Perforating dermatoses

Published on 18/03/2015 by admin

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Perforating dermatoses

Sarah Markoff and Mark G. Lebwohl

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

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The perforating dermatoses are a varied group of conditions characterized by the transepidermal elimination of dermal material. Four primary conditions are included in the discussion of the perforating disorders:

Management strategy

Definitive diagnosis of the perforating disorders depends on the demonstration of transepidermal elimination on skin biopsy. Differentiation between the different forms of perforating disorders can be accomplished by Masson trichrome stains for collagen (reactive perforating collagenosis), Verhoeff–van Gieson stains for elastic tissue (EPS), and step-sectioning to look for hair follicles (perforating folliculitis). Differentiation between these various disorders may be important, as EPS is associated with other diseases such as pseudoxanthoma elasticum, Down syndrome, osteogenesis imperfecta, Ehlers–Danlos syndrome, Rothmund–Thomson syndrome, Marfan syndrome, and penicillamine treatment. Reactive perforating collagenosis is commonly associated with diabetes and renal failure.

Occasionally, acquired reactive perforating collagenosis has occurred in patients treated with particular medications such as indinavir, erlotinib, sorafenib, and sirolimus, and perforating folliculitis has been reported in patients treated with tumor necrosis factor-α blockers and in a patient with cystic fibrosis.

Management of the perforating diseases involves determination of underlying etiologies. Most often, conditions such as diabetes mellitus and renal failure will be known to the patient who presents with perforating skin lesions. When the underlying cause is not apparent, serum chemistry for renal and liver function tests and oral glucose tolerance test or hemoglobin A1C may be helpful.

Once the diagnosis of underlying diseases is ascertained, treatment is directed at associated symptoms. Pruritus can be managed initially with topical or intralesional corticosteroids, topical anesthetics and menthol, as well as oral antihistamines, but the latter agents are usually not sufficiently effective. Minimizing pruritus is important because many of the perforating disorders typically exhibit a Koebner phenomenon, meaning that lesions develop in traumatized or scratched skin. Topical antipruritic agents such as menthol, phenol, or camphor, and topical anesthetics such as lidocaine and pramocaine are useful. Topical doxepin hydrochloride or oral antihistamines may also be of some benefit. Trimming the fingernails to minimize trauma to the skin and avoidance of scratching are key elements of treatment. Topical tretinoin and topical tazarotene have been shown to be effective for some patients. For those patients whose condition is exacerbated by sun exposure, sunscreens may be helpful. Conversely, in patients with renal disease UVB is dramatically effective for pruritus and has been reported to benefit perforating skin lesions as well. If UVB, narrowband UVB, and topical retinoids are ineffective, oral retinoids, allopurinol or antibiotics can be tried.

Specific investigations

First-line therapies

imageTretinoin 0.1% D
imageTazarotene gel 0.1% E
imageUVB E
imageNarrowband UVB D

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