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Three cases of pustulotic arthro-osteitis associated with episcleritis
Three cases of pustulotic arthro-osteitis associated with episcleritis Shuichi Ikegawa, MD,a Fusazou Urano, MD,b Sadahiro Suzuki, MD,b Noboru Fujisawa, MD, c and Yutaka Nishii, MDd Nagano City, Japan Three cases of pustulotic arthro-osteitis (PAO) associated with episcleritis were described. In each patient, the episcleritis developed more than 10 years after the onset of PAO. These episcleritis were treated with topical corticosteroids. PAO is classified as a member of the seronegative spondylarthritis group of diseases. Though complications of seronegative spondylarthritis include uveitis and episcleritis. PAO associated with episcleritis was not reported. Episcleritis should be considered as a complication of PAO. (J Am Acad Dermatol 1999;41:845-6.)
P
ustulosis palmoplantaris (PPP) is a common disease in which erythematous and scaly plaques with sterile pustules develop on the palms of the hands and soles of the feet. It is sometimes complicated by pain and swelling in the sternocostoclavicular regions, a form of arthritis known as intercostoclavicular ossification (ISSCO).1 In addition, PPP is rarely complicated by sacroiliitis, spondylitis, or peripheral arthritis. The various types of arthritis associated with PPP are referred to as pustulotic arthroosteitis (PAO),2,3 which is now classified as a member of the seronegative spondylarthritis group of diseases that includes ankylosing spondylitis, Reiter’s syndrome, and psoriatic arthritis. PAO has not been associated with HLA-B27 positivity, although the other seronegative spondylarthritides have been.1 Complications of seronegative spondylarthritis include uveitis and episcleritis.4,5 We now describe 3 cases of PAO complicated by episcleritis, in what appears to constitute the first report of eye involvement in PPP.
CASE REPORTS Case 1 In 1985, a 45-year-old woman became aware of erythematous plaques with pustules in her palmoplantar region.
This supplement is made possible through an educational grant from O rtho Dermatological to the American Academy of Dermatolog y. From the Divisions of Dermatolog y,a Rheumatology, Arthritis and Lupus Cente r,b Ophthalmology,c and Internal Medicin e,d Shinonoi General Hospital , Nagano City, Japan. Reprint requests : Shuichi I kegawa, MD, Division of Dermatolog y, Shinonoi Ai 666-1, Shinonoi General Hospital , Nagano City, Nagano-ken, Japan. Copyright © 1999 by the American Academy of Dermatolog y, Inc. 0190-9622/99/$8.00 + 0 16/4/98278
Fig 1. Episcleritis of patient described in Case 2.
Several years later, she began to experience pain and swelling in joints of the anterior chest wall. At age 63, she developed chest and back pain and was admitted to the hospital. The palmoplantar pustulotic erythematous lesions were diagnosed as PPP. Intersternocostoclavicular radiography revealed slight ossification but the vertebrae showed no pathologic abnormalities, and the patient was further diagnosed as having PAO with ISSCO. After 1 month, the palmoplantar eruptions and arthritis pain improved; however, redness and pain subsequently appeared in the left eye and were diagnosed as episcleritis, a condition that recurred periodically thereafter. The episcleritis was treated with topical corticosteroids and improved within a month.
Case 2 In 1975, a 30-year-old man developed erythematous plaques with pustules on his palms and soles. He was diagnosed with PPP and treated. At the age of 44, he became aware of pain and swelling in the joints of the anterior chest wall and lower back. At age 50, he was examined at
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the hospital because of worsening PPP. Radiographic examination revealed severe ossification of the intersternocostoclavicular region and moderate ossification of the lumbar spine, and the condition was diagnosed as PAO. He was subsequently treated with topical corticosteroid ointment and nonsteroidal antiinflammatory drugs. From the age of 54, the patient experienced recurrent unilateral episcleritis (Fig 1), which was treated with topical corticosteroids, and the condition improved within a month.
Case 3 In 1967, erythematous plaques studded with pustules developed on the palms and soles of a 45-year-old man. Several years later, recurrent pain and swelling developed in the joints of his anterior chest wall. On presentation at the hospital at age 66, the patient’s condition was diagnosed as PAO. He underwent repeated improvement and exacerbation of the disease symptoms with the eruptions of PPP often disappearing completely. At age 75, an upper respiratory infection and high fever developed. Solitary pea-sized pustules appeared on his palms and soles, as did walnut-sized indurated erythematous plaques on his trunk and arms. He was admitted to the hospital. Laboratory analysis on admission showed slight renal dysfunction. Although he complained of pain in the anterior chest wall, radiographic examination revealed no pathologic abnormalities. A biopsy specimen of the indurated erythema of the trunk revealed septal panniculitis, and the lesion was diagnosed as erythema nodosum. Further laboratory studies for syphilis and tuberculosis were negative. After admission, redness of the left eye appeared and was diagnosed as episcleritis. The patient was treated with a daily oral dose of prednisolone 30 mg and most of his symptoms disappeared within 2 months. The episcleritis was treated with topical corticosteroids and healed within a month, although it recurred repeatedly thereafter. This patient was thus considered to have experienced PAO complicated by acute generalized pustular bacterid, erythema nodosum, renal dysfunction, and episcleritis.
DISCUSSION We have described 3 cases of PAO associated with episcleritis. In each patient, the episcleritis developed more than 10 years after the onset of PAO. In case 3, acute generalized pustular bacterid was associated with typical PAO and was complicated by erythema nodosum and renal dysfunction. Acute generalized pustular bacterid begins after upper respiratory infection and the eruptions comprise solitary sterile pustules that appear over the entire body,
J AM ACAD DERMATOL NOVEMBER 1999
but mostly on the palms, soles, and lower legs.6 It is also sometimes complicated by erythema nodosum or renal dysfunction. A case similar to case 3 was described by Arikawa et al7; in this patient, a 60-yearold woman, acute generalized pustular bacterid was associated with typical PAO. Generalized pustular bacterid is thought to result from a focal infection, but it can also be considered as an acute form of PPP. PAO is manifest most often as ISSCO and rarely associated with sacroiliitis, spondylitis, and peripheral arthritis.1-3 Thus, PAO has come to be classified as a type of seronegative spondylarthritis negative for rheumatoid factor1; Whereas, seronegative spondylarthritis is often complicated by uveitis or episcleritis.6 PAO complicated by episcleritis or uveitis has not been previously described. Diseases associated with episcleritis include rheumatoid arthritis, collagen disease, such as systemic lupus erythematosus; Wegener’s granulomatosis, ankylosing spondylitis, Reiter’s syndrome, psoriatic arthritis, relapsing polychondritis, erythema nodosum, herpes zoster, tuberculosis, and syphilis.4,5 Idiopathic episcleritis also occurs. It is likely that an immunologic reaction associated with persistent PAO resulted in the delayed onset of episcleritis in our 3 patients. Episcleritis should thus be considered as a complication of PAO. REFERENCES 1. Sonozaki H, Mitsui H, Miyanaga Y, Okitsu K, Igarashi M, Hayashi Y, et al. Clinical features of 53 cases with pustulotic arthroosteitis. Ann Rheum Dis 1981;40:547-53. 2. Bergdahl K, Bjorksten B, Gustavson KH, Linden S, Probst F. Pustulosis palmoplantaris and its relation to chronic recurrent multifocal osteomyelitis. Dermatologica 1979;159:37-45. 3. Jurik AG, Ternowitz T. Frequency of skeletal disease, arthroosteitis, in patients with pustulosis palmoplantaris. Am Acad Dermatol 1988;18:666-71. 4. Burton JL.The skin and eyes. In: Champion RH, Burton JL, Ebling FJG, editors. The textbook of dermatology. Oxford: Blackwell; 1992. p. 2664-5. 5. Watson P. Disease of the sclera and episclera. In: Schwab IR, editor. Clinical ophthalmology. Vol 4. Philadelphia: Lippincott; 1987. P. 1-43. 6. Tan RSH. Acute generalized pustular bacterid. Br J Dermatol 1974;91:209-15. 7. Arikawa J, Murata Y, Higaki Y, Nogita T, Kawashima M. A case of acute generalized pustular bacterid in a patient with pustulotic arthro-osteitis. [In Japanese.] Rionshouhihuka 1995;49:155-7.
P
ustulosis palmoplantaris (PPP) is a common disease in which erythematous and scaly plaques with sterile pustules develop on the palms of the hands and soles of the feet. It is sometimes complicated by pain and swelling in the sternocostoclavicular regions, a form of arthritis known as intercostoclavicular ossification (ISSCO).1 In addition, PPP is rarely complicated by sacroiliitis, spondylitis, or peripheral arthritis. The various types of arthritis associated with PPP are referred to as pustulotic arthroosteitis (PAO),2,3 which is now classified as a member of the seronegative spondylarthritis group of diseases that includes ankylosing spondylitis, Reiter’s syndrome, and psoriatic arthritis. PAO has not been associated with HLA-B27 positivity, although the other seronegative spondylarthritides have been.1 Complications of seronegative spondylarthritis include uveitis and episcleritis.4,5 We now describe 3 cases of PAO complicated by episcleritis, in what appears to constitute the first report of eye involvement in PPP.
CASE REPORTS Case 1 In 1985, a 45-year-old woman became aware of erythematous plaques with pustules in her palmoplantar region.
This supplement is made possible through an educational grant from O rtho Dermatological to the American Academy of Dermatolog y. From the Divisions of Dermatolog y,a Rheumatology, Arthritis and Lupus Cente r,b Ophthalmology,c and Internal Medicin e,d Shinonoi General Hospital , Nagano City, Japan. Reprint requests : Shuichi I kegawa, MD, Division of Dermatolog y, Shinonoi Ai 666-1, Shinonoi General Hospital , Nagano City, Nagano-ken, Japan. Copyright © 1999 by the American Academy of Dermatolog y, Inc. 0190-9622/99/$8.00 + 0 16/4/98278
Fig 1. Episcleritis of patient described in Case 2.
Several years later, she began to experience pain and swelling in joints of the anterior chest wall. At age 63, she developed chest and back pain and was admitted to the hospital. The palmoplantar pustulotic erythematous lesions were diagnosed as PPP. Intersternocostoclavicular radiography revealed slight ossification but the vertebrae showed no pathologic abnormalities, and the patient was further diagnosed as having PAO with ISSCO. After 1 month, the palmoplantar eruptions and arthritis pain improved; however, redness and pain subsequently appeared in the left eye and were diagnosed as episcleritis, a condition that recurred periodically thereafter. The episcleritis was treated with topical corticosteroids and improved within a month.
Case 2 In 1975, a 30-year-old man developed erythematous plaques with pustules on his palms and soles. He was diagnosed with PPP and treated. At the age of 44, he became aware of pain and swelling in the joints of the anterior chest wall and lower back. At age 50, he was examined at
845
846 Ikegawa et al
the hospital because of worsening PPP. Radiographic examination revealed severe ossification of the intersternocostoclavicular region and moderate ossification of the lumbar spine, and the condition was diagnosed as PAO. He was subsequently treated with topical corticosteroid ointment and nonsteroidal antiinflammatory drugs. From the age of 54, the patient experienced recurrent unilateral episcleritis (Fig 1), which was treated with topical corticosteroids, and the condition improved within a month.
Case 3 In 1967, erythematous plaques studded with pustules developed on the palms and soles of a 45-year-old man. Several years later, recurrent pain and swelling developed in the joints of his anterior chest wall. On presentation at the hospital at age 66, the patient’s condition was diagnosed as PAO. He underwent repeated improvement and exacerbation of the disease symptoms with the eruptions of PPP often disappearing completely. At age 75, an upper respiratory infection and high fever developed. Solitary pea-sized pustules appeared on his palms and soles, as did walnut-sized indurated erythematous plaques on his trunk and arms. He was admitted to the hospital. Laboratory analysis on admission showed slight renal dysfunction. Although he complained of pain in the anterior chest wall, radiographic examination revealed no pathologic abnormalities. A biopsy specimen of the indurated erythema of the trunk revealed septal panniculitis, and the lesion was diagnosed as erythema nodosum. Further laboratory studies for syphilis and tuberculosis were negative. After admission, redness of the left eye appeared and was diagnosed as episcleritis. The patient was treated with a daily oral dose of prednisolone 30 mg and most of his symptoms disappeared within 2 months. The episcleritis was treated with topical corticosteroids and healed within a month, although it recurred repeatedly thereafter. This patient was thus considered to have experienced PAO complicated by acute generalized pustular bacterid, erythema nodosum, renal dysfunction, and episcleritis.
DISCUSSION We have described 3 cases of PAO associated with episcleritis. In each patient, the episcleritis developed more than 10 years after the onset of PAO. In case 3, acute generalized pustular bacterid was associated with typical PAO and was complicated by erythema nodosum and renal dysfunction. Acute generalized pustular bacterid begins after upper respiratory infection and the eruptions comprise solitary sterile pustules that appear over the entire body,
J AM ACAD DERMATOL NOVEMBER 1999
but mostly on the palms, soles, and lower legs.6 It is also sometimes complicated by erythema nodosum or renal dysfunction. A case similar to case 3 was described by Arikawa et al7; in this patient, a 60-yearold woman, acute generalized pustular bacterid was associated with typical PAO. Generalized pustular bacterid is thought to result from a focal infection, but it can also be considered as an acute form of PPP. PAO is manifest most often as ISSCO and rarely associated with sacroiliitis, spondylitis, and peripheral arthritis.1-3 Thus, PAO has come to be classified as a type of seronegative spondylarthritis negative for rheumatoid factor1; Whereas, seronegative spondylarthritis is often complicated by uveitis or episcleritis.6 PAO complicated by episcleritis or uveitis has not been previously described. Diseases associated with episcleritis include rheumatoid arthritis, collagen disease, such as systemic lupus erythematosus; Wegener’s granulomatosis, ankylosing spondylitis, Reiter’s syndrome, psoriatic arthritis, relapsing polychondritis, erythema nodosum, herpes zoster, tuberculosis, and syphilis.4,5 Idiopathic episcleritis also occurs. It is likely that an immunologic reaction associated with persistent PAO resulted in the delayed onset of episcleritis in our 3 patients. Episcleritis should thus be considered as a complication of PAO. REFERENCES 1. Sonozaki H, Mitsui H, Miyanaga Y, Okitsu K, Igarashi M, Hayashi Y, et al. Clinical features of 53 cases with pustulotic arthroosteitis. Ann Rheum Dis 1981;40:547-53. 2. Bergdahl K, Bjorksten B, Gustavson KH, Linden S, Probst F. Pustulosis palmoplantaris and its relation to chronic recurrent multifocal osteomyelitis. Dermatologica 1979;159:37-45. 3. Jurik AG, Ternowitz T. Frequency of skeletal disease, arthroosteitis, in patients with pustulosis palmoplantaris. Am Acad Dermatol 1988;18:666-71. 4. Burton JL.The skin and eyes. In: Champion RH, Burton JL, Ebling FJG, editors. The textbook of dermatology. Oxford: Blackwell; 1992. p. 2664-5. 5. Watson P. Disease of the sclera and episclera. In: Schwab IR, editor. Clinical ophthalmology. Vol 4. Philadelphia: Lippincott; 1987. P. 1-43. 6. Tan RSH. Acute generalized pustular bacterid. Br J Dermatol 1974;91:209-15. 7. Arikawa J, Murata Y, Higaki Y, Nogita T, Kawashima M. A case of acute generalized pustular bacterid in a patient with pustulotic arthro-osteitis. [In Japanese.] Rionshouhihuka 1995;49:155-7.