Generalized Nonspecific Pustular Lesions in Tietze's Syndrome

Tietze’s syndrome (TS) was defined as a nonsuppurative, benign, and self-limited arthropathy characterized by tenderness and painful swelling of one or more sternocostal, costochondral, or sternoclavicular joints in the absence of other possible causes. This clinical entity should not be confused with other chest wall pain in which there is no swelling. This disease can usually be seen in young people. Although the etiopathogenesis is not well known, symptoms generally follow microtraumas and/or respiratory tract infections. It is estimated that somewhere in the vicinity of 20%–25% of noncardiac chest pain has a musculoskeletal basis. Such a symptom generates anxiety in both patients and their medical attendants for fear that this symptom represents a life-threatening event, such as an acute coronary syndrome. Dermatologic comorbidities with TS have been reported previously, such as palmoplantar pustulosis (PPP) and psoriasis vulgaris. The majority of PPP associated with TS has skin eruptions not only confined to the palms and soles but also on the backs of hands and feet, the arms, the legs, and the trunk. Our patient with TS had pustular lesions that clinically resembled pustular psoriasis at a first glance. These should be recognized as a potential part of TS that as in our case may resolve with nonsteroidal anti-inflammatory drug therapy. A 21-year-old man, previously diagnosed with familial Mediterranean fever (FMF), was admitted to our clinic for the diagnosis and treatment of disseminated pustular lesions, especially localized to both legs and trunk, healing with atrophy. Medical history was consistent with disseminated and nonperiodic joint pain, 3 to 4 times per year, localized to the chest since he was 7 to 8 years old along with the truncal dermatologic lesions. Family history was negative for both FMF and TS. It was noticed that there was a localized swelling and an increased sensitivity at both sternoclavicular joints suggestive of TS during the chest wall examination. Dermatologic examination revealed wide macular plaques consisting of small atrophic scarred lesions surrounded with hyperpigmented area, which were found on both legs, especially on the pretibial areas. There were postinflammatory circular atrophic lesions, which were 1 to 2 mm in width and were scattered all over the body, especially on the trunk. Histopathologic examination of those lesions was consistent with subcorneal pustule formation containing polymorphonuclear leukocytes localized inside the epidermal layer, as well as pseudoepitheliomatous hyperplasia areas with granulation formation at the subepithelial area, and perivascular inflammatory infiltrate with neutrophils (perivascular dermatitis) (Fig. 1). Direct immune fluorescent investigation showed no deposit formation. Biochemical examination was normal except for microcytic anemia with an erythrocyte sedimentation rate of 40 mm/h. Serum immunoglobulin and complement levels, thyroid function tests, antithyroid peroxidase, antithyroglobulin, VDRL, antihuman immunodeficiency virus, hepatitis markers (hepatitis B surface antigen, antihepatitis C virus), autoimmune disease markers (antinuclear antibody, antidouble stranded DNA, anti-Sci-70, anticentromere antibody, anti-SSA-B, anti-SM), antistreptolysin O, rheumatoid factor, and tuberculin skin test were all normal. Culture of pustule contents was negative for bacteria. FMF gene (MDFV/pyrine gene) analysis was also negative. Conventional radiographic examination of the thorax was completely normal. However, bone scintigraphy of the whole body demonstrated an increased activity both at the right shoulder and both sternoclavicular joints consistent with arthritis (Fig. 2).