Wong-Type Dermatomyositis Showing Porokeratosis-Like Changes (Columnar Dyskeratosis): A Case Report and Review of the Literature

Abstract

Background: Wong-type dermatomyositis (DM) exhibits simultaneous pityriasis rubra pilaris (PRP) features. Case Report: A 50-year-old woman presented with a heliotrope rash, Gottron's papules, and a poikilodermic, erythematous rash in shawl distribution without evidence of muscle weakness. Despite topical corticosteroids, the eruption progressed 9 months later to include generalized hyperkeratotic follicular papules, islands of sparing, and atrophic macules with a collarette of scale suggestive of porokeratosis. Mild dysphonia was the only sign of muscle weakness. Serology showed positive ANA. Histopathology revealed interface dermatitis with dermal mucin and melanophages, irregular psoriasiform hyperplasia, alternating mounds of para- and orthokeratosis, and tiers of dyskeratotic cells (columnar dyskeratosis). Systemic corticosteroid therapy was not tolerated; acitretin diminished the hyperkeratosis. While hyperpigmentation persisted, no progression of cutaneous or muscular symptoms has occurred after 22 months of follow-up and cessation of the therapy. Overall, her course did not differ from the natural history documented in the literature review of Wong-type DM. The most similar case also exhibited pseudocornoid lamella changes. Conclusion: Wong-type DM is a clinicopathologic DM-PRP hybrid that can also exhibit porokeratosis-like features best described as columnar dyskeratosis. Recognizing these types of lesions in DM is warranted in order to make an accurate assessment of their prognostic significance.

Covert clues: the non-hallmark cutaneous manifestations of dermatomyositis

“…Hyperkeratosis of the palms and soles is often present. Histopathologically, an overlap between the two disease entities is likewise observed, with vacuolar interface alterations and abundant dermal mucin occurring with alternating mounds of para-and orthokeratosis and follicular plugging (115,116). Onset of PRP varies and can occur prior to, simultaneously with, or after the diagnosis of DM.…”

Wong‐type dermatomyositis with pulmonary involvement in a teenager: case report

“…In this variant, the skin has follicular papules that may mimic PRP on clinical presentation and on skin biopsy. This subtype affects patients between nine and 89 years of age [5], while classic DM predominantly affects females and may present with severe muscle involvement and internal organ damage [5]. Twenty-nine cases have been reported in the literature [5,6].…”

“…Cutaneous signs of DM, if present, can lead to diagnosis. On histological study, the most common findings are follicular hyperkeratosis and compact orthokeratosis within non-follicular epidermal invaginations [5]. Most cases show findings of DM, such as vacuolar interface dermatitis or dermal mucin deposition; exceptional findings include dyskeratosis, compact orthokeratosis alternating with parakeratosis in horizontal and vertical directions, and arrector pili inflammation [6].…”

Dermatomyositis vom Wong‐Typ mit Lungenbeteiligung im Teenageralter: ein Fallbericht

“…Bei dieser Variante zeigen sich an der Haut follikuläre Papeln, die klinisch und in der Hautbiopsie wie eine PRP imponieren können. Dieser Subtyp tritt bei Patienten jeder Altersgruppe (von 9 bis 89 Jahren) auf [5], die klassische DM findet sich bevorzugt bei weiblichen Patienten und kann mit einer schweren Muskelbeteiligung und der Schädigung innerer Organe einhergehen [5]. In der Literatur finden sich Berichte über 29 Fälle einer DM vom Wong-Typ [5,6].…”

“…Die charakteristischen Hauterscheinungen der DM können, wenn vorhanden, diagnostisch wegweisend sein. Die häufigsten histologischen Befunde sind follikuläre Hyperkeratose und kompakte Orthokeratose innerhalb nicht-follikulärer epidermaler Invaginationen [5]. In den meisten Fällen finden sich Anhaltspunkte für DM wie eine vakuoläre Interface-Dermatitis oder dermale Muzin-Ablagerungen; ausnahmsweise kommen auch Dyskeratosen, kompakte Orthokeratosen im Wechsel mit Parakeratosen in vertikaler und horizontaler Richtung sowie Entzündungen der Haarbalgmuskeln (Musculi arrectores pilorum) vor [6].…”