10.1016/j.jaad.2004.06.051Get rights and content

Paraneoplastic dermatoses comprise a heterogeneous group of noninherited skin conditions that manifest internal malignancy. Familiarity with paraneoplastic dermatoses is important to both clinician and pathologist alike, as recognition of such a condition offers opportunity for early diagnosis and treatment of internal malignancy; monitoring for tumor recurrence; and insight into pathophysiology which may yield possible clues to treatment. Herein are reviewed 16 of the best established paraneoplastic dermatoses that display distinctive clinical and pathologic findings.

Learning objective

At the conclusion of this leaning activity, participants should be able to recognize, diagnose, and describe the clinical and pathologic findings of paraneoplastic dermatoses.

Section snippets

History and background

Most (80%) cases of acanthosis nigricans are idiopathic or associated with benign conditions such as obesity and endocrine abnormalities.9, 10 Acanthosis nigricans associated with malignancy was reported separately by Pollitzer and Janovsky in 1890.11 More than 1,000 cases have since been reported.12

Demographics

The majority of cases of acanthosis nigricans that present during childhood are of a benign form related either to heredity or to insulin resistance. Sudden presentation during adulthood, however,

History and background

Dermatomyositis in association with malignancy was first reported in 1916,40 when Stretz described a case associated with gastric carcinoma and, later that year, Kankeleit reported one in association with breast cancer.41 Dermatomyositis is a severe inflammatory myopathy of unknown cause characterized by proximal muscle weakness and associated with cutaneous findings. It is now appreciated that dermatomyositis may also occur in the absence of myositis. Dermatomyositis has been divided into 2

History and background

Erythema gyratum repens was first described in 1952 by Gammel, who observed it in a patient with breast cancer.50 The Latin term repens, meaning “to crawl” or “to creep,” describes the striking, characteristic, expanding erythematous borders in this rare paraneoplastic condition.51 Approximately 50 cases have been reported in the literature.52

Demographics

The male-to-female ratio is 2:1, and the average age of onset is 63 years.53 All of the reported patients have been Caucasian,51 though reporting bias may

Neutrophilic dermatoses

Traditionally, Sweet's syndrome and pyoderma gangrenosum have been classified as separate entities. The atypical forms of both have been previously reported as separate disorders associated with various forms of leukemia. However, when associated with myeloproliferative disorders, Sweet's syndrome and pyoderma gangrenosum are probably 2 points on a continuum of inflammatory neutrophilic dermatoses that are neither infectious nor metastatic.63

History and background

Multicentric reticulohistiocytosis was first described by Weber and Freudenthal in 1937,76 and its association with malignancy was first proposed by Barrow and Holubar in 1969.77, 78, 79

Demographics

Approximately 80% of reported cases are in Caucasians.78 The female-to-male ratio has been estimated to be around 1.85:178 to 3:1.77 The average age of onset is 50 years, with a range of 6 to 86 years.78

Clinical presentation

Pink, brown, or gray papules and nodules, ranging in diameter from a few millimeters to 2 cm, appear on the

History and background

Scleromyxedema, also known as generalized lichen myxedematosus, is a rare, chronic fibromucinous disorder that first was reported by Dubreuilh in 1906.89 Its name was coined in 1954 by Gottron to describe the generalized lichenoid papular eruption.90, 91 Scleromyxedema is characterized by a generalized lichenoid eruption with sclerosis, mucin deposition, and fibroblast proliferation, monoclonal gammopathy, and absence of thyroid disease.92, 93

Demographics

Most patients affected are between 30 and 50 years

History and background

Hypertrichosis lanuginosa acquisita was first reported by Turner in 1865 in a breast cancer patient.100 Approximately 50 cases have been reported in the literature since.101 Hypertrichosis lanuginosa can occur as a congenital autosomal dominant form, in which there is a generalized persistence of fetal lanugo hair, and may be associated with deformities of the external ears and teeth. By contrast, the sudden appearance of lanugo hair later in life may signal malignancy.102

Demographics

Hypertrichosis

Summary

Paraneoplastic dermatoses comprise a heterogeneous group of disorders from both a clinical and histologic point of view. As such, they show a variable degree of correlation between the malignancy and the cutaneous lesions. Though the skin conditions are not always specific for an internal malignancy (they can occur sporadically in the absence of a malignancy), most cases of paraneoplastic dermatoses parallel the clinical course of the neoplasm. Clues helpful in distinguishing paraneoplastic

References (106)

  • M.R. Heaphy et al.

    The sign of Leser-Trelat in a case of adenocarcinoma of the lung

    J Am Acad Dermatol

    (2000)
  • R.A. Schwartz

    Sign of Leser-Trelat

    J Am Acad Dermatol

    (1996)
  • M. Richard et al.

    Acrokeratosis paraneoplastica (Bazex' syndrome)

    J Am Acad Dermatol

    (1987)
  • D.S. Caldwell et al.

    Rheumatologic manifestations of cancer

    Med Clin North Am

    (1986)
  • A. Sklavounou et al.

    Paraneoplastic pemphigus: a review

    Oral Oncol

    (1998)
  • A.S. Boyd et al.

    Erythema gyratum repens: a paraneoplastic eruption

    J Am Acad Dermatol

    (1992)
  • L.E. Eubanks et al.

    Erythema gyratum repens

    Am J Med Sci

    (2001)
  • J.W. White

    Gyrate erythema

    Dermatol Clin

    (1985)
  • M.P. Marinkovich et al.

    Necrolytic migratory erythema without glucagonoma in patients with liver disease

    J Am Acad Dermatol

    (1995)
  • K. Thorisdottir et al.

    Necrolytic migratory erythema: a report of three cases

    J Am Acad Dermatol

    (1994)
  • M.A. Chastain

    The glucagonoma syndrome: a review of its features and discussion of new perspectives

    Am J Med Sci

    (2001)
  • E.A. Mullans et al.

    Iatrogenic necrolytic migratory erythema: a case report and review of nonglucagonoma-associated necrolytic migratory erythema

    J Am Acad Dermatol

    (1998)
  • W. Caughman et al.

    Neutrophilic dermatosis of myeloproliferative disorders. Atypical forms of pyoderma gangrenosum and Sweet's syndrome associated with myeloproliferative disorders

    J Am Acad Dermatol

    (1983)
  • E.E. Boh et al.

    Cutaneous manifestations of gastrointestinal diseases

    Dermatol Clin

    (2002)
  • J.L. Lesher et al.

    Multicentric reticulohistiocytosis

    J Am Acad Dermatol

    (1984)
  • S. Kossard et al.

    Necrobiotic xanthogranuloma with paraproteinemia

    J Am Acad Dermatol

    (1980)
  • S. Ugurlu et al.

    Necrobiotic xanthogranuloma: long-term outcome of ocular and systemic involvement

    Am J Ophthalmol

    (2000)
  • A. Godby et al.

    Fatal scleromyxedema: report of a case and review of the literature

    J Am Acad Dermatol

    (1998)
  • E.M. Jackson et al.

    Diffuse cutaneous mucinoses

    Dermatol Clin

    (2002)
  • A.M. Dinneen et al.

    Scleromyxedema

    J Am Acad Dermatol

    (1995)
  • K.J. Mullins et al.

    Primary solitary amyloidosis of the spine: a case report and review of the literature

    Surg Neurol

    (1997)
  • R.I. van der Waal et al.

    Amyloidosis of the tongue as a paraneoplastic marker of plasma cell dyscrasia

    Oral Surg Oral Med Oral Pathol Oral Radiol Endod

    (2002)
  • S.M. Breathnach

    Amyloid and amyloidosis

    J Am Acad Dermatol

    (1988)
  • H.O. Curth

    Skin lesions and internal carcinoma

  • D.I. McLean

    Cutaneous paraneoplastic syndromes

    Arch Dermatol

    (1986)
  • P.R. Cohen et al.

    Mucocutaneous paraneoplastic syndromes

    Semin Oncol

    (1997)
  • S. Sabir et al.

    Cutaneous manifestations of cancer

    Curr Opin Oncol

    (1999)
  • S. Pollitzer

    Acanthosis nigricans: a symptom of a disorder of the sympathetic

    JAMA

    (1909)
  • D.S. Skiljevic et al.

    Generalized acanthosis nigricans in early childhood

    Pediatr Dermatol

    (2001)
  • P.R. Cohen

    Cutaneous paraneoplastic syndromes

    Am Fam Physician

    (1994)
  • E.M. Higgins et al.

    Cutaneous manifestations of malignant disease

    Br J Hosp Med

    (1992)
  • U. Bottoni et al.

    Florid cutaneous and mucosal papillomatosis with acanthosis nigricans revealing a primary lung cancer

    J Eur Acad Dermatol Venereol

    (2000)
  • D.L. Rogers

    Acanthosis nigricans

    Semin Dermatol

    (1991)
  • C.A. Stuart et al.

    Acanthosis nigricans

    J Basic Clin Physiol Pharmacol

    (1998)
  • V. Ramirez-Amador et al.

    Oral manifestations as a hallmark of malignant acanthosis nigricans

    J Oral Pathol Med

    (1999)
  • R.A. Schwartz et al.

    Acquired ichthyosis: a marker for internal disease

    Am Fam Physician

    (1984)
  • J.C. Cather et al.

    Ichthyosiform sarcoidosis

    J Am Acad Dermatol

    (1999)
  • Y. Politi et al.

    Cutaneous paraneoplastic syndromes

    Acta Derm Venereol

    (1993)
  • A.R. Haste

    Acquired ichthyosis from breast cancer

    Br Med J

    (1967)
  • J. Clarke

    Malignant acanthosis nigricans

    Clin Exp Dermatol

    (1977)

    • Cited by (0)

    Funding sources: None.

    Conflict of interest: None identified.

    View full text
    Copyright © 2006 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.