Oral pityriasis rubra pilaris

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Oral pityriasis rubra pilaris Luis Edmundo Martinez Calixto, DDS,a Lakshmanan Suresh, BDS,a Emiko Matsumura, DDS, PhD,a Alfredo Aguirre, DDS, MS,b and Lida Radfar, DDS, MS,c Buffalo, NY STATE UNIVERSITY OF NEW YORK AT BUFFALO

Pityriasis rubra pilaris is a chronic, papulosquamous dermatosis of unknown etiology. Oral mucosal involvement of this condition is rare with only 3 previously reported cases in the English literature. A case of a 68-year-old man with pityriasis rubra pilaris with involvement of tongue and palate is presented. Clinical features, histopathologic features, and management of pityriasis rubra pilaris are discussed. We hereby present an additional case, the fourth worldwide, and review the literature. (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;101:604-7)

Pityriasis rubra pilaris (PRP) is a chronic skin disorder of unknown etiology characterized by keratotic follicular papules, palmoplantar hyperkeratosis, follicular plugging, and perifollicular erythema.1 The incidence of PRP in the United States has been reported to be 1 case per 3,500-5,000 patients.1 No racial or sex predilection is seen in PRP.1 There is a bimodal age distribution of PRP with peaks in the first and the fifth decades.2,3 The lesions in PRP usually begin in the scalp and progress caudally.4,5 The lesions are usually symmetric and bilateral. The skin of the palms, soles, phalanges, wrists, and thighs are the commonly affected areas, but any area of the skin and even the nails may be affected.1,6-8 Oral mucosal involvement of PRP is rare and, to our knowledge, there have been only three reported cases in the English literature.9-11 The purpose of this article is to report a case of PRP with oral involvement and to characterize the clinical, histopathological findings and treatment.

Fig. 1. Classic salmon-colored skin lesions of PRP with intermingling islands of normal skin on the abdomen.

CASE REPORT A 68-year-old white man presented to the Oral Medicine Clinic at School of Dental Medicine, State University of New York at Buffalo, with a chief complaint of ‘‘pain and irritation of the tongue.’’ History revealed that his symptoms of pain and irritation of the tongue began one year ago and has


Resident, Advanced Oral and Maxillofacial Pathology, Department of Oral Diagnostic Sciences, School of Dental Medicine, State University of New York at Buffalo, NY. b Professor, Oral and Maxillofacial Pathology, Department of Oral Diagnostic Sciences, School of Dental Medicine, State University of New York at Buffalo, NY. c Associate Professor, Oral Medicine, Department of Oral Diagnostic Sciences, School of Dental Medicine, State University of New York at Buffalo, NY. Received for publication Jun 14, 2005; returned for revision Aug 9, 2005; accepted for publication Aug 22, 2005. 1079-2104/$ - see front matter Ó 2006 Mosby, Inc. All rights reserved. doi:10.1016/j.tripleo.2005.08.036


Fig. 2. Erythroderma with keratosis of the palms. since been gradually getting worse. Precipitating factors of pain included intake of hot and spicy food. His past medical history was significant for hypertension, benign prostrate hypertrophy, and PRP of the skin diagnosed 3 years earlier. Because of the possibility that PRP may be a manifestation of a serious underlying systemic disease, the patient underwent a thorough physical and laboratory examination that failed to reveal any abnormality. His medications included

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Fig. 3. Keratotic plaques and atrophy of the nails.

Fig. 5. Incisional biopsy of the dorsum of the tongue showing a parakeratinized epithelium with a mild chronic inflammatory infiltrate composed of lymphocytes in the connective tissue, mild spongiosis and lymphocytic exocytosis with focal basal cell degeneration (hematoxylin-eosin, original magnification 3 200).

Fig. 4. White keratin plaques and nodes on the dorsum of the tongue. nifedipine and acitretin. He was allergic to penicillin. He did not smoke and was an occasional drinker. The family history was not contributory. On physical examination, generalized scaly plaques of salmon color with sharp borders intermingling with islands of normal skin (Fig. 1), with palmoplantar erythroderma (Fig. 2) were noted. The nails showed white and brown keratotic plaques with atrophy (Fig. 3). The skin lesions corresponded to the diagnosis of PRP. Intra-oral examination revealed white plaques and nodules on the dorsal and ventral surfaces of the tongue (Fig. 4). The hard and soft palate were also involved. An incisional biopsy of the dorsum of the tongue was carried out under local anesthesia. Histological examination revealed parakeratinized surface epithelium with connective tissue showing a chronic inflammatory infiltrate composed mainly of lymphocytes (Fig. 5). A diagnosis of chronic non-specific mucositis consistent with PRP was rendered. Previous slides of skin biopsy done 3 years earlier were requested and showed psoriasiform epidermal changes which included broad rete ridges, narrow dermal papillae, and sparse superficial dermal lymphocytic infiltration. Follicular keratin plugging with moderate to dense perivascular, and perifollicular lymphocytic infiltration were also seen. Histological features were consistent with PRP (Fig. 6).

Fig. 6. Skin biopsy showing hyperparakeratosis, with a mild conical keratin plug (arrow), intermittent hypergranulosis, broad and thin rete pegs, and perivascular chronic inflammation in the superficial dermis (hematoxylin-eosin, original magnification 3 20). The patient was placed on dexamethasone elixir (0.5 mg/ 5 ml) q.i.d. for two weeks. Clinically, his oral lesions were reduced by 70%. Nevertheless, all the symptoms resolved and the patient was able to eat foods without any problems within 3 weeks of treatment.

DISCUSSION Pityriasis rubra pilaris is a skin disorder of follicular keratinization characterized by progressive erythroderma that contains patches of normal-appearing skin

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606 Martinez Calixto et al. Table I. Modified Griffith’s classification for PRP Classification

Frequency (%)

Clinical presentation

Type I (Classic adult)


Type II (Atypical adult) Type III (Classic juvenile) Type IV (Circumscribed juvenile)

5 10 25

Erythroderma with islands of skin sparing, palmoplantar keratoderma, and follicular hyperkeratosis Chronic skin changes including areas of eczema, and alopecia Similar to type I, but the onset is within the first 2 years of life Sharply demarcated areas of follicular hyperkeratosis and erythroderma of the knees and the elbows Early onset with a chronic course and it is characterized by prominent follicular hyperkeratosis, and infrequent erythroderma May have cystic and pustular nodules

Type V (Atypical juvenile) Type VI

5 Unknown

Table based on data from Griffith.12

called ‘‘islands of sparing’’. Other common clinical presentations include hyperkeratotic lesions of the palms and soles that are distinctly orange, follicular papules on the dorsal aspects of phalanges and exterior aspects of the wrists and thighs, and ectropion.1,6,7 The lesions are usually symmetrical and diffuse, and with time the lesions spread and coalesce.6 The nails show a yellow-brown discoloration, subungual hyperkeratosis, nail-plate thickening, and splinter hemorrhages.8 The Table I illustrates the six recognized types of PRP.1,12,13 The clinical findings of our case were consistent with type I (classic adult-type). Oral mucosal involvement is rare and the first case was reported involving the palate by Sutton and Sutton in 1939.10 Since then two additional cases of PRP involving the buccal mucosa have been described.9,11 In those cases, the oral presentation of PRP ranged from discrete white plaques confined to the palate10 to generalized bilateral gray-white plaques with a rough surface in the buccal mucosae11 and erythematous lesions with white streaks clinically reminiscent of lichen planus in the labial mucosae, gingivae, and soft palate.9 Our case had erythematous white papules and plaques on the dorsal and ventral surfaces of the tongue, and on the hard and soft palate, a clinical appearance suggestive of papular lichen planus. The differential diagnosis of oral PRP should include papular lichen planus, Darier’s disease (DD), papillary hyperplasia of the palate, and nicotine stomatitis. Oral papular lichen planus may be difficult to distinguish from the oral manifestations of DD. However, oral popular lichen planus presents with an associated symmetric and pruritic papulosquamous dermatosis with characteristic Wickham’s striae and typical microscopic and immunologic features that are distinct from PRP.14 Oral DD is most commonly seen in the palate followed by the gingival, buccal mucosa, and tongue. In about 30% of patients with DD, parotid swelling is also present.15 Clinically, DD presents with skin eruptions of scattered brownish keratotic papules forming malodorous plaques, whereas PRP demonstrates characteristic bilateral cutaneous hyperkeratosis

with surrounding erythema.16 Papillary hyperplasia of the palate is associated with a complete denture and is not accompanied by a skin disorder. Nicotine stomatitis is confined to the hard palate, it is associated with a history of heavy smoking and is not linked to a cutaneous condition. The microscopic features of PRP of the skin may be non-specific and consist of orhtokeratosis, parakeratosis in vertical and horizintal directions, confluent hypergranulosis, broad epidermal ridges, lymphocytic exocytosis, spongiosis, acantholysis, and, in some cases, a lichenoid infiltrate in the dermis.14 The presence of acantholysis may be reminiscent of the microscopic features of DD. Furthermore, PRP can be confused with psoriasis not only at the microscopic level but also clinically.14 The microscopic features of oral PRP were described in only 1 of the 3 oral cases published so far. In that case, perivascular edema in the papillary lamina propria and a moderately dense superficial chronic inflammatory cell infiltrate were observed. In addition, subepithelial clefting and slight vacuolar degeneration of the basal cell layer were also reported.9 Our case showed a moderate diffuse infiltrate of lymphocytes in the papillary lamina propria amidst numerous blood capillaries, mild lymphocytic exocytosis, and focal basal cell degeneration. These microscopic findings are not inconsistent with those previously reported in oral PRP. The pathogenesis of PRP is unknown. There may be an increased rate of epidermal cell growth due to an unknown stimulus.17 This is supported by abnormalities in biochemical markers of epidermal differentiation found in patients with PRP. Activated T suppressor cells and impaired T helper cells have also been isolated from patients with PRP.18 Pityriasis rubra pilaris can be the initial manifestation of a previously undiagnosed underlying disease such as internal malignancy, myasthenia gravis, AIDS, or leukemia.7,19-23 There are no specific laboratory tests available to confirm the diagnosis of PRP. The diagnosis is made based on the correlation between clinical findings and

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histologic findings. Histologic features are not pathognomonic. The most common histologic feature of PRP are psoriasiform epidermal changes that include broad rete ridges, narrow dermal papillae, and sparse superficial dermal lymphocytic infiltration. Follicular plugging by keratin and moderate to dense perivascular and perifollicular lymphocytic infiltration may also be seen.24 Some of the cases may show focal acantholytic dyskeratosis.25,26 The presence of acantholysis, hypergranulosis, follicular plugging, and the absence of dilated capillaries and sub-epithelial abscess may help distinguish PRP from psoriasis. Our case showed all of the above histological features in the skin biopsy and presence of focal parakeratinized epithelium with chronic inflammatory infiltrate composed of lymphocytes in the tongue biopsy. The goals of treatment are to reduce morbidity and to prevent complications. Currently, retinoids (isotretinoin, etretinate, vitamin A) and antimetabolites (methotrexate and azathioprine) are the most successful in the management of PRP.4,5 Topical calcipotriol has been tried successfully and, more recently, fumaric esters therapy has shown promissory results.27,28 Our patient was managed with systemic retinoids and corticosteroid mouth rinse. REFERENCES 1. Arnold AW, Buechner SA. Circumscribed juvenile pityriasis rubra pilaris. J Eur Acad Dermatol Venereol 2004;18:705-7. 2. Chan H, Liu FT, Naguwa S. A review of pityriasis rubra pilaris and rheumatologic associations. Clin Dev Immunol 2004;11: 57-60. 3. Davidson CL Jr, Winkelmann RK, Kierland RR. Pityriasis rubra pilaris. A follow-up study of 57 patients. Arch Dermatol 1969; 100:175-8. 4. Clayton BD, Jorizzo JL, Hitchcock MG, et al. Adult pityriasis rubra pilaris: a 10-year case series. J Am Acad Dermatol 1997; 36:959-64. 5. Cohen PR, Prystowsky JH. Pityriasis rubra pilaris: a review of diagnosis and treatment. J Am Acad Dermatol 1989;20:801-7. 6. Dicken CH. Treatment of classic pityriasis rubra pilaris. J Am Acad Dermatol 1994;31:997-9. 7. Bonomo RA, Korman N, Nagashima-Whalen L, Briggs J, Graham R, Salata RA. Pityriasis rubra pilaris: an unusual cutaneous complication of AIDS. Am J Med Sci 1997;314:118-21. 8. Sonnex TS, Dawber RP, Zachary CB, Millard PR, Griffiths AD. The nails in adult type 1 pityriasis rubra pilaris. A comparison with Sezary syndrome and psoriasis. J Am Acad Dermatol 1986;15:956-60. 9. Baden HP, Roth SI. Oral lesions in pityriasis rubra pilaris. Oral Surg Oral Med Oral Pathol 1968;25:691-4.

Martinez Calixto et al. 607 10. Sutton RL, Sutton RL Jr. Diseases of the Skin. Vol. 1. 10th ed. St. Louis: CV Mosby; 1939. p. 329. 11. Marshall J. Case of pityriasis rubra pilaris wth lesions of buccal mucosa. AMA Arch Derm Syphilol 1952;66:626-7. 12. Griffiths WA. Pityriasis rubra pilaris: the problem of its classification. J Am Acad Dermatol 1992;26:140-2. 13. White KL. Pityriasis rubra pilaris. Dermatol Online J 2003;9:6. 14. McKee PH, Calonje E, Granter SR. Spongiotic, psoriasiform and pustular dermatoses. In: McKee PH, Calonje E, Granter SR, editors. Pathology of the Skin with Clinical Correlations. 3rd ed. Philadelphia, PA: Elsevier Mosby; 2005. p. 208-9 and 217-8. 15. Mcleod RI, Munro CS. The incidence and distribution of oral lesions in patients with Darier’s disease. Br Dental J 1991;171: 133-6. 16. Magro CM, Crowson AN. The clinical and histomorphological features of pytirasis rubra pilaris: a comparative analysis with psoriasis. J Cutan Pathol 1997:416-24. 17. Marks R, Griffiths A. The epidermis in pityriasis rubra pilaris: a comparison with psoriasis [abstract]. Br J Dermatol 1973; 89(Suppl 9):19-20. 18. Shvili D, David M, Mimouni M. Childhood-onset pityriasis rubra pilaris with immunological abnormalities. Pediatr Derm 1987;4:21-3. 19. Sanchez-Regana M, Lopez-Gil F, Salleras M, Umbert P. Pityriasis rubra pilaris as the initial manifestation of internal neoplasia. Clin Exp Dermatol 1995;20:436-8. 20. Kurzydlo AM, Gillespie R. Paraneoplastic pityriasis rubra pilaris in association with bronchogenic carcinoma. Australas J Dermatol 2004;45:130-2. 21. Reinhardt LA, Rosen T. Pityriasis rubra pilaris as the initial manifestation of leukemia. Cutis 1983;31:100-2. 22. Waldorf DS, Hambrick GW Jr. Vitamin A responsive pityriasis rubra pilaris with myasthenia gravis. Arch Dermatol 1965;92: 424-7. 23. Gonzalez-Lopez A, Velasco E, Pozo T, Del Villar A. HIV-associated pityriasis rubra pilaris responsive to triple antiretroviral therapy. Br J Dermatol 1999;140:931-4. 24. Soeprono FF. Histologic criteria for the diagnosis of pityriasis rubra pilaris. Am J Dermatopathol 1986;8:277-83. 25. Kao GF, Sulica VI. Focal acantholytic dyskeratosis occurring in pityriasis rubra pilaris. Am J Dermatopathol 1989;11:172-6. 26. Hoover WD Jr, Maize JC. Focal acantholytic dyskeratosis occurring in pityriasis rubra pilaris. Am J Dermatopathol 1990;12: 321-3. 27. Van de Kerkhof PC, Steijlen PM. Topical treatment of pityriasis rubra pilaris with calcipotriol. Br J Dermatol 1994;130:675-8. 28. Coras B, Vogt TH, Ulrich H, Landthaler M, Hohenleutner U. Fumaric acid esters therapy: a new treatment modality in pityriasis rubra pilaris? Br J Dermatol 2005;152:388-9. Reprint requests: Dr. Lida Radfar, DDS, MS 355 Squire Hall Department of Oral Diagnostic Sciences School of Dental Medicine State University of New York at Buffalo Buffalo, NY 14214 [email protected]

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