Case report
Facial discoid dermatosis* Christine J. Ko, MD, Peter Heald, MD, Richard J. Antaya, MD, Jean L. Bolognia, MD
Departments of Dermatology and Pathology, and Dermatology, Yale University School of Medicine, New Haven, CT, USA Correspondence Christine J. Ko MD Department of Dermatology and Pathology Yale University School of Medicine 333 Cedar St PO Box 208059 New Haven CT 06520 E-mail:
[email protected] *Patient 2 in this study was presented at the New England Dermatological Society Conference in New Haven, CT, September 2007.
Introduction In adults, the clinical differential diagnosis of discrete, thin papulosquamous lesions of the face includes tinea faciei, seborrheic dermatitis, psoriasis (especially if near the hairline and in the setting of psoriasis elsewhere on the body), and cutaneous lupus erythematosus (primarily discoid). Rarely, parapsoriasis or mycosis fungoides limited to the face would be considered. Most commonly, histologic examination is performed for confirmation when cutaneous lupus erythematosus is either the leading diagnosis or a strong consideration. On occasion, the clinician may then be surprised, or even confused, when the histologic features are said to suggest the diagnosis of pityriasis rubra pilaris (PRP), despite the absence of lesions elsewhere on the body and a lack of progression over time. Herein we describe three patients with persistent, discrete papulosquamous lesions that were limited to the face, had histologic features of PRP, and proved unresponsive to topical therapies. The name we suggest for this disorder is ‘‘facial discoid dermatosis.’’
Department of Dermatology at Yale University School of Medicine are outlined in Table 1. The lesions varied in size from 4 mm to 3.5 cm, were unilateral or bilateral, and limited to the face (Fig. 1). They were first noted in the second to fourth decade of life. Their color ranged from pink to pink–orange and the associated scale was dry and sometimes annular. The most common clinical diagnoses were psoriasis, cutaneous lupus erythematosus, and seborrheic and contact dermatitis. Multiple topical medications were utilized in these patients, including corticosteroids (classes 4–7), calcipotriene, antifungals, calcineurin inhibitors, imiquimod, and retinoids; one patient was also treated with oral doxycycline and pulsed-dye laser. None of these therapies led to any clinical improvement. With the exception of one lesion (in one patient) that resolved after it was biopsied, none of the patients had resolution or progression of lesions. Summary of histologic features
Interface changes were notably absent, and there was acanthosis with dry parakeratosis. Two of the three cases had follicular plugging (Fig. 2, Table 1).
Case series Summary of clinical characteristics
The demographic features of the three patients observed over a period of 15 years in the faculty practice of the ª 2010 The International Society of Dermatology
Discussion We present three patients with discoid, papulosquamous lesions on the face that were resistant to multiple topical International Journal of Dermatology 2010, 49, 189–192
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Ko et al.
Table 1 Clinical and histologic findings in three patients with facial discoid dermatosis Patient
1
2
3
Gender Age of onset No. lesions Size of lesions Location Lesion morphology
Male 39 12–14 4–15 mm Cheeks ⁄ beard area Thin orange–pink papules and plaques with scale; well-defined, bilateral Psoriasis, cutaneous lupus erythematosus
Female 11 2 15 mm Right cheek Discoid, pink, slightly scaly
Female 19 2 1–3.5 cm Forehead Scaly, pink thin plaques
Contact dermatitis, psoriasis, tinea, persistent bite reaction
Psoriasiform epidermal hyperplasia with follicular plugging ANA-negative Anti-Ro ⁄ La Ab – negative Numerous melanocytic nevi
Focal perifollicular parakeratosis, acanthosis, perivascular lymphocytes KOH-negative
Facial discoid dermatosisa, contact dermatitis, cutaneous lupus erythematosus, seborrheic dermatitis Psoriasiform epidermal hyperplasia, follicular plugging
Hand dermatitis
Atopic dermatitis
Stable for 15 years despite topical corticosteroids, calcipotriene
First lesion resolved postbiopsy; second stable for 3 years despite topical corticosteroids, ketoconazole, imiquimod, and tacrolimus as well as oral doxycycline and PDL
Stable for 7 years despite topical antifungals, tacrolimus, pimecrolimus, and tazarotene
Clinical differential
Histopathologic findings Laboratory evaluation Other dermatologic findings Clinical follow-up
None
ANA, antinuclear antibody; PDL, pulsed dye laser. a Diagnosis made after observing patient 2.
therapies (Table 1); in one patient, lesions even failed to improve with oral antibiotics and pulsed-dye laser treatments (case 2). Clinically, the disease remained localized to the face, and its stable nature, over a period of years, was noteworthy. The key features of facial discoid dermatosis are summarized in Table 2. While the clinical differential diagnosis included a number of entities, from psoriasis and cutaneous lupus erythematosus to contact dermatitis, the specific histologic
(a)
features of these disorders were not only absent, but the findings among this group of patients were remarkably similar (Table 3). Therefore, the major question is whether facial discoid dermatosis should be considered a separate clinical entity or, given its overlap with PRP, a previously unrecognized type of PRP,1,2 i.e., type VII PRP. Clinical similarities include the orange–pink color and dry scale, and histologically, hyperkeratosis and parakeratosis with follicular plugging are seen in both disor-
(b)
(c)
(a) Patient 1, (b) patient 3, and (c) patient 2 with well-demarcated pink papules and plaques with arcuate scale on the face. Patient 2’s initial lesion resolved postbiopsy
Figure 1
International Journal of Dermatology 2010, 49, 189–192
ª 2010 The International Society of Dermatology
Ko et al.
Facial discoid dermatosis
Case Report
Figure 2 Photomicrographs from patient 1 (above) and patient 2 (below). Hematoxylin and eosin staining, 4· on left and 10· on right. Acanthosis with hyperkeratosis and follicular plugging Table 2 Key features of facial discoid dermatosis Facial discoid dermatosis Discoid Minimal dry scale, may be annular Limited to the face Stable over years Treatment-resistant Nonspecific histology, with features resembling pityriasis rubra pilaris
ders (Table 3). Lastly, both entities can be difficult to treat. That said, there are clear differences. None of our patients had extrafacial disease, follicular papules, disease
progression, or clinical resolution over a period of 3– 15 years. Perhaps this question of two separate entities versus two variants of a single disorder will remain unanswered until we have gained greater insights into the pathogenesis of PRP. Recent studies have described foci of acantholysis in biopsy specimens of PRP,3 suggesting the possibility of abnormal desmosomal adhesion. Once the underlying disease process in PRP had been clarified, a search for similar findings in facial discoid dermatosis could be conducted. Nonetheless, it is hoped that recognition of the distinctive clinical and histologic features of ‘‘facial discoid dermatosis’’ will decrease the number of
Table 3 Histologic features of facial discoid dermatosis compared with other inflammatory disorders in the differential diagno-
sis
Hyper- ⁄ para-keratosis ‘‘Checkerboard’’ alternating ortho- ⁄ para- keratosis Follicular plugging Neutrophils in the stratum corneum Hypogranulosis Acanthosis Acantholysis Spongiosis Interface change Dermal mucin
ª 2010 The International Society of Dermatology
Facial discoid dermatosis
Cutaneous lupus erythematosus
Psoriasis
Seborrheic dermatitis
Contact dermatitis
Pityriasis rubra pilaris
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patients who currently lack a diagnosis for their persistent facial lesions. References 1 Griffiths WA. Pityriasis rubra pilaris. Clin Exp Dermatol 1980; 5: 105–112.
International Journal of Dermatology 2010, 49, 189–192
Ko et al.
2 Miralles ES, Nunez M, De Las Heras ME, et al. Pityriasis rubra pilaris and human immunodeficiency virus infections. Br J Dermatol 1995; 133: 990–993. 3 Howe K, Foresman P, Griffin T, Johnson W. Pityriasis rubra pilaris with acantholysis. J Cutan Pathol 1996; 23: 270–274.
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