Reactive Perforating Collagenosis Clinical Presentation

Updated: Mar 07, 2017
  • Author: Rachel M Fisher, MBChB, MRCP; Chief Editor: Dirk M Elston, MD  more...
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Presentation

History

Small, keratotic papules develop after minor trauma. The lesions start as pin-sized lesions, and they grow into larger papules/nodules over a few weeks. They heal with minor scarring.

Intense pruritus is a feature of the acquired form of reactive perforating collagenosis. Many patients have diabetes or renal failure, and many will be on hemodialysis.

A positive family history may be elicited in the childhood form of reactive perforating collagenosis.

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Physical Examination

Skin lesions of reactive perforating collagenosis

Flesh-colored, umbilicated, dome-shaped papules or nodules as large as 10 mm in diameter are typical. They have an adherent, keratinous plug. A giant variant of reactive perforating collagenosis has been described. [16]

Reactive perforating collagenosis lesions may occur in a linear configuration, exhibiting the Koebner phenomenon.

Residual scarring may be seen from previously healed lesions.

Skin distribution of reactive perforating collagenosis

Lesions are most commonly found on the extensor surfaces of the limbs and the dorsa of the hands. Reactive perforating collagenosis lesions may also occur on the trunk and the face. See the image below.

Typical keratotic papules. Typical keratotic papules.
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Causes

The childhood form of reactive perforating collagenosis is inherited. [5] A number of affected families have been reported. The mode of inheritance is not clear. Reports of affected families reveal autosomal dominant inheritance, autosomal recessive inheritance, and sporadic cases.

A case has been described arising in a child with Down syndrome. [6]

The underlying cause of reactive perforating collagenosis is unknown, but an abnormal response to superficial trauma (eg, scratching) may be involved. Papules have been reported following scratches, acne spots, insect bites, [7] and scabies. [8] Lesions of reactive perforating collagenosis have been experimentally induced in susceptible skin by scratching. [9]

The acquired form of reactive perforating collagenosis may occur in association with chronic renal failure, often in a patient with underlying diabetes. In both the United Kingdom and the United States, the prevalence of this disorder in patients on dialysis is approximately 10%. [3, 4]

The acquired form of reactive perforating collagenosis also can occur in association with other nephropathies without diabetes.

Acquired reactive perforating collagenosis has been reported in association with hypothyroidism, hyperparathyroidism, and liver dysfunction, [2] dermatomyositis, [10] and rheumatoid vasculitis. [11]

Malignancies, including lymphoma, hepatocellular carcinoma, [12] periampullary carcinoma, [13] and thyroid cancer, [14] have been associated with the acquired form of reactive perforating collagenosis.

Indinavir has induced reactive perforating dermatosis in 2 patients with HIV disease. [15]

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Complications

The inherited form of reactive perforating collagenosis is not associated with any systemic complications.

The acquired form of reactive perforating collagenosis occurs in patients with multiple medical problems, but whether the development of the lesions implies a poorer prognosis is unclear.

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