Arthritis and palmoplantar pustulosis

Pustulosis palmaris et plantaris may be associated with a number of articular diseases. Known to be present in Fiessinger-Leroy-Reiter syndrome and psoriasis arthropatica, this skin disease has been classified by Japanese authors as a new nosological entity: pustular osteo-arthritis. Pustulosis palmaris et plantaris seems to represent a meeting point for axial rheumatisms close to ankylozing spondylitis.     

Acute pseudoseptic arthritis and palmoplantar pustulosis

Summary The case of a 60-year-old woman who developed acute peripheral arthritis of a pseudoseptic character (high synovial leucocytosis and fever) associated to a palmoplantar pustulosis is reported.     

Arthropathy associated with palmoplantar pustulosis

Summary A survey of 170 patients with a diagnosis of palmoplantar pustulosis (PPP) has been undertaken. Detailed family histories of our patients were compiled, dermatological and rheumatological examinations were made. Radiography was performed in patients with clinical suspicions of spinal and/or sacroiliacal, sternoclavicular and sternocostal joint involvement. Active chest-wall symptoms were screened by scintigraphy. Twenty-five patients (16 females, 9 males, with an age range of 32–66, mean 51 years) had some rheumatic complaints. Fifteen of them (60%) had anterior chest-wall involvement, 6 (24%) sacroiliitis (3 of whom also had AS) and 11 (44%) peripheral arthropathy. Six other patients suffered from transient and migratory joint pain. The joint disease was mild in all but 6 cases. Laboratory tests showed increased ESR in 6 patients (24%); only 3 of these patients had elevated CRP. A slightly elevated haptoglobin and/or orosomucoid were found in 12 cases (48%). Elevated IgA was present in 4, IgG in 2 and IgM in 1 patient. Fungal and bacteriological cultures of the skin were negative. A family history of psoriasis was present in 4 patients (16%), of psoriatic arthropathy in 1 patients (4%) and of PPP in 2 patients (8%). Six patients (24%) had concomitant psoriasis. No association between PPP H1a-AC antigens was found. A high incidence of HLA-B27 antigen, present in 8 patients (32%), was documented. A similar association with HLA-B27 has been found in patients with psoriatic arthritis (16). The feeble association with HLA-B13,-17,-37 and CW6 found in psoriasis was not present. The findings show immuno-genetic and clinical similarities between arthropathy associated with PPP and psoriasis and support the view that there is an association between the two diseases.     

Hyperlipidemia and osteoarticular manifestations

Hyperlipoproteinaemia, notably types II and IV, may give rise to various musculoskeletal disorders. Mono-, oligo- or polyarthritis, or even simple arthralgias, are often encountered in patients with severe type IIa hyperlipoproteinaemia, the most satisfactory tentative explanation for this being a microcrystalline pathology. Tendinitis is also frequent, particularly in children. The same manifestations have also been reported, although more occasionally, in type IV hyperlipoproteinaemia. Skeletal lesions, such as xanthoma or lipoma ossificans, are extremely rare. Other musculoskeletal disorders, including gout and aseptic osteonecrosis, are often associated with hyperlipidaemia. Some diseases may induce secondary hyperlipidaemia but have their own rheumatological manifestations. Finally, lipid-lowering drugs, such as fibrates and statines, sometimes induce disabling myalgias.     

Sternocostoclavicular hyperostosis (SCCHO) with palmoplantar pustulosis (PPP)

Three cases of sternocostoclavicular hyperostosis (SCCHO) or pustulotic arthro-osteitis (PAO) associated with plantar pustulosis are presented. Two of them were associated with psoriasis vulgaris although the pustulosis was of a different histological type in each case. Anatomo-pathological study of two of these three cases shows that this hyperostosis is secondary to an inflammatory osteomedulloperiosteal remodelling suggesting an infectious origin which remains to be proven. The relationship of this syndrome with some seronegative spondylarthropathies cannot, however, be excluded.     

A case of primary biliary cirrhosis complicated by Behcet's disease and palmoplantar pustulosis

A 46-year-old woman was diagnosed with palmoplantar pustulosis (PPP) at the Department of Dermatology, Fukushima Medical University Hospital in 2000, and was treated with ointment. However, because liver dysfunction developed in 2003, she was referred to our department, where primary biliary cirrhosis (PBC) was also diagnosed on the basis of clinical findings. One year later, at the age of 49, she developed manifestations of Behget's disease (BD), including erythema nodosum in the lower extremities. Because she had a history of uveitis, recurrent oral ulceration was present, and the HLA typing was positive for B51, BD was additionally diagnosed. Liver function normalized within three months of the start of treatment with ursodesoxycholic acid (UDCA). This is the first case of PBC associated with BD and PPP.     

Chronic osteitis and arthritis of palmoplantar pustulosis. A familial form of B-27 negative spondylarthropathy

A 35-year old woman presented with pustulosis palmaris et plantaris evolving in acute episodes parallel to those of a rheumatic disease consisting of bilateral sacro-iliac arthritis, manubriosternal fusion and sterno-clavicular arthritis. Her brother had spondylitis compatible with the diagnosis of pustular osteo-arthritis though without pustulosis. He presented with bilateral sacro-iliac arthritis, manubriosternal fusion, sterno-clavicular arthritis and an ossified fragment detached from the antero-inferior angle of C6. The two patients had the same HLA phenotype: A1, A9, B5, B41, CW4, DR2, DR4, absence of B27. In a specimen from the sacro-iliac joint the authors found active bone remodelling and inflammatory osteitis with fibrosis and infiltrates rich in mononucleate cells. An electron microscopic study--to the authors' knowledge, the first to be performed in such cases--demonstrated intracytoplasmic filamentous inclusions in two macrophages of the synovial fringe. Only one other case of the familial form has been published so far. Like the rheumatism of acne conglobata, the spondylitis of pustulosis palmaris et plantaris is one of the causes of sterno-costo-clavicular hyperostosis.     

复方甘草酸苷片联合窄谱UVB治疗掌跖脓疱病疗效观察

目的探讨复方甘草酸苷片(美能)联合窄谱中波紫外线(NB-UVB)治疗掌跖脓疱病的临床疗效。方法将66例患者随机分成3组,各22例,治疗组:口服复方甘草酸苷片联合窄谱UVB照射;对照1组:仅口服复方甘草酸苷片;对照2组:窄谱UVB照射,3组均4周为1个疗程,分别于治疗前及治疗后第4周和第8周后观察疗效。结果治疗组、对照1组和对照2组第4周末的治愈率分别为40.91%、18.18%、13.64%,有效率分别为72.73%、40.91%、36.36%,第8周末的治愈率分别为72.73%、31.82%、22.73%,有效率分别为90.91%、63.64%、54.55%,治疗组分别与对照1组和对照2组比较,治疗后4和8周末的疗效和有效率差异均有统计学意义。结论复方甘草酸苷片联合窄谱UVB治疗掌跖脓疱病安全、有效。     

Catamenial pneumothorax and other thoracic manifestations of endometriosis

Thoracic endometriosis syndrome is an uncommon entity with varied clinical manifestations. Its pathogenesis is not understood completely. Recurrent unilateral right-sided pneumothorax that occurs within days of the onset of menstruation is the most common presentation. Clinical suspicion and recognition of the temporal relationship of the patient's symptoms with menses are essential to establish the diagnosis. Radiographic studies, bronchoscopy, and thoracoscopy may support the diagnosis. Pathologic evidence of thoracic endometrial tissue is not present universally. Therapeutic interventions, which include medical and surgical options, must be individualized for each patient.