Pityriasis rubra pilaris

Abstract

Erythema gyratum repens (EGR) is a rare skin syndrome often associated with internal malignancies, and thus considered paraneoplastic (1). There are cases of erythema gyratum repens in the literature associated with other dermatoses without underlying malignancies'. We present a case of resolving psoriasis which evolved into erythema gyrarum repens when treated with acitretin.

Case Report

A 64-year-old white man presented to the dermatology department at the University of Arkansas for Medical Sciences in December 2001 with diagnosis of psoriasis by recent biopsy. The patient had tried multiple topical steroids without significant relief. The patient stated that the eruption developed after using ciclopirox topical solution for onychomycosis. The patient complained of intense pruritus. Two more biopsies were performed and were again consistent with psoriasis (Figures 1 and 2). The patient was started on acitretin 50 mg qd, clobetasol scalp solution, and gemfibrozil 600 mg qd. After one month the patient improved slightly but had again worsened by his two month follow-up.

[Figure 1 and 2 Omitted]

The patient's physical exam varied from generalized, scaly erythema with orange-red skin color to a generalized wood-grain erythema with trailing scale (Figures 3A, 3B). The acitretin was discontinued, and the eruption was modified to an exfoliative erythroderma. The patient requested re-initiation of acitretin. The wood-grain findings returned for two months, with later improvement of his skin and resolution without sequelae.

Histologic sections of two punch biopsies done on the wood-grain erythema showed essentially identical findings. There was slight overlying hyperkeratosis, but no significant parakeratosis. The epidermis was acanthotic and mildly spongiotic. Within the dermis, there was a mild superficial perivascular lymphocytic infiltrate. The following laboratory data was obtained: ANA negative, CBC normal, AST 24, ALT 30, cholesterol 199, TG 178, KOH negative. CT scans of chest/abdomen/pelvis were negative except for a benign-appearing unilateral renal cyst.

Comments

Erythema gyratum repens (EGR) is a figurate or gyrate erythema first described by Gammell in 1953 (2). Gammell's patient had a pruritic eruption resembling a "knotty cypress board" on her extremities and trunk. The patient was found to have breast cancer, and after radical mastectomy the eruption cleared in 6 weeks (2). Since 1953 there have been approximately 50 cases of erythema gyratum repens reported, and in 84%2 of those cases there have been associated malignancies. The most common malignancies with EGR are as follows: lung (32%), esophageal (8%), and breast (6%) (3). The eruption has also been reported in cancer of the cervix, stomach, pharynx, prostate, bladder, tongue, colon, pancreas3, and kidney (renal cell carcinoma) (4). Erythema gyratum repens has also been seen in otherwise healthy patients without malignancy (5).

The male to female ratio with EGR is 2:1 (6), and all the cases have occurred in Caucasians in their seventh decade. The eruption usually affects the trunk and extremities and spares the hands, feet, and face. EGR has been described as 'wood-like', 'zebralike', 'serpiginous', 'gyrate', and 'whorled' (3). Repens (from Latin, meaning 'to crawl')r reflects the erruption's rate of advance at a rate of 1 cm/day (3).

Histological findings are nonspecific but hyperkeratosis, parakeratosis, and spongiosis are seen (3). Direct immunofluorescence of some cases has shown granular IgG and C3 at basement membrane zone while other studies have been negative (8). One paper reported an increase in the number of Langerhans cells in the upper epidermis as opposed to the normal suprabasilar location indicating that the antigen in EGR may be in the upper epidermis (9).

Other diseases that have been associated with EGR include pulmonary tuberculosis, benign breast hypertrophy, ichthyosis, palmoplantar hyperkeratosis (8), linear IgA disease (10), psoriasis (11), paraneoplastic bullous pemphigoid (12), Sjogren's syndrome, and pityriasis rubra pilaris (PRP) (13).

Four cases of erythema gyratum repens associated with resolving PRP have been reported. Cheesbrough and Williamson in 1985 reported the first two cases describing an EGR-like eruption appearing as PRP resolved (13). Gebauer et al. in 1993 reported two additional cases of EGR occurring in patients being treated with etretinate (1 mg/kg/day) for PRP (14). In all four of these cases the EGR appeared as the PRP began to clear.

To our knowledge we present the first case of EGR that occurred during treatment of psoriasis with acitretin.

References

(1.) Boyd AS, Neldner KH, Menter A. Erythema gyrathem repens: A paraneoplastic eruption. JAAD 1992; 26:757-762.

(2.) Gammell JA. Erythema gyratum repens. AMA Arch Derm Syph 1953; 66:494-505.

(3.) Caux E Lebbe C, Thomine E, Benyahia, Flageul B, Joly P, Rybojad, Morel E Erythema gyratum repens. A case studied with immunofluorescence, immunoelectron microscopy and immunohistochemistry. British Journal of Dermatology 1994; 131 : 102 - 107.

(4.) Kwatra A, McDonald RE, Corriere JN. Erythema gyratum repens in association with renal cell carcinoma. The Journal of Urology 1998; 159:2077.

(5.) Garrett SJ, Roenigk JJ. Erythema gyratum repens in a healthy woman. JAAD 1992; 26(1):121-122.

(6.) Eubanks LE, McBurney E, Reed R. Erythema gyratum repens. The American Journal of the Medical Sciences 2001; 321:302-305.

(7.) Kurzrock R, Cohen PR. Erythema gyratum repens. JAMA 1995; 273:594.

(8.) Albers SE, Fenske NA, Glass LE Erythema gyratum repens: Direct immunofluorescence microscopic findings. JAAD 1993; 29:493-494.

(9.) Wakeel RA, Ormerod AI), Sewell HE White MI. Subcorneal accumulation of Langerhans cells in erythema gyratum repens. British Journal of Dermatology 1992; 26:189-192.

(10.) Caputo R, Bencini PL, Vigo GE Berti E, and Veraldi S. Eruption Resembling Erythema gyratum repens in Linear IgA Dermatosis. Dermatology 1995; 190:235-237.

(11.) Jablonska S, Blaszczyk M, Kozlowska A. Erythema gyratum repens-like psoriasis. International Journal of Dermatology 2000; 39:695-697.

(12.) Hauschild A, Swensson O, Christophers E. Paraneoplastic bullous pemphigoid resembling erythema gyratum repens. British Association of Dermatologists 1999; 140:538-568.

(13.) Cheesbrough MJ, Williamson DM. Erythema gyratum repens, a stage in the resolution of pityriasis rubra pilaris? Clinical and Experimental Dermatology 1985; 10:466-471.

(14.) Gebauer K, Singh G. Resolving Pityriasis Rubra Pilaris Resembling Erythema Gyratum Repens. Arch Dermatology 1993; 129:917-918.

Address for Correspondence:

Matthew E. Bryan MD

University of Arkansas for Medical Sciences

Department of Dermatology

4301 W. Markham, Slot 576, Little Rock, AR 72205

Phone: 501-526-6551

Fax: 501-686-7264

E-mail: bryanmatthewe@uams.edu

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