Perforating Folliculitis

Perforating Folliculitis

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Perforating disorders are characterized by transepidermal elimination of altered keratin or dermal connective tissue material. These disorders include perforating folliculitis (as shown below), Kyrle disease, elastosis perforans serpiginosa, reactive perforating collagenosis, and acquired perforating dermatosis. Cases of overlap are described, and diagnostic criteria are not well-defined for all the entities. [1] Clinically, the lesions are hyperkeratotic to verrucous papules and nodules.

In perforating folliculitis, keratotic follicular papules develop, particularly over extensor surfaces. Microscopically, the disorder is characterized by disruption of the infundibular portion of the follicular wall, with transepidermal (transfollicular) elimination of connective-tissue elements and cellular debris.

Perforating folliculitis may present as an isolated finding, apparently unrelated to other disease states, but also can be associated with chronic renal failure and diabetes mellitus. Perforating folliculitis is closely related, if not identical, to the acquired perforating dermatosis that occurs with chronic renal disease. Kyrle disease (hyperkeratosis follicularis et parafollicularis in cutem penetrans) may simply represent an exaggerated form of perforating folliculitis. In addition, another disorder of transepidermal elimination, elastosis perforans serpiginosa, occasionally displays involvement of follicular units.

As in Kyrle disease, the concept of an extrinsic keratin plug penetrating the epidermis generally has been discredited. Abnormally premature keratinization at the expense of proliferation is a possible explanation, as proposed by Carter and Constantine and Tappeiner et al in Kyrle disease. [2, 3] A role for fibronectin has been postulated. In addition, a primary alteration of connective tissue or deposition of foreign material within the superficial dermis, with subsequent engulfment and elimination by proliferative follicular epithelium, also is conceivable as a mechanism. Such a response to experimental implantation of foreign material has been described.

In addition, evidence suggests a pathologic role for excessively coiled hairs. Mehregan first proposed that curled hairs within follicular canals may act as springs, penetrating the lateral follicular wall, thereby initiating the process of transepidermal elimination. [4] Support for this concept has been provided by an ultrastructural study of acquired perforating dermatosis that showed hair shaft fragments within transepidermal channels, even in patients in whom follicular involvement was not demonstrable on routine light microscopy. Factors that may promote coiling of hairs include follicular hyperkeratosis (occasional perforated follicles can be identified in keratosis pilaris) or contact dermatitis (eg, resulting from formaldehyde in clothing). Finally, trauma, such as scratching of pruritic skin, may well play a significant role in lesional development, possibly by setting in motion one or more of the pathologic events described above. [5]

A number of reported cases of perforating folliculitis appear to be idiopathic, but specific associations also have been observed. Although some associations could be coincidental, the association with chronic renal failure (including both dialysis-dependent and nondialysis patients) is relatively common, suggesting a pathogenetic link. [6, 7, 8, 9, 10, 11] Perforating folliculitis also is observed relatively commonly in association with diabetes mellitus.

Less common associations include sclerosing cholangitis, [12, 13] hypertension, atherosclerotic cardiovascular disease, acanthosis nigricans, psoriasis, [14] and phrynoderma. [15] A single case report described an association with Poland syndrome (unilateral absence of the pectoralis major muscle and ipsilateral symbrachydactyly), but this patient also had diabetes mellitus, hyperuricemia, and dilated cardiomyopathy. [16] A single case highlighted the presence of perforating folliculitis in a patient with human immunodeficiency virus infection. [17] A case of perforating folliculitis was also reported in a child with cystic fibrosis. [18]  One case of perforating folliculitis arising in a patient with preexisting antisynthetase syndrome has been reported. [19]

Drug-induced cases include associations with infliximab and etanercept as possible inciting agents in a patient with rheumatoid arthritis [20] and dose-dependent association of sorafenib with skin lesions of perforating folliculitis. [21, 22, 23, 24, 25, 26] A report of a relationship with nilotinib suggests that drugs that inhibit c-kit and PDGF-R affect normal hair follicle development. PDGF-R has been previously linked in murine and human in vitro models to affect the hair follicle cycle. [27]  A case of bendamustine-rituximab chemotherapy-induced perforating folliculitis was reported in 2017. [28]

Incidence of perforating folliculitis worldwide is not known precisely, although the disorder is not uncommon. In Detroit, Michigan, 50 cases were reported during a 2-year period in the early 1970s, although this observation was followed by a declining incidence of new cases.

Although generally no ethnic predilection has been identified, 1 study found a higher incidence of Kyrle disease in chronic renal failure among African American individuals.

Perforating folliculitis occurs equally in males and females; no sex predilection has been reported.

Perforating folliculitis is more common in the second through fourth decades of life.

Perforating folliculitis morbidity is associated with the cosmetic appearance of lesions and the pruritus that occasionally accompanies them. Although cutaneous disease is insignificant, substantial morbidity or mortality rates can be seen in association with the primary underlying diseases, such as diabetes mellitus or chronic renal failure.

Skin lesions can improve or clear, either spontaneously or with therapy. Further therapeutic experience may permit the development of specific treatment guidelines.

Reports of improvement of perforating folliculitis lesions with stabilization of renal damage (in patients with chronic renal failure) are encouraging and underscore the important role of internal medicine or surgery consultants in treating these patients.

Inform perforating folliculitis patients about the nature of the disease and its relation to any underlying medical condition that may be present.

Instruct perforating folliculitis patients to avoid rubbing or scratching the lesions, since this may result in their spread or in the development of secondary infection.

Fully inform patients about the treatment being used, including its proper use or application and possible adverse effects.

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Shaw KC, Kaley JR, Darling MD, Patterson JW, Chokoeva AA, Lotti T, et al. A case of perforating folliculitis in association with antisynthetase syndrome. J Biol Regul Homeost Agents. 2015 Jan-Mar. 29 (1 Suppl):95-8. [Medline].

Gilaberte Y, Coscojuela C, Vazquez C, Rosello R, Vera J. Perforating folliculitis associated with tumour necrosis factor-alpha inhibitors administered for rheumatoid arthritis. Br J Dermatol. 2007 Feb. 156(2):368-71. [Medline].

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Monteiro R, Bhat I, Abraham A, Rajlakshmi T. Perforating Folliculitis Secondary to Bendamustine-Rituximab Chemotherapy: A Case Report. Indian Dermatol Online J. 2017 Jul-Aug. 8 (4):290-292. [Medline].

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Suguru Imaeda, MD Chief of Dermatology, Yale University Health Services; Chief of Dermatology, Veterans Affairs Connecticut Healthcare System; Assistant Professor, Department of Dermatology, Yale University School of Medicine

Suguru Imaeda, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Connecticut State Medical Society, Sigma Xi, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Rosalie Elenitsas, MD Herman Beerman Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System

Rosalie Elenitsas, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society of Dermatopathology, Pennsylvania Academy of Dermatology

Disclosure: Received royalty from Lippincott Williams Wilkins for textbook editor.

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Andrea Leigh Zaenglein, MD Professor of Dermatology and Pediatrics, Department of Dermatology, Hershey Medical Center, Pennsylvania State University College of Medicine

Andrea Leigh Zaenglein, MD is a member of the following medical societies: American Academy of Dermatology, Society for Pediatric Dermatology

Disclosure: Received consulting fee from Galderma for consulting; Received consulting fee from Valeant for consulting; Received consulting fee from Promius for consulting; Received consulting fee from Anacor for consulting; Received grant/research funds from Stiefel for investigator; Received grant/research funds from Astellas for investigator; Received grant/research funds from Ranbaxy for other; Received consulting fee from Ranbaxy for consulting.

The authors and editors of Medscape Reference gratefully acknowledge the significant contributions of previous author, James W. Patterson, MD, to the development and writing of this article which has been recently updated.

Perforating Folliculitis

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