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Palmoplantar Eruption THE CLINICAL PICTURE SALVADOR ARIAS-SANTIAGO, MD HU SEIN HUSEIN-EL AHMED, MD JO SÉ ANEIROS-FERNÁNDEZ, MD Department of Dermatology, San Cecilio Department of Dermatology, San Cecilio Department of Pathology, San Cecilio University University Hospital, Granada, Spain University Hospital, Granada, Spain Hospital, Granada, Spain MARÍA SIERRA GIRÓN-PRIETO, MD R AMÓN NARANJO-SINTES, PhD Department of Dermatology, San Cecilio University Department of Dermatology, San Cecilio University Hospital, Granada, Spain Hospital, Granada, Spain The Clinical Picture Palmoplantar eruption F 1IGURE F 2IGURE 38-year-old woman presents with re- density in the sternoclavicular joints, and a The key finding A current asymptomatic lesions on the three-phase technetium-99m-labeled bone is noninfectious palms and soles and on the sides of both scan and gallium scan reveal radionuclide feet. The lesions have been developing for 2 uptake in the sternoclavicular and manubri- inflammatory months, unaccompanied by fever or other sys- osternal joints (FIGURE 3). Computed tomog- osteitis in a temic symptoms. raphy of the thorax and abdomen reveal no She has a history of episodes of arthritis of abnormalities. patient with the anterior chest wall, which she has treated skin lesions with nonsteroidal anti-inflammatory drugs Q: Which is the most likely diagnosis? (NSAIDs). □ Pustular psoriasis Physical examination reveals pustules on □ Impetigo contagiosa the palms and soles (FIGURE 1 and F IGURE 2). No □ Syndrome of synovitis, acne, pustulosis, lesions are noted on the oral and genital mu- hyperostosis, osteitis (SAPHO) cosae. □ Dyshidrotic eczema Laboratory tests of C-reactive protein, □ Acute exanthematous pustulosis erythrocyte sedimentation rate, viral serolo- (drug eruption) gies, and antinuclear antibodies are normal. A pustule culture is negative, and a cutaneous A: SAPHO syndrome is the most likely di- biopsy shows parakeratosis and elongation of agnosis. The presence of pustules and aseptic rete ridges, neutrophils migrating from papil- osteitis of the anterior chest wall is compatible lary capillaries to the epidermis, and spongi- with SAPHO syndrome. Treatment with topi- form Kogoj pustule. cal clobetasol propionate (Temovate) for pus- Thoracic radiography shows increased tules and NSAIDs for osteitis brought a good response. doi:10.3949/ccjm.77a.09175 Pustular psoriasis is an uncommon type of CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 77 • NUMBER 10 OCTOBER 2010 729 Downloaded from www.ccjm.org on September 28, 2021. For personal use only. All other uses require permission. PL A MOPLANTAR ERUPTION F IGURE 3. Radionuclide uptake in the sternoclavicular joint (red arrows) and manubriosternal joint (blue arrow) was noted on bone scans. psoriasis characterized by erythema and pus- Chamot et al1 in 1987. The onset is usually tules involving the flexural and anogenital in young adulthood, and is similar in men areas. Cutaneous lesions of psoriasis vulgaris and women. It is characterized by synovitis, may be present before an acute pustular epi- acne, pustulosis, hyperostosis, and osteitis. Of sode. Withdrawal of systemic corticosteroids paramount importance is the finding of a non- Suspect SAPHO in a patient with psoriasis has been reported as infectious inflammatory osteitis in a patient syndrome if a a precipitating factor. with skin lesions. Impetigo contagiosa is a superficial cuta- pustular skin neous infection characterized by an erythema- Clinical findings: disease is tous macule that evolves into a vesicle or Pustules plus rheumatic pain associated with pustule. These lesions are more common in SAPHO syndrome must be suspected when a children. Culture of the fluid usually reveals patient is affected by a pustular skin disease rheumatic pain Staphylococcus aureus or S pyogenes. associated with rheumatic pain. If examina- Dyshidrotic eczema is a pruritic vesicular tion shows that the pain is caused by a sterile eruption of unknown cause on the palm and inflammation of bone or joints, the diagnosis soles (bilateral and symmetric). The typical tends to be confirmed.2 histologic findings are spongiotic and intraepi- Osteoarticular involvement tends to be dermal vesicles. limited to the anterior chest wall. It may Acute exanthematous pustulosis is a include aseptic osteitis, hyperostosis, and drug-induced reaction characterized by con- symmetrical arthritis. Peripheral and axial fluent erythema, blisters, and pustules, mucous osteitis is one of the main characteristics of membrane erosions with fever, and lymphad- the syndrome and is found in around 90% of enopathy. Cultures of the pustules are nega- cases. tive, and biopsy can help confirm the diagno- Cutaneous manifestations are present in sis of drug eruption. two-thirds of patients and consist chiefly of severe acne (acne fulminans, acne conglo- ■ SAPHO SYNDROME bata, and hidradenitis suppurativa), pustular psoriasis, and palmoplantar pustulosis. Neu- SAPHO syndrome is a rare condition of un- trophilic dermatoses associated with this syn- known pathogenesis originally described by drome include Sweet syndrome and pyoderma 730 CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 77 • NUMBER 10 OCTOBER 2010 Downloaded from www.ccjm.org on September 28, 2021. For personal use only. All other uses require permission. ARIAS-SANTIAGO AND COLLEAGUES gangrenosum. Acne lesions are usually seen in ■ REFERENCES men, whereas palmoplantar pustulosis is seen 1. Chamot AM, Benhamou CL, Kahn MF, Beraneck L, 3 Kaplan G, Prost A. Acne-pustulosis-hyperostosis-osteitis in women, often accompanying osteoarticu- syndrome. Results of a national survey. 85 cases [in lar manifestations. French]. Rev Rhum Mal Osteoartic 1987; 54:187–196. 2. Benhamou CL, Chamot AM, Kahn MF. Synovitis-acne- Radiologic findings in the spine are pustulosis hyperostosis-osteomyelitis syndrome (SAPHO). spondylodiskitis, osteosclerosis, sacroiliac A new syndrome among the spondyloarthropathies? joint involvement, and paravertebral ossi- Clin Exp Rheumatol 1988; 6:109–112. fication. In anterior chest wall hyperosto- 3. Hayem G, Bouchaud-Chabot A, Benali K, et al. SAPHO syndrome: a long-term follow-up study of 120 cases. sis, common findings are bone hypertrophy Semin Arthritis Rheum 1999; 29:159–171. and sclerosis with a soft-tissue component. 4. Moll C, Hernández MV, Cañete JD, et al. Ilium osteitis Laboratory test results are uncharacteristic, as the main manifestation of the SAPHO syndrome: response to infliximab therapy and review of the litera- with variable signs of inflammation, and ture. Semin Arthritis Rheum 2008; 37:299–306. the C-reactive protein and sedimentation 5. Olivieri I, Padula A, Palazzi C. Pharmacological manage- rate are usually elevated in the absence of ment of SAPHO syndrome. Expert Opin Investig Drugs 2006; 15:1229–1233. leukocytosis. 6. Arias-Santiago S, Sanchez-Cano D, Callejas-Rubio JL, Fernández-Pugnaire MA, Ortego-Centeno N. Adalimum- Pathogenesis remains elusive ab treatment for SAPHO syndrome. Acta Derm Venereol 2010; 90:301–302. The pathogenesis of this syndrome remains elusive. Since SAPHO syndrome usually ADDRESS: Salvador Arias-Santiago, MD, San Cecilio University Hospital, Av Dr. Olóriz 16, Granada 18012, Spain; e-mail involves the axial skeleton, some investiga- [email protected]. tors have suggested a possible link between the SAPHO syndrome and the seronegative spondyloarthopathies.3 It has also been relat- ed to an infection by Propionibacterium acnes and Corynebacterium species,4 which have been isolated in cultures of bone and skin le- sions. However, the fact that these bacteria are contaminant agents makes their involve- ment in the pathogenesis of this syndrome unlikely. More recently, high concentrations of tumor necrosis factor alpha in bone speci- mens of patients with SAPHO syndrome have been reported, thus highlighting the central role of this cytokine in maintaining inflammation. Treatment is to relieve symptoms Since understanding of the pathogenesis of SAPHO syndrome is limited, a wide range of therapies has been used,5 mostly to relieve symptoms. These include NSAIDs; steroids; antibiotics; bisphosphonates such as pamidro- nate (Aredia) and zoledronic acid (Reclast); and immunosuppressors and immunomodu- lators such as methotrexate (Trexall), leflu- nomide (Arava), sulfasalazine (Azulfidine), cyclosporine (Sandimmune). The results with these therapies have been quite varied. Good response has been reported with tumor necro- sis factor alpha blockers—infliximab (Remi- cade), etanercept (Enbrel), and adalimumab (Humira).6 ■ CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 77 • NUMBER 10 OCTOBER 2010 731 Downloaded from www.ccjm.org on September 28, 2021. For personal use only. All other uses require permission..
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