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Waiting for Wong


DII small banner By Warren R. Heymann, MD
Oct. 6, 2016

The Wong variant of dermatomyositis was originally described by O’Leary during the Eisenhower administration (1953) and named for Wong during Nixon’s tenure (1969). I have been involved with dermatology since Carter’s term in office (1979). Since that time (Reagan, Bush, Clinton, Bush, Obama) I have yet to see a case of the condition.

To date, two-dozen cases of Wong-type dermatomyositis (WD) have been reported. The hallmark of WD is that it mimics pityriasis rubra, pilaris (PRP) clinically and histologically. They are differentiated microscopically by the presence of vacuolar alteration at the epidermal-dermal junction and mucin deposition in WD. Compact orthokeratosis within follicular and non-follicular epidermal invaginations (1). Plugged follicular infundibula mandates that similar disorders be considered — PRP, lupus, follicular psoriasis, keratosis pilaris, and folliculotropic cutaneous T cell lymphoma. WD may be seen in children and adults. Aside from other classical features of dermatomyositis (heliotrope rash, Gottron papules, myositis, pulmonary disease, etc.), malignancies have been reported in Wong’s paper, however, in no subsequent reports of the entity (1).
 
Adding to the confusion, there have now been two reports of “pseudocornoid” lamellae in WD. The first report of porokeratotic lesions was in a 53-year-old woman with WD. Histologically they were described as a “focal loss of the granular layer with a dell in the epidermis containing a stacked column of horny cells some retaining their nuclei” (2). Umanoff et al detail the case of a 50 year-old woman with WD, manifested by a heliotrope rash, Gottron papules, poikiloderma in a shawl-distribution, follicular papules, and lesions with a collarette of scale suggesting porokeratosis. The histology was also called a pseudocornoid lamella (columnar dyskeratosis), not a typical cornoid lamella, because of a vertical rather than diagonal arrangement of the column. (I have never pretended to have the dermatopathologic skills of a Bernie, Wallace, or Dirk, but for the life of me, I would have easily called these lesions cornoid lamellae, not “pseudocornoid” lamellae. Regardless, porokeratotic lesions may be a rare manifestation of an exceedingly rare dermatosis).
 
Another recent finding in the realm of WD was the report of a 54 year-old man with multiple violaceous palmar and digital papules, myositis, and pulmonary disease correlating with anti-MDA5 antibodies. His PRP-like features included salmon-pink truncal patches with islands of sparing; follicular papules were noted on the extremities. The presence of vacuolar alteration on his skin biopsy confirmed that these lesions were those of WD (3).

As of January 20th, 2017 we will have either President Clinton or President Trump. Will I see a case of WD in the next administration? I don’t know — but if I make the diagnosis, that will be HUGE.

1. Mutasim DF, et al. Wong-type dermatomyositis: A mimic of many dermatoses. J Cutan Pathol 2016; 43: 781-6.
2. Lupton JR, et al. An unusual presentation of dermatomyositis: The Wong variant revisited. J Am Acad Dermatol 2000; 43: 908-12.
3. Canavan T, et al. A case of Wong-type dermatomyositis with concomitant anti-MDA5 features. J Am Acad Dermatol 2014; 70; e62-3.

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