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Ulcerative vulvitis in atypical Reiter's syndome
CASE
REPORTS
Ulcerative vulvitis in atypical Reiter’s syndome Helen E. Lotery, MBBCh,a Rudolph P. Galask, MD, MS,a,b,c Mary Seabury Stone, MD,b,d and Richard D. Sontheimer, MDb Iowa City, Iowa We report a case of atypical Reiter’s syndrome occurring in a female patient who had severe, ulcerative vulvar disease develop in association with conjunctivitis, low back pain, stomatitis, and psoriasiform skin lesions. Vulvar lesions have rarely been described in Reiter’s syndrome and are not well characterized. (J Am Acad Dermatol 2003;48:613-6.)
R
eiter’s syndrome in its classic form consists of a triad of arthritis, urethritis, and conjunctivitis. It is uncommon in females and its presentation may be atypical or incomplete.1
CASE REPORT A 46-year-old woman presented with a 9-month history of painful genital ulceration associated with offensive discharge and bleeding. She had been without medical attention during most of this time as a result of financial hardship. In the weeks before referral to our institution she had been treated with antibiotics (cefixime, azithromycin, cephalexin, and ciprofloxacin) for presumed infection, without success. On further questioning the patient admitted to a 9-month history of painful mouth ulcers; a 9-month history of lower back pain and stiffness which was worse in the morning; a 3-month history of red, sore eyes; and a 3-month history of asymptomatic skin lesions on the limbs. In addition she reported an involuntary weight loss of 30 lb during the 9-month period. She had no urinary symptoms. She had no diarrhea, abdominal pain, or other gastrointestinal symptoms. Medical history included a caesarean hysterectomy at age 24 as a result of chorioamnionitis complicating her fourth pregnancy. In addition she had a history of treatment for alcohol abuse. She had had several sexual partners in the past but no known From the Departments of Obstetrics and Gynecology,a Dermatology,b Microbiology,c and Pathology,d University of Iowa Hospitals and Clinics. Funding sources: None. Conflict of interest: None identified. Reprints not available from authors. Correspondence: Richard D. Sontheimer, MD, Department of Dermatology, University of Iowa Hospitals and Clinics, 200 Hawkins Dr, Iowa City, IA 52242. E-mail: [email protected]. Copyright © 2003 by the American Academy of Dermatology, Inc. 0190-9622/2003/$30.00 ⫹ 0 doi:10.1067/mjd.2003.225
sexually transmitted infections. She had been in her current sexual relationship for 2 years. She had no personal or family history of skin disease or arthropathy. At the time of presentation she was on no routine medication. Since her initial presentation to her family physician she had been given multiple diagnoses suggested by several physicians including syphilis, AIDS, genital warts, candidiasis, herpes simplex, psoriasis, pemphigus vegetans, and vulvar cancer. Examination of the genital area revealed linear ulcerations in the inguinal folds with surrounding verrucous lesions. Adenitis was not a prominent feature. There were widespread pustules, erosions, and erythema affecting the groin, labia majora, labia minora, perineum, and perianal area. The labia majora and minora were edematous and covered with profuse, purulent discharge (Fig 1). Examination of the rest of the skin revealed discrete, scaling, hyperkeratotic lesions on the knees and elbows (Fig 2), and perionychial erythema and crusting affecting several toes. The plantar aspects of both feet were normal at the initial examination but subsequently developed small hyperkeratotic papules. There was superficial ulceration under skin folds of the lower abdomen. Examination of the eyes revealed conjunctival erythema and edema. In the mouth there were multiple small, shallow, circinate ulcers on the buccal mucosa and soft palate. The initial investigations in our institution were performed by the gynecology service and comprised examination under anesthesia followed by 3 vulvar biopsies. There was no evidence of any disease in the vagina. The cervix was absent as a result of previous hysterectomy. The vulvar biopsies all revealed similar findings, namely hyperkeratosis, acanthosis, and neutrophilic microabscesses deep in the epidermis and a dense, mixed inflammatory cell infiltrate in the dermis with focal multinucleated giant cells (Fig 3). There was focal psoriasiform 613
614 Lotery et al
J AM ACAD DERMATOL APRIL 2003
Fig 1. Groin and vulva.
Fig 2. Elbow and forearm.
hyperplasia and the presence of spongiform pustules in the malpighian layer (Fig 4). Direct immunofluorescence microscopy studies were negative. Gram stain and Gomori’s methenamine silver stain were also negative. Further biopsies were then performed by the dermatology and ophthalmology services. Skin biopsy from the elbow revealed hyperkeratosis, parakeratosis, irregular acanthosis, neutrophilic epidermal microabcesses, spongiform pustules, and a mononuclear perivascular infiltrate in the papillary dermis. Gram stain was negative for organisms. Direct immunofluorescence microscopy was negative. Conjunctival biopsies revealed intense acute and chronic inflammation in the stroma. Direct immunofluorescence microscopy and Gram stain were again negative. Weakly positive Chlamydia trachomatis serology was observed (IgG positive 1:16, IgM positive 1:10). IgM was also positive 1:10 for Chlamydia psittaci and Chlamydia pneumoniae, suggesting probable cross-reactivity. Other abnormal findings included
Fig 3. Biopsy specimen of vulva showing hyperkeratosis, acanthosis, neutrophilic microabscesses, and mixed inflammatory cell infiltrate in dermis with focal multinucleated giant cells. (Hematoxylin-eosin stain; original magnification ⫻4.)
Fig 4. Biopsy specimen of vulva showing focal psoriasiform hyperplasia and spongiform pustules. (Hematoxylin-eosin stain; original magnification ⫻10.)
increased platelet count (667,000/mm3), white blood cell count (12.9 k/mm3), erythrocyte sedimentation rate (64 mm/h), and C-reactive protein (5.7
J AM ACAD DERMATOL VOLUME 48, NUMBER 4
mg/dL). The following investigations were normal or negative: routine biochemistry including liver function tests; conjunctival cultures for Chlamydia; vaginal vault cultures for Chlamydia; repeated vulvar cultures for herpes simplex virus and yeast; HIV; rapid plasma reagin; hepatitis serology; radiograph of sacroiliac joints; HLA antigen B27; and serum halogens (bromide and iodide). The patient’s sexual partner was not available for testing for sexually transmitted infection. We made the diagnosis of atypical Reiter’s syndrome on the basis of the constellation of clinical features. This diagnosis was supported by the positive serology for C trachomatis. Histologic features included focal psoriasiform hyperplasia and spongiform pustules consistent with psoriasiform skin lesions (Fig 4) along with other features not typical of psoriasis (Fig 3). The patient was treated with oral acitretin at a dosage of 50 mg daily with subsequent increase to 60 mg daily. In addition she used topical therapy, namely Domeboro’s soaks (aluminum acetate) and triamcinolone 0.1% cream to the abdominal and vulvar lesions; polymyxin B and trimethoprim eye drops; and triamcinolone 0.1% mouthwash. During the following 4 months she improved considerably. The conjunctivitis resolved completely, and the skin lesions decreased in number and size. The vulvar ulcers and verrucous lesions resolved but vulvar erythema, edema, tenderness, and discharge persisted. Unfortunately, at the end of the 4-month period the patient stopped taking acitretin. She was seen again at our dermatology clinic 6 weeks later when all her lesions were recurring and worsening. Acitretin was again prescribed but this time improvement was not as before and 8 months later acitretin was discontinued. We were unsure of the patient’s compliance during this period. Consideration was given to commencement of methotrexate but this was thought to be inadvisable because of the patient’s high alcohol intake. Tacrolimus 0.03% ointment was then initiated for the vulvar and limb involvement with limited benefit. The patient was given tacrolimus 0.1% ointment to use but thereafter did not return to our clinic. Treatment with azathioprine or cyclosporine was also being considered at the time that the patient defaulted.
DISCUSSION Traditionally the classic features of Reiter’s syndrome are urethritis, arthritis, and conjunctivitis occurring in a male patient in response to a urogenital or enteric infection, particularly chlamydial urethritis. Arthritis, in particular, is traditionally a hallmark symptom.2 However, there is considerable variation
Lotery et al 615
in the clinical features of Reiter’s syndrome and it is recognized even in male patients that mucocutaneous manifestations may dominate the initial disease presentation.3 Women constitute a very small proportion of the reported cases and their disease may have an atypical presentation. One case has been described with mucocutaneous features preceding the onset of arthritis by more than 4 years.4 Our patient had erosive vulvar lesions, psoriasiform skin lesions, conjunctivitis, mouth ulcers, and low back pain with stiffness that was worse in the morning, which together pointed to a clinical diagnosis of Reiter’s syndrome. There was no history of diarrhea, abdominal pain, or other gastrointestinal symptoms to suggest inflammatory bowel disease. Routine radiograph of the sacroiliac joints did not reveal arthritic changes but this did not exclude early reactive arthritis, which may be mild and self-limiting.5 Further imaging techniques were not performed. Enthesitis may be associated with Reiter’s syndrome but none was demonstrated in our patient. HLA antigen B27 positivity is strongly associated with Reiter’s syndrome, typically occurring in 70% to 90% of cases,6 but some case series have revealed a significantly lower association with HLA antigen B27, particularly in female patients.1 Our patient tested negative for antigen HLA B27. This is another feature of our patient’s case that distinguishes it from the typical form of Reiter’s syndrome. The positive serology for C trachomatis lends support to the clinical diagnosis of Reiter’s syndrome. The association between C trachomatis and Reiter’s syndrome is well established.6 Just as our case differed clinically from typical Reiter’s syndrome, so the histologic features differed also. The classic histology of skin lesions in Reiter’s syndrome resembles pustular psoriasis. In our case, although vulvar biopsies showed focal psoriasiform hyperplasia and spongiform pustules in the malpighian layer (Fig 4), other areas showed pseudoepitheliomatous hyperplasia and deep epidermal microabscesses (Fig 3). Because pemphigus vegetans was excluded by repeated negative immunofluorescence microscopy, these histologic findings were thought to be most consistent with an infectious process such as blastomycosis, or halogenoderma. However, these histologic diagnoses were not consistent with the clinical findings. In addition, multiple special stains were negative for organisms, and serum halogen levels were normal. It was, therefore, not possible to make a definitive diagnosis on the basis of the histologic features. However, Reiter’s syndrome is a clinical diagnosis. There may be supportive evidence from histology and/or evi-
616 Lotery et al
dence of recent urogenital or enteric infection, but this is not necessary to make the diagnosis. Other investigations that, if positive, may be contributory to a diagnosis of Reiter’s syndrome include urethral culture and polymerase chain reaction for Chlamydia, and cultures and serology for enteric organisms. Serum zinc levels may be useful to rule out acrodermatitis enteropathica as a differential diagnosis of ulcerative oral and genital disease, or to help plan management in a case of Reiter’s syndrome exacerbated by a low serum zinc level. Repeated titers for Chlamydia later in the disease course may have helped to confirm the significance of the positive titers at the time of presentation. Unfortunately, these tests were not performed on our patient. However, it is unlikely that any culture or polymerase chain reaction for Chlamydia or enteric organisms would have been positive, because of the treatment with multiple antibiotics before presentation to our institution and the absence of any gastrointestinal symptoms. We did not ascertain from the patient the length of the various antibiotic courses, but all the antibiotics including azithromycin had been taken as prescribed by a local physician. Lymphogranuloma venereum might have been considered in the initial differential diagnosis of our patient’s vulvar disease, and rectal examination and cultures would then have been indicated. However, adenitis was not a prominent feature. In addition, psoriasiform skin lesions, conjunctivitis, and oral ulceration are not associated with lymphogranuloma venereum. Ideally the patient’s sexual partner should have been investigated for C trachomatis infection but, unfortunately, we had no access to him and we are not aware of any test results from a local physician. We do not have the benefit of long-term follow up information because the patient defaulted from return appointments. This information would be useful because it is recognized that long time periods may elapse between different manifestations of Reiter’s syndrome.3 However, even in the relatively limited period of follow-up, our patient had a constellation of persistent findings that could not be explained by any other disease process, namely ul-
J AM ACAD DERMATOL APRIL 2003
cerative vulvitis, psoriasiform skin lesions on the limbs and soles, stomatitis, conjunctivitis, low back pain and stiffness, weight loss, positive serology for C trachomatis, and high erythrocyte sedimentation rate. The most prominent manifestation of our patient’s disease was on the vulva. We know of only 4 previously reported descriptions of vulvar lesions in Reiter’s syndrome. One of these patients had red, crusted plaques on the vulva associated with vaginal discharge, but no erosive lesions.4 Two reports described circinate erosions on the vulva that resembled the lesions of circinate balanitis in male patients and were given the name “circinate vulvitis.”7 A further report described similar lesions arising in a patient with coincidental lichen sclerosus et atrophicus.8 Our patient had linear ulcers, verrucous lesions, and pustules rather than circinate lesions. Her case highlights the wide variety of clinical features associated with Reiter’s syndrome and emphasizes that female patients do not necessarily have the traditional, typical form of the disease. In addition the histologic features may differ from the typical features of psoriasis. The diagnosis of Reiter’s syndrome is predominantly clinical and should be considered in cases of unusual genital ulceration. REFERENCES 1. Yli-Kerttula UI. Clinical characteristics in male and female Reiter’s syndrome. Clin Rheumatol 1984;3:351-60. 2. Wilkens RF, Arnett FC, Butler T. Reiter’s syndrome, evaluation of preliminary criteria for definite disease. Arthritis Rheum 1981; 24:844-9. 3. Calin A. Keratoderma blennorrhagicum and mucocutaneous manifestations of Reiter’s syndrome. Ann Rheum Dis 1979;38: 68-72. 4. Edwards L, Hansen RC. Reiter’s syndrome of the vulva: the psoriasis spectrum. Arch Dermatol 1992;128:811-4. 5. Csonka GW. Workshop 1: features and prognosis of Reiter’s syndrome. Ann Rheum Dis 1979;38:4-7. 6. Graham RM. Reiter’s disease. In: Champion RH, Burton JL, Burns DA, Breathnach SM, editors. Rook, Wilkinson, Ebling textbook of dermatology. 6th ed. London (UK): Blackwell Science; 1998. p. 2759-71. 7. Thambar IV, Dunlop R, Thin RN, Huskisson EC. Circinate vulvitis in Reiter’s syndrome. Br J Venereal Dis 1977;53:260-2. 8. Daunt SO, Kotowski KE, O’Reilly AP, Richardson AT. Ulcerative vulvitis in Reiter’s syndrome: a case report. Br J Venereal Dis 1982;58:405-7.
REPORTS
Ulcerative vulvitis in atypical Reiter’s syndome Helen E. Lotery, MBBCh,a Rudolph P. Galask, MD, MS,a,b,c Mary Seabury Stone, MD,b,d and Richard D. Sontheimer, MDb Iowa City, Iowa We report a case of atypical Reiter’s syndrome occurring in a female patient who had severe, ulcerative vulvar disease develop in association with conjunctivitis, low back pain, stomatitis, and psoriasiform skin lesions. Vulvar lesions have rarely been described in Reiter’s syndrome and are not well characterized. (J Am Acad Dermatol 2003;48:613-6.)
R
eiter’s syndrome in its classic form consists of a triad of arthritis, urethritis, and conjunctivitis. It is uncommon in females and its presentation may be atypical or incomplete.1
CASE REPORT A 46-year-old woman presented with a 9-month history of painful genital ulceration associated with offensive discharge and bleeding. She had been without medical attention during most of this time as a result of financial hardship. In the weeks before referral to our institution she had been treated with antibiotics (cefixime, azithromycin, cephalexin, and ciprofloxacin) for presumed infection, without success. On further questioning the patient admitted to a 9-month history of painful mouth ulcers; a 9-month history of lower back pain and stiffness which was worse in the morning; a 3-month history of red, sore eyes; and a 3-month history of asymptomatic skin lesions on the limbs. In addition she reported an involuntary weight loss of 30 lb during the 9-month period. She had no urinary symptoms. She had no diarrhea, abdominal pain, or other gastrointestinal symptoms. Medical history included a caesarean hysterectomy at age 24 as a result of chorioamnionitis complicating her fourth pregnancy. In addition she had a history of treatment for alcohol abuse. She had had several sexual partners in the past but no known From the Departments of Obstetrics and Gynecology,a Dermatology,b Microbiology,c and Pathology,d University of Iowa Hospitals and Clinics. Funding sources: None. Conflict of interest: None identified. Reprints not available from authors. Correspondence: Richard D. Sontheimer, MD, Department of Dermatology, University of Iowa Hospitals and Clinics, 200 Hawkins Dr, Iowa City, IA 52242. E-mail: [email protected]. Copyright © 2003 by the American Academy of Dermatology, Inc. 0190-9622/2003/$30.00 ⫹ 0 doi:10.1067/mjd.2003.225
sexually transmitted infections. She had been in her current sexual relationship for 2 years. She had no personal or family history of skin disease or arthropathy. At the time of presentation she was on no routine medication. Since her initial presentation to her family physician she had been given multiple diagnoses suggested by several physicians including syphilis, AIDS, genital warts, candidiasis, herpes simplex, psoriasis, pemphigus vegetans, and vulvar cancer. Examination of the genital area revealed linear ulcerations in the inguinal folds with surrounding verrucous lesions. Adenitis was not a prominent feature. There were widespread pustules, erosions, and erythema affecting the groin, labia majora, labia minora, perineum, and perianal area. The labia majora and minora were edematous and covered with profuse, purulent discharge (Fig 1). Examination of the rest of the skin revealed discrete, scaling, hyperkeratotic lesions on the knees and elbows (Fig 2), and perionychial erythema and crusting affecting several toes. The plantar aspects of both feet were normal at the initial examination but subsequently developed small hyperkeratotic papules. There was superficial ulceration under skin folds of the lower abdomen. Examination of the eyes revealed conjunctival erythema and edema. In the mouth there were multiple small, shallow, circinate ulcers on the buccal mucosa and soft palate. The initial investigations in our institution were performed by the gynecology service and comprised examination under anesthesia followed by 3 vulvar biopsies. There was no evidence of any disease in the vagina. The cervix was absent as a result of previous hysterectomy. The vulvar biopsies all revealed similar findings, namely hyperkeratosis, acanthosis, and neutrophilic microabscesses deep in the epidermis and a dense, mixed inflammatory cell infiltrate in the dermis with focal multinucleated giant cells (Fig 3). There was focal psoriasiform 613
614 Lotery et al
J AM ACAD DERMATOL APRIL 2003
Fig 1. Groin and vulva.
Fig 2. Elbow and forearm.
hyperplasia and the presence of spongiform pustules in the malpighian layer (Fig 4). Direct immunofluorescence microscopy studies were negative. Gram stain and Gomori’s methenamine silver stain were also negative. Further biopsies were then performed by the dermatology and ophthalmology services. Skin biopsy from the elbow revealed hyperkeratosis, parakeratosis, irregular acanthosis, neutrophilic epidermal microabcesses, spongiform pustules, and a mononuclear perivascular infiltrate in the papillary dermis. Gram stain was negative for organisms. Direct immunofluorescence microscopy was negative. Conjunctival biopsies revealed intense acute and chronic inflammation in the stroma. Direct immunofluorescence microscopy and Gram stain were again negative. Weakly positive Chlamydia trachomatis serology was observed (IgG positive 1:16, IgM positive 1:10). IgM was also positive 1:10 for Chlamydia psittaci and Chlamydia pneumoniae, suggesting probable cross-reactivity. Other abnormal findings included
Fig 3. Biopsy specimen of vulva showing hyperkeratosis, acanthosis, neutrophilic microabscesses, and mixed inflammatory cell infiltrate in dermis with focal multinucleated giant cells. (Hematoxylin-eosin stain; original magnification ⫻4.)
Fig 4. Biopsy specimen of vulva showing focal psoriasiform hyperplasia and spongiform pustules. (Hematoxylin-eosin stain; original magnification ⫻10.)
increased platelet count (667,000/mm3), white blood cell count (12.9 k/mm3), erythrocyte sedimentation rate (64 mm/h), and C-reactive protein (5.7
J AM ACAD DERMATOL VOLUME 48, NUMBER 4
mg/dL). The following investigations were normal or negative: routine biochemistry including liver function tests; conjunctival cultures for Chlamydia; vaginal vault cultures for Chlamydia; repeated vulvar cultures for herpes simplex virus and yeast; HIV; rapid plasma reagin; hepatitis serology; radiograph of sacroiliac joints; HLA antigen B27; and serum halogens (bromide and iodide). The patient’s sexual partner was not available for testing for sexually transmitted infection. We made the diagnosis of atypical Reiter’s syndrome on the basis of the constellation of clinical features. This diagnosis was supported by the positive serology for C trachomatis. Histologic features included focal psoriasiform hyperplasia and spongiform pustules consistent with psoriasiform skin lesions (Fig 4) along with other features not typical of psoriasis (Fig 3). The patient was treated with oral acitretin at a dosage of 50 mg daily with subsequent increase to 60 mg daily. In addition she used topical therapy, namely Domeboro’s soaks (aluminum acetate) and triamcinolone 0.1% cream to the abdominal and vulvar lesions; polymyxin B and trimethoprim eye drops; and triamcinolone 0.1% mouthwash. During the following 4 months she improved considerably. The conjunctivitis resolved completely, and the skin lesions decreased in number and size. The vulvar ulcers and verrucous lesions resolved but vulvar erythema, edema, tenderness, and discharge persisted. Unfortunately, at the end of the 4-month period the patient stopped taking acitretin. She was seen again at our dermatology clinic 6 weeks later when all her lesions were recurring and worsening. Acitretin was again prescribed but this time improvement was not as before and 8 months later acitretin was discontinued. We were unsure of the patient’s compliance during this period. Consideration was given to commencement of methotrexate but this was thought to be inadvisable because of the patient’s high alcohol intake. Tacrolimus 0.03% ointment was then initiated for the vulvar and limb involvement with limited benefit. The patient was given tacrolimus 0.1% ointment to use but thereafter did not return to our clinic. Treatment with azathioprine or cyclosporine was also being considered at the time that the patient defaulted.
DISCUSSION Traditionally the classic features of Reiter’s syndrome are urethritis, arthritis, and conjunctivitis occurring in a male patient in response to a urogenital or enteric infection, particularly chlamydial urethritis. Arthritis, in particular, is traditionally a hallmark symptom.2 However, there is considerable variation
Lotery et al 615
in the clinical features of Reiter’s syndrome and it is recognized even in male patients that mucocutaneous manifestations may dominate the initial disease presentation.3 Women constitute a very small proportion of the reported cases and their disease may have an atypical presentation. One case has been described with mucocutaneous features preceding the onset of arthritis by more than 4 years.4 Our patient had erosive vulvar lesions, psoriasiform skin lesions, conjunctivitis, mouth ulcers, and low back pain with stiffness that was worse in the morning, which together pointed to a clinical diagnosis of Reiter’s syndrome. There was no history of diarrhea, abdominal pain, or other gastrointestinal symptoms to suggest inflammatory bowel disease. Routine radiograph of the sacroiliac joints did not reveal arthritic changes but this did not exclude early reactive arthritis, which may be mild and self-limiting.5 Further imaging techniques were not performed. Enthesitis may be associated with Reiter’s syndrome but none was demonstrated in our patient. HLA antigen B27 positivity is strongly associated with Reiter’s syndrome, typically occurring in 70% to 90% of cases,6 but some case series have revealed a significantly lower association with HLA antigen B27, particularly in female patients.1 Our patient tested negative for antigen HLA B27. This is another feature of our patient’s case that distinguishes it from the typical form of Reiter’s syndrome. The positive serology for C trachomatis lends support to the clinical diagnosis of Reiter’s syndrome. The association between C trachomatis and Reiter’s syndrome is well established.6 Just as our case differed clinically from typical Reiter’s syndrome, so the histologic features differed also. The classic histology of skin lesions in Reiter’s syndrome resembles pustular psoriasis. In our case, although vulvar biopsies showed focal psoriasiform hyperplasia and spongiform pustules in the malpighian layer (Fig 4), other areas showed pseudoepitheliomatous hyperplasia and deep epidermal microabscesses (Fig 3). Because pemphigus vegetans was excluded by repeated negative immunofluorescence microscopy, these histologic findings were thought to be most consistent with an infectious process such as blastomycosis, or halogenoderma. However, these histologic diagnoses were not consistent with the clinical findings. In addition, multiple special stains were negative for organisms, and serum halogen levels were normal. It was, therefore, not possible to make a definitive diagnosis on the basis of the histologic features. However, Reiter’s syndrome is a clinical diagnosis. There may be supportive evidence from histology and/or evi-
616 Lotery et al
dence of recent urogenital or enteric infection, but this is not necessary to make the diagnosis. Other investigations that, if positive, may be contributory to a diagnosis of Reiter’s syndrome include urethral culture and polymerase chain reaction for Chlamydia, and cultures and serology for enteric organisms. Serum zinc levels may be useful to rule out acrodermatitis enteropathica as a differential diagnosis of ulcerative oral and genital disease, or to help plan management in a case of Reiter’s syndrome exacerbated by a low serum zinc level. Repeated titers for Chlamydia later in the disease course may have helped to confirm the significance of the positive titers at the time of presentation. Unfortunately, these tests were not performed on our patient. However, it is unlikely that any culture or polymerase chain reaction for Chlamydia or enteric organisms would have been positive, because of the treatment with multiple antibiotics before presentation to our institution and the absence of any gastrointestinal symptoms. We did not ascertain from the patient the length of the various antibiotic courses, but all the antibiotics including azithromycin had been taken as prescribed by a local physician. Lymphogranuloma venereum might have been considered in the initial differential diagnosis of our patient’s vulvar disease, and rectal examination and cultures would then have been indicated. However, adenitis was not a prominent feature. In addition, psoriasiform skin lesions, conjunctivitis, and oral ulceration are not associated with lymphogranuloma venereum. Ideally the patient’s sexual partner should have been investigated for C trachomatis infection but, unfortunately, we had no access to him and we are not aware of any test results from a local physician. We do not have the benefit of long-term follow up information because the patient defaulted from return appointments. This information would be useful because it is recognized that long time periods may elapse between different manifestations of Reiter’s syndrome.3 However, even in the relatively limited period of follow-up, our patient had a constellation of persistent findings that could not be explained by any other disease process, namely ul-
J AM ACAD DERMATOL APRIL 2003
cerative vulvitis, psoriasiform skin lesions on the limbs and soles, stomatitis, conjunctivitis, low back pain and stiffness, weight loss, positive serology for C trachomatis, and high erythrocyte sedimentation rate. The most prominent manifestation of our patient’s disease was on the vulva. We know of only 4 previously reported descriptions of vulvar lesions in Reiter’s syndrome. One of these patients had red, crusted plaques on the vulva associated with vaginal discharge, but no erosive lesions.4 Two reports described circinate erosions on the vulva that resembled the lesions of circinate balanitis in male patients and were given the name “circinate vulvitis.”7 A further report described similar lesions arising in a patient with coincidental lichen sclerosus et atrophicus.8 Our patient had linear ulcers, verrucous lesions, and pustules rather than circinate lesions. Her case highlights the wide variety of clinical features associated with Reiter’s syndrome and emphasizes that female patients do not necessarily have the traditional, typical form of the disease. In addition the histologic features may differ from the typical features of psoriasis. The diagnosis of Reiter’s syndrome is predominantly clinical and should be considered in cases of unusual genital ulceration. REFERENCES 1. Yli-Kerttula UI. Clinical characteristics in male and female Reiter’s syndrome. Clin Rheumatol 1984;3:351-60. 2. Wilkens RF, Arnett FC, Butler T. Reiter’s syndrome, evaluation of preliminary criteria for definite disease. Arthritis Rheum 1981; 24:844-9. 3. Calin A. Keratoderma blennorrhagicum and mucocutaneous manifestations of Reiter’s syndrome. Ann Rheum Dis 1979;38: 68-72. 4. Edwards L, Hansen RC. Reiter’s syndrome of the vulva: the psoriasis spectrum. Arch Dermatol 1992;128:811-4. 5. Csonka GW. Workshop 1: features and prognosis of Reiter’s syndrome. Ann Rheum Dis 1979;38:4-7. 6. Graham RM. Reiter’s disease. In: Champion RH, Burton JL, Burns DA, Breathnach SM, editors. Rook, Wilkinson, Ebling textbook of dermatology. 6th ed. London (UK): Blackwell Science; 1998. p. 2759-71. 7. Thambar IV, Dunlop R, Thin RN, Huskisson EC. Circinate vulvitis in Reiter’s syndrome. Br J Venereal Dis 1977;53:260-2. 8. Daunt SO, Kotowski KE, O’Reilly AP, Richardson AT. Ulcerative vulvitis in Reiter’s syndrome: a case report. Br J Venereal Dis 1982;58:405-7.