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Reactive perforating collagenosis in Adult
Other Resources UpToDate PubMed

Reactive perforating collagenosis in Adult

Contributors: Jeffrey M. Cohen MD, Rosemara Hughart MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Reactive perforating collagenosis (RPC) is a type of perforating disorder that is characterized by transdermal elimination of collagen fibers. There are two forms of RPC. The most common form is acquired RPC, which is generally observed in patients with chronic kidney disease and diabetes mellitus. Acquired RPC can rarely occur in the setting of medications or malignancies. Like other members of the acquired perforating dermatosis group, the lesions generally occur only on the legs or in a generalized distribution.

There is also a familial form of RPC, which has an onset in childhood. Familial RPC can persist throughout childhood and into adulthood, and generally presents on the hands and upper extremities. Lesions are induced by trauma.

The pathogenesis is unknown, but the familial disease is generally thought to be inherited in an autosomal recessive manner (although an autosomal dominant pattern has been observed in some families). Males and females are equally affected, and lesions generally start within the first few years of life in the inherited form.

The primary lesion is a skin-colored papule 1-3 mm in diameter that reaches a maximum size of about 6 mm within a month; 6-8 weeks later, it regresses spontaneously (often leaving temporary areas of hypopigmentation behind or slight scarring). There is a central keratotic plug seen in more advanced lesions that is firmly adherent and, if removed, results in bleeding. Koebner phenomenon may occur, resulting in new lesions that are often linearly arranged. Pruritus is often severe. There are often periods of disease remission and exacerbation.

For more information on familial reactive perforating collagenosis, see OMIM.

Codes

ICD10CM:
L87.1 – Reactive perforating collagenosis

SNOMEDCT:
64036004 – Reactive perforating collagenosis

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Elastosis perforans serpiginosa – Lesions often occur in young adults rather than young children; they are also often annular or serpiginous and do not exhibit Koebner phenomenon. The lesions generally only affect one anatomic area.
  • Perforating folliculitis – Papules are generally asymptomatic, whereas lesions of reactive perforating collagenosis are very pruritic. The keratotic plug is generally white and often contains a curled-up hair in the center, in contrast to the dark brown or blacker keratotic plugs of reactive perforating collagenosis.
  • Kyrle disease – Lesions usually occur in adults (although rarely in children); they are commonly bilateral and favor the lower extremities. The individual papules are generally reddish and often coalesce into plaques.
  • Arthropod bites
  • Papular urticaria
  • Molluscum contagiosum
  • Excoriations from various causes
  • Prurigo nodularis
  • Perforating of exogenous foreign material
  • Perforating of endogenous foreign material
  • Multiple keratoacanthomas

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed:09/06/2021
Last Updated:09/06/2021
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Reactive perforating collagenosis in Adult
Reactive perforating collagenosis : Arm, Bite or trauma site, Face, Leg, Scaly papule, Smooth papule, Symmetric extremities distribution, Pruritus
Clinical image of Reactive perforating collagenosis
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