- Email: [email protected]
Overlap syndrome of type Wong variant dermatomyositis and rheumatoid arthritis
DERMATOLOGICA SINICA 32 (2014) 58e61
Contents lists available at SciVerse ScienceDirect
Dermatologica Sinica journal homepage: http://www.derm-sinica.com
CASE REPORT
Overlap syndrome of type Wong variant dermatomyositis and rheumatoid arthritis Yen-Jen Wang 1, Yu-Hung Wu 1, 2, 3, *, Tien-Ling Chen 4 1
Department of Dermatology, Mackay Memorial Hospital, Taipei, Taiwan Mackay Medicine, Nursing and Management College, Taipei, Taiwan 3 Mackay Medical College, New Taipei City, Taiwan 4 Department of Rheumatology, Mackay Memorial Hospital, Taipei, Taiwan 2
a r t i c l e i n f o
a b s t r a c t
Article history: Received: Jul 6, 2012 Revised: Mar 26, 2013 Accepted: Apr 7, 2013
Wong type dermatomyositis describes a small group of patients with dermatomyositis clinically presented with pityriasis rubra pilaris-like eruptions, including diffuse palmoplantar hyperkeratosis and follicular hyperkeratosis. Histopathologic findings include follicular hyperkeratosis and arrector pili myositis. There have been 20 cases reported since Wong’s large series. We report a 56-year-old male with overlap syndrome of Wong type dermatomyositis and rheumatoid arthritis. He also fulfilled the criteria of anti-tRNA synthetase syndrome due to the presence of anti-Jo-1 antibody and clinical features of both rheumatoid arthritis and myositis. No internal malignancy was found after a complete systemic survey and within 1 year follow-up period. There was a good response to oral steroid therapy. Copyright Ó 2013, Taiwanese Dermatological Association. Published by Elsevier Taiwan LLC. All rights reserved.
Keywords: anti-Jo-1 antibody dermatomyositis overlap syndrome pityriasis rubra pilaris rheumatoid arthritis
Introduction
Case report
Dermatomyositis is an idiopathic inflammatory myopathy with cutaneous manifestations. Characteristic findings include Gottron’s papules, heliotrope rash, shawl sign on the nape, shoulders, and upper back, and a “V sign” on the V-shaped region of the neck and upper chest. Other cutaneous findings include nail changes, vasculitis, localized lipoatrophy, panniculitis, bullous lesions, and, rarely, hyperkeratotic skin eruptions.1 Pityriasis rubra pilaris (PRP) describes a group of uncommon, idiopathic, chronic papulosquamous conditions that are characterized by orange-red scaling plaques with follicular plugging, perifollicular erythema, and palmoplantar hyperkeratosis. A PRP-like eruption has been described in patients with dermatomyositis.2 We report a 56-year-old man with dermatomyositis and rheumatoid arthritis who developed skin eruptions clinically resembling PRP with histologic features of both dermatomyositis and PRP.
A 56-year-old male patient was referred from the rheumatology clinic with symptoms of pain in multiple joints, morning stiffness, muscle weakness, and skin rash for several months. The abduction, adduction, flexion, and extension of the shoulders and the hips of the patient were tested, which showed muscle power of 4 for the shoulders and 3e4 for the hips bilaterally, according to the Medical Research Council scale. He had difficulty raising himself from the bed, climbing stairs, and was unable to hold heavy objects as usual. These symptoms were followed by the skin rash 6 months later. Examination revealed diffuse hyperkeratosis on the bilateral soles and palms with orange hue (Figure 1A and B), erythematous scaly patches on the shoulders, back, and knees, puffy fingers, erythematous patches over the metacarpal and proximal interphalangeal joints (Figure 1C), and follicular papules over the bilateral knee joints (Figure 1D). This patient did not have other cutaneous features characteristic of dermatomyositis, such as heliotrope rash, shawl sign, V sign, or periungual telangiectasia. Blood tests showed elevated: antinuclear antibody titer 1:640 (normal range: <1:40); anti-SSA: 509 AU/mL (normal range: <100 AU/mL), anti-SSB: 433 AU/mL (normal range: <100 AU/mL); rheumatoid factor: 436.0 IU/mL (normal range: <20.0 IU/mL), antiJo-1: 147 AU/mL (normal range: <100 AU/mL); serum creatine kinase: 606 IU/L (normal range: 38e397 IU/L); aspartate transaminase: 42 IU/L (normal range: 15e41 IU/L), erythrocyte
Conflicts of interest: The authors declare that they have no financial or non-financial conflicts of interest related to the subject matter or materials discussed in this article. * Corresponding author. Department of Dermatology, Mackay Memorial Hospital, 92, Section 2, Zhongshan North Road, Taipei City 10449, Taiwan. Tel.: þ886 2 25433535; fax: þ886 2 25433642. E-mail addresses: [email protected], [email protected] (Y.-H. Wu).
1027-8117/$ e see front matter Copyright Ó 2013, Taiwanese Dermatological Association. Published by Elsevier Taiwan LLC. All rights reserved. http://dx.doi.org/10.1016/j.dsi.2013.04.002
Y.-J. Wang et al. / Dermatologica Sinica 32 (2014) 58e61
59
Figure 1 Diffuse hyperkeratosis with orange hue on (A) bilateral soles and (B) palms with sharp margins. (C) Scaly reddish patches on dorsum of fingers. (D) Follicular hyperkeratotic papules (arrow) on the knee.
sedimentation rate: 20 mm/hour (normal range: <12); and lactic dehydrogenase: 307 IU/L (normal range: 98e192 IU/L). Anticyclic citrullinated peptides and C-reactive protein were within normal limits, while HLA-B27 was negative. A skin biopsy obtained from the palm revealed an acanthotic epidermis overlying with loosely arranged hyperkeratosis (Figure 2A). There were scattered dyskeratotic corneocytes and parakeratosis in horizontal and vertical arrays (Figure 2B). Vacuolar degeneration in the basal cell layer, lymphocyte exocytosis, and scattered dyskeratotic cells were noted, indicating interface dermatitis (Figure 2C). There were also focal cornoid lamellas demonstrated by the loss of the granular layer with a dell in the epidermis containing a stacked column of horny cells, some retaining their nuclei (Figure 2D). A mild to moderate lymphocytic perivascular infiltrate was present in the upper dermis. The overall pathological features presented an unusual combination of interface dermatitis, PRP, and porokeratosis. A muscle biopsy specimen taken from the left thigh revealed perivascular lymphocytic infiltration and degenerative changes in the muscle tissue. The needle electromyography showed spontaneous activities and low amplitudes, with short duration and polyphasic motor unit action potentials in the right deltoid, vastus medialis, and tibialis anterior muscles, suggestive of myopathic change. A bone scan revealed arthritis involving the bilateral shoulder and wrist joints, carpi, metacarpal, and proximal interphalangeal joints of both hands. Proximal muscle weakness, increased serum muscle enzyme, and typical findings on electromyography and muscle biopsy results in our patient were compatible with dermatomyositis. The unusual clinical and pathological presentations of PRP-like features indicated Wong type dermatomyositis. Furthermore, according to
2010 rheumatoid arthritis (RA) classification criteria,2 the patient had a score of 9/10 (cut off point for definite RA: 6/10), compatible with RA. The patient was diagnosed as having overlap syndrome of dermatomyositis and RA. Due to the positive anti-Jo-1 antibody and clinical features of rheumatoid arthritis and myositis, our patient also fulfilled the criteria of anti-tRNA synthetase syndrome, a wellknown overlap condition. A complete malignancy workup was performed. The chest radiograph showed only increased bilateral lung markings; the upper gastroendoscopy revealed shallow ulcers in the lower-body of the stomach. There was positive occult blood, and therefore the patient had a colonoscopy examination and transrectal polypectomy. A tubular adenoma at the hepatic flexure, and a mixed tubular adenoma and hyperplastic polyp were removed. No carcinoma was found. Nasopharyngoscopy and serum tumor markers including carcinoembryonic antigen, a-fetoprotein, CA19-9, prostate-specific antigen, EpsteineBarr virus-IgA and EpsteineBarr virus early antigen, and nuclear antigen IgA were normal. He was treated with: daily oral prednisolone 30 mg, plaquenil 200 mg, and etoricoxib 60 mg; topical salicylic acid 2.5% ointment twice daily for the palmoplantar lesions and desoximetasone 0.25% ointment twice daily for the body. The skin lesions gradually improved, so the dosage of oral prednisolone was tapered to 10 mg/day. He did not report muscle weakness after the prednisolone being tapered. The laboratory data, including aspartate aminotransferase, lactate dehydrogenase, erythrocyte sedimentation rate, and serum creatine kinase, decreased following the treatment. Serum creatine kinase decreased from 606 IU/L to 66 IU/L. He is now being maintained on this regimen and regularly followed up at the rheumatology and dermatology clinics.
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Y.-J. Wang et al. / Dermatologica Sinica 32 (2014) 58e61
Figure 2 (A) A loose hyperkeratotic stratum corneum and acanthosis. (B) High magnification showed scattered dyskeratotic keratinocytes, parakeratosis in horizontal and vertical arrays and some dyskeratotic cells in the stratum corneum. (C) Interface dermatitis featured by vacuolar degeneration of basal keratinocytes, lymphocyte exocytosis, and scattered dyskeratotic cells. (D) Focal cornoid lamella characterized by loss of the granular layer and dyskeratotic keratinocytes beneath the parakeratotic columns (hematoxylin and eosin, original magnification, A 40, B 400, C 400, D 200).
Discussion Wong type dermatomyositis refers to a small subgroup of patients with dermatomyositis present with clinical features more characteristic of PRP; only a few cases have been reported. This entity was first described by O’Leary in 1953 in a patient with dermatomyositis, erythroderma, and plantar keratoderma.3 Wong subsequently reported 23 patients of Asian descent with dermatomyositis, of whom 11 had clinical lesions resembling PRP.3 These lesions were described as follicular, erythematous, and hyperkeratotic papules located on the backs of the hands, usually arranged in a linear array over the bony prominences.3e5 The relatively high occurrence of the syndrome in South East Asia may be influenced by racial factors. Twenty additional patients have been described since Wong’s series.3e5 The most characteristic features in these patients were hyperkeratotic follicular papules, involving at least the extremities. Other features included generalized erythematous squamous eruptions, and thick keratinization over acral sites.3e5 The common histologic findings in these patients were follicular hyperkeratosis with arrector pili myositis. However, hyperkeratosis unrelated to hair follicles has also been described.5 Although there were no demonstrable follicular hyperkeratosis or arrector pili myositis, due to the biopsy site of the palm in our patient, parakeratosis in the horizontal and vertical arrays suggested features of PRP. The cornoid lamella found on histopathology in our patient is unusual, although it may be an incidental finding and have no clinical significance. However, there was one reported case of Wong type dermatomyositis showing clinical and histologic features highly suggestive of porokeratosis.3 The incidence of PRP and porokeratosis-like lesions occurring together is unknown, because
they are usually asymptomatic and can be easily overlooked on cutaneous examination.3 Our patient developed symptoms of dermatomyositis and rheumatoid arthritis, satisfying the criteria of overlap syndrome: at least two connective tissue diseases occurring at the same or at different times in the same patient.6 An association of polymyositis/ dermatomyositis with RA has long been established.7 Polymyositis/ dermatomyositis can develop at any stage of RA.8 In recent years, genomic studies have shown marked overexpression of type I interferon inducible genes in the peripheral blood of patients with systemic lupus erythematosus, dermatomyositis, polymyositis, multiple sclerosis, rheumatoid arthritis, systemic sclerosis, and Sjögren’s syndrome, indicating that these diseases share activation of a common type I interferon pathway.9 Our patient also fulfilled the criteria of anti-t-RNA synthetase syndrome. This is a well-known overlap syndrome characterized by a specific autoantibody marker that usually has clinical manifestations milder than those observed in patients with any single connective tissue disease included in overlap syndrome.6 Patients who produce anti-Jo-1 antibodies less frequently display the classic manifestations of dermatomyositis. This may be the reason that our patient had fewer characteristic cutaneous features of dermatomyositis. About 60e90% of patients affected with anti-Jo-1 positive anti-t-RNA synthetase syndrome develop symmetric arthritis that can fulfill the 2010 American College of Rheumatology/ European League Against Rheumatism criteria for RA.6 Interstitial lung involvement has been described in 50e85% of patients with antiJo-1 antibodies.10,11 Our patient had arthralgia, arthritis, and myositis; he did not have a fever, Raynaud’s phenomenon, dyspnea, or any other symptoms/signs of interstitial lung disease. Anti-Jo-1
Y.-J. Wang et al. / Dermatologica Sinica 32 (2014) 58e61
antibody has often been associated with other antibodies, including anti-Ro/SSA and anti-La/SSB,12 which were also noted in our patient. In conclusion, this patient developed a rare overlap syndrome of Wong type PRP-like dermatomyositis, rheumatoid arthritis. The clinical and serological features are also consistent with anti-t-RNA synthetase syndrome. The complex of clinical and histological manifestations may confuse physicians, and in this case, the occurrence of multiple connective tissue disorders was not completely understood. Patients who have had overlap features with dermatomyositis should be followed for internal malignancy, as in every case of adult dermatomyositis. References 1. Khan S, Christopher-Stine L. Polymyositis, dermatomyositis, and autoimmune necrotizing myopathy: clinical features. Rheum Dis Clin North Am 2011;37:143e58. 2. Aletaha D, Neogi T, Silman AJ, et al. 2010 rheumatoid arthritis classification criteria: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Ann Rheum Dis 2010;69:1580e8. 3. Lupton JR, Figueroa P, Berberian BJ, Sulica VI. An unusual presentation of dermatomyositis: the type Wong variant revisited. J Am Acad Dermatol 2000;43:908e12.
61
4. Polat M, Lenk N, Ustün H, Oztas¸ P, Artüz F, Alli N. Dermatomyositis with a pityriasis rubra pilaris-like eruption: an uncommon cutaneous manifestation in dermatomyositis. Pediatr Dermatol 2007;24:151e4. 5. Requena L, Grilli R, Soriano L, Escalonilla P, Fariña C, Martin L. Dermatomyositis with a pityriasis rubra pilaris-like eruption: a little-known distinctive cutaneous manifestation of dermatomyositis. Br J Dermatol 1997;136: 768e71. 6. Iaccarino L, Gatto M, Bettio S, et al. Overlap connective tissue disease syndromes. Autoimmun Rev 2013;12:363e73. 7. Martínez-Cordero E, León DE, Ortega LA. Association of polymyositis with rheumatoid arthritis. Rheumatol Int 2001;20:119e23. 8. Nagashima T, Minota S. Dermatomyositis in patients with rheumatoid arthritis during adalimumab therapy. J Rheumatol 2011;38:574. author reply 5. 9. Higgs BW, Liu Z, White B, et al. Patients with systemic lupus erythematosus, myositis, rheumatoid arthritis and scleroderma share activation of a common type I interferon pathway. Ann Rheum Dis 2011;70:2029e36. 10. Dugar M, Cox S, Limaye V, Blumbergs P, Roberts-Thomson PJ. Clinical heterogeneity and prognostic features of South Australian patients with antisynthetase autoantibodies. Intern Med J 2011;41:674e9. 11. Ingegnoli F, Lubatti C, Ingegnoli A, Boracchi P, Zeni S, Meroni PL. Interstitial lung disease outcomes by high-resolution computed tomography (HRCT) in Anti-Jo1 antibody-positive polymyositis patients: a single centre study and review of the literature. Autoimmun Rev 2012;11:335e40. 12. Váncsa A, Gergely L, Ponyi A, et al. Myositis-specific and myositis-associated antibodies in overlap myositis in comparison to primary dermatopolymyositis: relevance for clinical classification: retrospective study of 169 patients. Joint Bone Spine 2010;77:125e30.
Contents lists available at SciVerse ScienceDirect
Dermatologica Sinica journal homepage: http://www.derm-sinica.com
CASE REPORT
Overlap syndrome of type Wong variant dermatomyositis and rheumatoid arthritis Yen-Jen Wang 1, Yu-Hung Wu 1, 2, 3, *, Tien-Ling Chen 4 1
Department of Dermatology, Mackay Memorial Hospital, Taipei, Taiwan Mackay Medicine, Nursing and Management College, Taipei, Taiwan 3 Mackay Medical College, New Taipei City, Taiwan 4 Department of Rheumatology, Mackay Memorial Hospital, Taipei, Taiwan 2
a r t i c l e i n f o
a b s t r a c t
Article history: Received: Jul 6, 2012 Revised: Mar 26, 2013 Accepted: Apr 7, 2013
Wong type dermatomyositis describes a small group of patients with dermatomyositis clinically presented with pityriasis rubra pilaris-like eruptions, including diffuse palmoplantar hyperkeratosis and follicular hyperkeratosis. Histopathologic findings include follicular hyperkeratosis and arrector pili myositis. There have been 20 cases reported since Wong’s large series. We report a 56-year-old male with overlap syndrome of Wong type dermatomyositis and rheumatoid arthritis. He also fulfilled the criteria of anti-tRNA synthetase syndrome due to the presence of anti-Jo-1 antibody and clinical features of both rheumatoid arthritis and myositis. No internal malignancy was found after a complete systemic survey and within 1 year follow-up period. There was a good response to oral steroid therapy. Copyright Ó 2013, Taiwanese Dermatological Association. Published by Elsevier Taiwan LLC. All rights reserved.
Keywords: anti-Jo-1 antibody dermatomyositis overlap syndrome pityriasis rubra pilaris rheumatoid arthritis
Introduction
Case report
Dermatomyositis is an idiopathic inflammatory myopathy with cutaneous manifestations. Characteristic findings include Gottron’s papules, heliotrope rash, shawl sign on the nape, shoulders, and upper back, and a “V sign” on the V-shaped region of the neck and upper chest. Other cutaneous findings include nail changes, vasculitis, localized lipoatrophy, panniculitis, bullous lesions, and, rarely, hyperkeratotic skin eruptions.1 Pityriasis rubra pilaris (PRP) describes a group of uncommon, idiopathic, chronic papulosquamous conditions that are characterized by orange-red scaling plaques with follicular plugging, perifollicular erythema, and palmoplantar hyperkeratosis. A PRP-like eruption has been described in patients with dermatomyositis.2 We report a 56-year-old man with dermatomyositis and rheumatoid arthritis who developed skin eruptions clinically resembling PRP with histologic features of both dermatomyositis and PRP.
A 56-year-old male patient was referred from the rheumatology clinic with symptoms of pain in multiple joints, morning stiffness, muscle weakness, and skin rash for several months. The abduction, adduction, flexion, and extension of the shoulders and the hips of the patient were tested, which showed muscle power of 4 for the shoulders and 3e4 for the hips bilaterally, according to the Medical Research Council scale. He had difficulty raising himself from the bed, climbing stairs, and was unable to hold heavy objects as usual. These symptoms were followed by the skin rash 6 months later. Examination revealed diffuse hyperkeratosis on the bilateral soles and palms with orange hue (Figure 1A and B), erythematous scaly patches on the shoulders, back, and knees, puffy fingers, erythematous patches over the metacarpal and proximal interphalangeal joints (Figure 1C), and follicular papules over the bilateral knee joints (Figure 1D). This patient did not have other cutaneous features characteristic of dermatomyositis, such as heliotrope rash, shawl sign, V sign, or periungual telangiectasia. Blood tests showed elevated: antinuclear antibody titer 1:640 (normal range: <1:40); anti-SSA: 509 AU/mL (normal range: <100 AU/mL), anti-SSB: 433 AU/mL (normal range: <100 AU/mL); rheumatoid factor: 436.0 IU/mL (normal range: <20.0 IU/mL), antiJo-1: 147 AU/mL (normal range: <100 AU/mL); serum creatine kinase: 606 IU/L (normal range: 38e397 IU/L); aspartate transaminase: 42 IU/L (normal range: 15e41 IU/L), erythrocyte
Conflicts of interest: The authors declare that they have no financial or non-financial conflicts of interest related to the subject matter or materials discussed in this article. * Corresponding author. Department of Dermatology, Mackay Memorial Hospital, 92, Section 2, Zhongshan North Road, Taipei City 10449, Taiwan. Tel.: þ886 2 25433535; fax: þ886 2 25433642. E-mail addresses: [email protected], [email protected] (Y.-H. Wu).
1027-8117/$ e see front matter Copyright Ó 2013, Taiwanese Dermatological Association. Published by Elsevier Taiwan LLC. All rights reserved. http://dx.doi.org/10.1016/j.dsi.2013.04.002
Y.-J. Wang et al. / Dermatologica Sinica 32 (2014) 58e61
59
Figure 1 Diffuse hyperkeratosis with orange hue on (A) bilateral soles and (B) palms with sharp margins. (C) Scaly reddish patches on dorsum of fingers. (D) Follicular hyperkeratotic papules (arrow) on the knee.
sedimentation rate: 20 mm/hour (normal range: <12); and lactic dehydrogenase: 307 IU/L (normal range: 98e192 IU/L). Anticyclic citrullinated peptides and C-reactive protein were within normal limits, while HLA-B27 was negative. A skin biopsy obtained from the palm revealed an acanthotic epidermis overlying with loosely arranged hyperkeratosis (Figure 2A). There were scattered dyskeratotic corneocytes and parakeratosis in horizontal and vertical arrays (Figure 2B). Vacuolar degeneration in the basal cell layer, lymphocyte exocytosis, and scattered dyskeratotic cells were noted, indicating interface dermatitis (Figure 2C). There were also focal cornoid lamellas demonstrated by the loss of the granular layer with a dell in the epidermis containing a stacked column of horny cells, some retaining their nuclei (Figure 2D). A mild to moderate lymphocytic perivascular infiltrate was present in the upper dermis. The overall pathological features presented an unusual combination of interface dermatitis, PRP, and porokeratosis. A muscle biopsy specimen taken from the left thigh revealed perivascular lymphocytic infiltration and degenerative changes in the muscle tissue. The needle electromyography showed spontaneous activities and low amplitudes, with short duration and polyphasic motor unit action potentials in the right deltoid, vastus medialis, and tibialis anterior muscles, suggestive of myopathic change. A bone scan revealed arthritis involving the bilateral shoulder and wrist joints, carpi, metacarpal, and proximal interphalangeal joints of both hands. Proximal muscle weakness, increased serum muscle enzyme, and typical findings on electromyography and muscle biopsy results in our patient were compatible with dermatomyositis. The unusual clinical and pathological presentations of PRP-like features indicated Wong type dermatomyositis. Furthermore, according to
2010 rheumatoid arthritis (RA) classification criteria,2 the patient had a score of 9/10 (cut off point for definite RA: 6/10), compatible with RA. The patient was diagnosed as having overlap syndrome of dermatomyositis and RA. Due to the positive anti-Jo-1 antibody and clinical features of rheumatoid arthritis and myositis, our patient also fulfilled the criteria of anti-tRNA synthetase syndrome, a wellknown overlap condition. A complete malignancy workup was performed. The chest radiograph showed only increased bilateral lung markings; the upper gastroendoscopy revealed shallow ulcers in the lower-body of the stomach. There was positive occult blood, and therefore the patient had a colonoscopy examination and transrectal polypectomy. A tubular adenoma at the hepatic flexure, and a mixed tubular adenoma and hyperplastic polyp were removed. No carcinoma was found. Nasopharyngoscopy and serum tumor markers including carcinoembryonic antigen, a-fetoprotein, CA19-9, prostate-specific antigen, EpsteineBarr virus-IgA and EpsteineBarr virus early antigen, and nuclear antigen IgA were normal. He was treated with: daily oral prednisolone 30 mg, plaquenil 200 mg, and etoricoxib 60 mg; topical salicylic acid 2.5% ointment twice daily for the palmoplantar lesions and desoximetasone 0.25% ointment twice daily for the body. The skin lesions gradually improved, so the dosage of oral prednisolone was tapered to 10 mg/day. He did not report muscle weakness after the prednisolone being tapered. The laboratory data, including aspartate aminotransferase, lactate dehydrogenase, erythrocyte sedimentation rate, and serum creatine kinase, decreased following the treatment. Serum creatine kinase decreased from 606 IU/L to 66 IU/L. He is now being maintained on this regimen and regularly followed up at the rheumatology and dermatology clinics.
60
Y.-J. Wang et al. / Dermatologica Sinica 32 (2014) 58e61
Figure 2 (A) A loose hyperkeratotic stratum corneum and acanthosis. (B) High magnification showed scattered dyskeratotic keratinocytes, parakeratosis in horizontal and vertical arrays and some dyskeratotic cells in the stratum corneum. (C) Interface dermatitis featured by vacuolar degeneration of basal keratinocytes, lymphocyte exocytosis, and scattered dyskeratotic cells. (D) Focal cornoid lamella characterized by loss of the granular layer and dyskeratotic keratinocytes beneath the parakeratotic columns (hematoxylin and eosin, original magnification, A 40, B 400, C 400, D 200).
Discussion Wong type dermatomyositis refers to a small subgroup of patients with dermatomyositis present with clinical features more characteristic of PRP; only a few cases have been reported. This entity was first described by O’Leary in 1953 in a patient with dermatomyositis, erythroderma, and plantar keratoderma.3 Wong subsequently reported 23 patients of Asian descent with dermatomyositis, of whom 11 had clinical lesions resembling PRP.3 These lesions were described as follicular, erythematous, and hyperkeratotic papules located on the backs of the hands, usually arranged in a linear array over the bony prominences.3e5 The relatively high occurrence of the syndrome in South East Asia may be influenced by racial factors. Twenty additional patients have been described since Wong’s series.3e5 The most characteristic features in these patients were hyperkeratotic follicular papules, involving at least the extremities. Other features included generalized erythematous squamous eruptions, and thick keratinization over acral sites.3e5 The common histologic findings in these patients were follicular hyperkeratosis with arrector pili myositis. However, hyperkeratosis unrelated to hair follicles has also been described.5 Although there were no demonstrable follicular hyperkeratosis or arrector pili myositis, due to the biopsy site of the palm in our patient, parakeratosis in the horizontal and vertical arrays suggested features of PRP. The cornoid lamella found on histopathology in our patient is unusual, although it may be an incidental finding and have no clinical significance. However, there was one reported case of Wong type dermatomyositis showing clinical and histologic features highly suggestive of porokeratosis.3 The incidence of PRP and porokeratosis-like lesions occurring together is unknown, because
they are usually asymptomatic and can be easily overlooked on cutaneous examination.3 Our patient developed symptoms of dermatomyositis and rheumatoid arthritis, satisfying the criteria of overlap syndrome: at least two connective tissue diseases occurring at the same or at different times in the same patient.6 An association of polymyositis/ dermatomyositis with RA has long been established.7 Polymyositis/ dermatomyositis can develop at any stage of RA.8 In recent years, genomic studies have shown marked overexpression of type I interferon inducible genes in the peripheral blood of patients with systemic lupus erythematosus, dermatomyositis, polymyositis, multiple sclerosis, rheumatoid arthritis, systemic sclerosis, and Sjögren’s syndrome, indicating that these diseases share activation of a common type I interferon pathway.9 Our patient also fulfilled the criteria of anti-t-RNA synthetase syndrome. This is a well-known overlap syndrome characterized by a specific autoantibody marker that usually has clinical manifestations milder than those observed in patients with any single connective tissue disease included in overlap syndrome.6 Patients who produce anti-Jo-1 antibodies less frequently display the classic manifestations of dermatomyositis. This may be the reason that our patient had fewer characteristic cutaneous features of dermatomyositis. About 60e90% of patients affected with anti-Jo-1 positive anti-t-RNA synthetase syndrome develop symmetric arthritis that can fulfill the 2010 American College of Rheumatology/ European League Against Rheumatism criteria for RA.6 Interstitial lung involvement has been described in 50e85% of patients with antiJo-1 antibodies.10,11 Our patient had arthralgia, arthritis, and myositis; he did not have a fever, Raynaud’s phenomenon, dyspnea, or any other symptoms/signs of interstitial lung disease. Anti-Jo-1
Y.-J. Wang et al. / Dermatologica Sinica 32 (2014) 58e61
antibody has often been associated with other antibodies, including anti-Ro/SSA and anti-La/SSB,12 which were also noted in our patient. In conclusion, this patient developed a rare overlap syndrome of Wong type PRP-like dermatomyositis, rheumatoid arthritis. The clinical and serological features are also consistent with anti-t-RNA synthetase syndrome. The complex of clinical and histological manifestations may confuse physicians, and in this case, the occurrence of multiple connective tissue disorders was not completely understood. Patients who have had overlap features with dermatomyositis should be followed for internal malignancy, as in every case of adult dermatomyositis. References 1. Khan S, Christopher-Stine L. Polymyositis, dermatomyositis, and autoimmune necrotizing myopathy: clinical features. Rheum Dis Clin North Am 2011;37:143e58. 2. Aletaha D, Neogi T, Silman AJ, et al. 2010 rheumatoid arthritis classification criteria: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Ann Rheum Dis 2010;69:1580e8. 3. Lupton JR, Figueroa P, Berberian BJ, Sulica VI. An unusual presentation of dermatomyositis: the type Wong variant revisited. J Am Acad Dermatol 2000;43:908e12.
61
4. Polat M, Lenk N, Ustün H, Oztas¸ P, Artüz F, Alli N. Dermatomyositis with a pityriasis rubra pilaris-like eruption: an uncommon cutaneous manifestation in dermatomyositis. Pediatr Dermatol 2007;24:151e4. 5. Requena L, Grilli R, Soriano L, Escalonilla P, Fariña C, Martin L. Dermatomyositis with a pityriasis rubra pilaris-like eruption: a little-known distinctive cutaneous manifestation of dermatomyositis. Br J Dermatol 1997;136: 768e71. 6. Iaccarino L, Gatto M, Bettio S, et al. Overlap connective tissue disease syndromes. Autoimmun Rev 2013;12:363e73. 7. Martínez-Cordero E, León DE, Ortega LA. Association of polymyositis with rheumatoid arthritis. Rheumatol Int 2001;20:119e23. 8. Nagashima T, Minota S. Dermatomyositis in patients with rheumatoid arthritis during adalimumab therapy. J Rheumatol 2011;38:574. author reply 5. 9. Higgs BW, Liu Z, White B, et al. Patients with systemic lupus erythematosus, myositis, rheumatoid arthritis and scleroderma share activation of a common type I interferon pathway. Ann Rheum Dis 2011;70:2029e36. 10. Dugar M, Cox S, Limaye V, Blumbergs P, Roberts-Thomson PJ. Clinical heterogeneity and prognostic features of South Australian patients with antisynthetase autoantibodies. Intern Med J 2011;41:674e9. 11. Ingegnoli F, Lubatti C, Ingegnoli A, Boracchi P, Zeni S, Meroni PL. Interstitial lung disease outcomes by high-resolution computed tomography (HRCT) in Anti-Jo1 antibody-positive polymyositis patients: a single centre study and review of the literature. Autoimmun Rev 2012;11:335e40. 12. Váncsa A, Gergely L, Ponyi A, et al. Myositis-specific and myositis-associated antibodies in overlap myositis in comparison to primary dermatopolymyositis: relevance for clinical classification: retrospective study of 169 patients. Joint Bone Spine 2010;77:125e30.