Saving face: The importance of recognizing facial discoid dermatosis
By Warren R. Heymann, MD, FAAD
Feb. 22, 2023
Vol. 5, No. 8
I get excited when astute dermatologists describe and label dermatoses that I have seen but could not adequately diagnose. Two recent examples include acute inflammatory edema and alpha-gal syndrome. I (and presumably you too) have encountered patients with persistent, well-defined, minimally scaly, erythematous plaques on the face that were psoriasiform, but without other evidence of psoriasis, seborrheic dermatitis, chronic cutaneous (discoid) lupus, tinea, or contact dermatitis. I have biopsied such patients to be certain that these lesions were not keratinocyte carcinomas, with the pathology reports interpreted as psoriasiform dermatitis. I have treated these patients with the proverbial kitchen sink (topical steroids, calcineurin inhibitors, retinoids, excimer laser, etc.) to little avail.
The eye sees only what the mind is prepared to comprehend — Robertson Davies (but often attributed to Henri Bergson)
In 2010, Ko et al reported 3 patients with discrete facial pink-orange papulosquamous lesions. None had extra-facial lesions, follicular papules, disease progression, or clinical resolution over a period of 3 to 15 years. The authors coined the term “facial discoid dermatosis” (FDD) based on the following key features: 1) a discoid appearance that may be annular; 2) minimal dry scale; 3) limitation to the face; 4) stability over years; 5) treatment-resistance (to topical steroids, calcipotriene, calcineurin inhibitors, calcipotriene, retinoids, imiquimod, doxycycline, and pulsed dye laser); 6) a non-specific histology (with features of pityriasis rubra pilaris [PRP] – acanthosis, parakeratosis, and follicular plugging). The authors speculated that perhaps FDD is a unique variant of PRP. Despite the lack of understanding of the nature of FDD, the authors concluded: “Nonetheless, it is hoped that recognition of the distinctive clinical and histologic features of “facial discoid dermatosis” will decrease the number of patients who currently lack a diagnosis for their persistent facial lesions.” (1)
Salman et al reported a case of FDD in a 26-year-old woman who failed multiple treatment modalities including oral itraconazole. Histologically, psoriasiform hyperplasia, foci of parakeratosis (some of which was around the follicular ostia), slight spongiosis, keratotic plugs, and Demodex mites were appreciated. (2)
Gan et al performed a retrospective cross-sectional analysis of clinical and histological data of known cases of recalcitrant psoriasiform dermatoses of the face diagnosed at National Skin Centre, Singapore, over 10 years. There were 8 Chinese patients with a mean age at onset of 29 years. The majority had pink to pink-orange well-defined plaques with dry scale (n = 6, 75%), distributed mostly on the cheeks (100%) and chin (n = 7, 88%). Hyperkeratosis, parakeratosis, a preserved granular layer, and psoriasiform hyperplasia were demonstrated in all biopsies. Other common findings included subtle subcorneal acantholysis, "checkerboard" alternating ortho-/parakeratosis, vacuolated keratinocytes, and follicular plugging. All patients showed little treatment response. One patient eventually developed features of type II PRP. The authors surmised that this recalcitrant psoriasiform facial dermatosis is a distinct entity, with consistent and reproducible clinical features and PRP-like histology, bearing a resemblance to FDD. They contend that FDD is likely a forme fruste of PRP, acknowledging that further studies will be necessary to delineate the nature of the eruption. (3)
Wellborn et al reported a case of FDD in a 44-year-old woman whose biopsy revealed parakeratosis, psoriasiform hyperplasia, follicular plugging, and involuted sebaceous lobules. Sebaceous lobule atrophy may be observed in psoriasis and seborrheic dermatitis but has not been appreciated in PRP. (4) Is this case a variant of FDD?
Despite the recalcitrance to most therapeutic maneuvers, there have been cases successfully treated with topical calcipotriol combined with clobetasol or betamethasone in combination with low-dose acitretin. (5,6) To the best of my knowledge, topical ivermectin has not been utilized in FDD but based on the biopsy report described by Salman et al (2), I would consider prescribing it empirically. If FDD is a forme fruste of PRP, I anticipate seeing reports of newer modalities for recalcitrant PRP (anti-IL-17, anti-IL-23, apremilast) for the disorder. Indeed, Rypka et al reported a case of PDD that improved with ustekinumab within 6 weeks. (7) The key to optimal therapy is understanding pathogenesis. Could FDD be related to the CARD14-associated papulosquamous eruption, which has overlapping features of psoriasis and PRP? (8)
Point to Remember: Facial discoid dermatosis is a recently defined recalcitrant disorder that requires differentiation from psoriasis, tinea, and cutaneous lupus. Most cases suggest a possible relationship to pityriasis rubra pilaris, but this requires further study.
Our expert’s viewpoint
Christine J. Ko, MD, FAAD
Professor of Dermatology and Pathology
Yale School of Medicine
This is a lovely review by Dr. Heymann of facial discoid dermatosis, a term coined to describe circular to annular lesions, seemingly limited to the face, unresponsive to many different treatments, with psoriasiform histopathology. As a diagnostic label, facial discoid dermatosis was intended to help categorize patients, given that discoid lupus erythematosus (with potential systemic implications) is often in the clinical differential diagnosis.
The underlying pathogenesis of facial discoid dermatosis remains unclear, and further research on this condition would be valuable. As Dr. Heymann mentions, genetic screening of these patients, possibly starting with the CARD14, may help to further support any relationship with other psoriasiform disorders like psoriasis and pityriasis rubra pilaris. Support for categorizing facial discoid dermatosis as a psoriasiform dermatosis includes microscopic findings (including sebaceous gland atrophy), clinical morphology (orange color, dry scale), and the described development of more extensive pityriasis rubra pilaris in a patient with facial discoid dermatosis. As facial discoid dermatosis is recalcitrant to many treatments but seems related to psoriasiform processes, trying the recently approved topical JAK inhibitor may be helpful, and it would be worthwhile to study a cohort of these patients. Given the seeming rarity of the condition (few cases reported to date), some sort of registry would likely be useful (and necessary) in order to study a greater number of these patients.
Ko CJ, Heald P, Antaya RJ, Bolognia JL. Facial discoid dermatosis. Int J Dermatol. 2010 Feb;49(2):189-92. doi: 10.1111/j.1365-4632.2009.04206.x. PMID: 20465645.
Salman A, Tekin B, Berenjian A, Cinel L, Demirkesen C. Facial discoid dermatosis: A further case of a novel entity. J Dermatol. 2015 Nov;42(11):1132-3. doi: 10.1111/1346-8138.13045. Epub 2015 Aug 18. PMID: 26283238.
Gan EY, Ng SK, Goh CL, Lee SSJ. Recalcitrant psoriasiform dermatosis of the face: Is it related to pityriasis rubra pilaris? J Cutan Pathol. 2018 Jul;45(7):491-497. doi: 10.1111/cup.13148. Epub 2018 Apr 23. PMID: 29604103.
Welborn M, Fletcher D, Motaparthi K. Atrophy of sebaceous lobules in facial discoid dermatosis: a link to psoriasis and seborrheic dermatitis? J Cutan Pathol. 2022 Mar;49(3):320-323. doi: 10.1111/cup.14190. Epub 2022 Jan 4. PMID: 34939214.
Amarnani R, Hughes S, Morris-Jones R, Kanwar AJ, Bunker CB. Persistent facial discoid dermatosis successfully treated with topical calcipotriol. Clin Exp Dermatol. 2022 Jan;47(1):229-231. doi: 10.1111/ced.14945. Epub 2021 Oct 14. PMID: 34648653.
Bohdanowicz M, DeKoven JG. Improvement in facial discoid dermatosis with calcipotriol/betamethasone ointment and low-dose acitretin. Clin Exp Dermatol. 2018 Oct;43(7):820-821. doi: 10.1111/ced.13611. Epub 2018 May 16. PMID: 29770475.
Rypka KJ, Fulk TS, Afsaneh A, Miller DD, Goldfarb NI. Improvement of Facial Discoid Dermatosis With Ustekinumab Treatment. JAMA Dermatol. 2022 Jun 29. doi: 10.1001/jamadermatol.2022.2478. Epub ahead of print. PMID: 35767241.
Craiglow BG, Boyden LM, Hu R, Virtanen M, Su J, Rodriguez G, McCarthy C, Luna P, Larralde M, Humphrey S, Holland KE, Hogeling M, Hidalgo-Matlock B, Ferrari B, Fernandez-Faith E, Drolet B, Cordoro KM, Bowcock AM, Antaya RJ, Ashack K, Ashack RJ, Lifton RP, MilstoneLM, Paller AS, Choate KA. CARD14-associated papulosquamous eruption: A spectrum including features of psoriasis and pityriasis rubra pilaris. J Am Acad Dermatol. 2018 Sep;79(3):487-494. doi: 10.1016/j.jaad.2018.02.034. Epub 2018 Mar 1. PMID: 29477734; PMCID: PMC6098739.
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