The Japanese Dermatological Association (JDA) has released a new clinical practice guideline outlining several evidence-based recommendations for the treatment of perforating dermatosis. The guideline, published in the Journal of Dermatology, includes recommendations on managing comorbidities, using topical steroids, applying ultraviolet (UV) therapy, and treatment discontinuation.

Guideline Development

To develop the guideline, the JDA reviewed published clinical evidence on perforating dermatosis published from 1989 to 2019. The paucity of randomized controlled trials led the guideline committee to seek evidence primarily from case series and reports. Based on the evidence available, the guideline committee then created clinical questions with corresponding recommendations for these questions. The key clinical questions created from the review included:


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1.      What are the common comorbidities in acquired reactive perforating collagenosis?

2.      Is treatment of diabetes and chronic renal disease effective for acquired reactive perforating collagenosis?

3.      Is topical steroid effective for acquired reactive perforating collagenosis?

4.      Is ultraviolet (UV) therapy effective for acquired reactive perforating collagenosis?

5.      What are the most common comorbidities of elastosis perforans serpiginosa?

6.      Should D-penicillamine be discontinued if elastosis perforans serpiginosa develops during oral D-penicillamine treatment?

7.      What are the common comorbidities of Kyrle disease?

8.      What are the common comorbidities of perforated folliculitis?

9.      If perforating folliculitis develop during molecular-targeted drug (kinase inhibitor) treatment, should clinicians discontinue the molecular-targeted drug?

Diagnostic Criteria

The guideline committee also outlined the diagnostic criteria of perforating dermatosis. These diagnostic criteria include a combination of basic findings, substances that are primarily transepidermally eliminated, clinical cutaneous findings, and reference findings. Different combination ratings produce different definite diagnoses of certain types of perforating dermatosis.

According to the guideline, each disease can be diagnosed if a patient meets the corresponding described criteria:

Acquired reactive perforating collagenosis: transepidermal elimination of degenerated cutaneous components according to histopathological findings, including collagen tissue from epidermis; umbilicated papules or nodules with a central adherent keratotic plug; onset at age  years or older

Elastosis perforans serpiginosa: transepidermal elimination of degenerated cutaneous components according to histopathological findings, including elastic fibers

Kyrle disease: transepidermal elimination of degenerated cutaneous components according to histopathological findings, including keratins

Perforating folliculitis: transepidermal elimination of degenerated cutaneous components according to histopathological findings, including collagen tissue from hair follicles

Severity Classification

Although the recommendations made in the guideline do not specifically mention severity, the panel nonetheless describes the severity classification of perforating dermatosis in their paper. Guideline committee members adopted the infiltration/papulation and excoriations based on the Eczema Area and Severity Index (EASI) score for atopic dermatitis to assess the severity of perforating dermatosis. Their severity classification system also includes the addition of a 0 to 10 pruritus numerical rating scale.

Management of Comorbidities

The key comorbidities of acquired reactive perforating collagenosis include diabetes and chronic kidney disease. As such, the guideline committee recommends considering treating coexisting diabetes and chronic kidney disease in these patients. This recommendation was based partially on case series reporting treatment of diabetes was effective in acquired reactive perforating collagenosis.

According to the panel, Down syndrome is a comorbidity that has been observed in elastosis perforins serpiginosa. Diabetes and chronic kidney disease may also be common in patients with Kyrle disease as well as perforated folliculitis. No recommendations were made on the screening and/or management of these comorbidities in patients with either elastosis perforans serpiginosa, Kyrle disease, and perforated folliculitis.

Topical Steroid Therapy

Evidence from 3 case series supported a recommendation for topical steroid therapy in the treatment of acquired reactive perforating collagenosis.

Recommendation on UV Therapy

The guideline also recommends the use of UV therapy as a treatment for acquired reactive perforating collagenosis based on evidence from 3 case reports.

Treatment Discontinuation Recommendations

The panel recommends discontinuing D-penicillamine in patients who have developed elastosis perforans serpiginosa during the use of the therapy, especially if a causal relationship is strongly suspected. The guideline recommends switching to a drug other than D-penicillamine.

Likewise, the authors of the guideline cite evidence suggesting perforating folliculitis may occur during the use of molecular-targeted drugs, like kinase inhibitors. If clinicians suspect a strong causal relationship, the guideline advises for discontinuation of these targeted drugs and switching to a different therapy.

Reference

Kawakami T, Akiyama M, Ishida-Yamamoto A, et al. Clinical practice guide for the treatment of perforating dermatosis. J Dermatol. 2020;47(12):1374-1382. doi:10.1111/1346-8138.15647