The diseases which present with cutaneous sclerodermatous changes are scleroderma, eosinophilic fasciitis, mixed connective tissue disease, sclerederma adultorum, scleromyxedema and cutaneous midline mucinosis. This paper reviews the characteristics and differential diagnosis among of the above mentioned diseases.
Diagnosis, Differential ; Fasciitis/diagnosis ; Human ; Myxedema/diagnosis ; Scleredema Adultorum/diagnosis ; Scleroderma, Circumscribed/diagnosis* ; Scleroderma, Systemic/diagnosis

OBJECTIVE: To describe the clinical and ultrasonographic (US) findings in patients with scleredema adultorum of Buschke, who presented with sclerotic skin on their posterior neck. MATERIALS AND METHODS: After obtaining IRB approval, eight patients with scleredema adultorum of Buschke were enrolled. They underwent US examination of their posterior neck. The diagnoses were confirmed pathologically. The clinical history and US images were evaluated retrospectively. Dermal thickness was compared between the patient group and the age- and sex-matched control group. RESULTS: The patients included seven males and one female with a mean age of 51.5 years. All patients presented with thickening of the skin and/or a palpable mass on the posterior neck. Five (62.5%) of the eight patients showed erythematous discoloration. Six patients (75.0%) had a history of diabetes. The Hemoglobin A1c level was found to be increased in all patients. US images did not show any evidence of a soft tissue mass or infection. The mean dermal thickness in patients (7.01 ± 1.95 mm) was significantly greater than that in the control group (3.08 ± 0.87 mm) (p = 0.001). Multiple strong echogenic spots in the dermis were seen in all patients. Seven patients (87.5%) showed posterior shadowing in the lower dermis. CONCLUSION: When a patient with a history of diabetes presents with a palpable mass or erythematous discoloration of the posterior neck and US shows the following imaging features: 1) no evidence of a soft tissue mass or infection, 2) thickening of the dermis, 3) multiple strong echogenic spots and/or posterior shadowing in the dermis, scleredema adultorum of Buschke should be considered in the differential diagnosis.
Dermis ; Diagnosis ; Diagnosis, Differential ; Ethics Committees, Research ; Female ; Humans ; Male ; Neck* ; Retrospective Studies ; Scleredema Adultorum* ; Shadowing (Histology) ; Skin ; Ultrasonography

Scleredema is a rare cutaneous mucinosis characterized by chronic diffuse induration of the skin, and it is occasionally associated with a monoclonal gammopathy (MG). Ankylosing spondylitis (AS) is noted to be another, chronic systemic inflammatory disorder of the axial skeleton that may accompany the MG. However, patients with scleredema and AS accompanied with a MG have not been reported in the literature. We here report a 40-yr-old man with scleredema and advanced AS accompanied with a MG of IgA-kappa protein. Widespread, long-standing scleredema has been developed over 10 yrs after the initial manifestation of AS. It is uncertain whether the coexistence of scleredema and AS is more than coincidental.
Adult ; Collagen/metabolism ; Human ; Immunoglobulins, kappa-Chain/chemistry ; Inflammation ; Lumbar Vertebrae/radiography ; Male ; Mucins/metabolism ; Paraproteinemias/*complications/diagnosis ; Scleredema Adultorum/*complications/diagnosis ; Skin/pathology ; Spondylitis, Ankylosing/*complications/diagnosis

OBJECTIVETo investigate the clinical and pathologic characteristics of patients with scleredema.

METHODThe clinical and pathologic data of 67 outpatients with scleredema who were admitted to PUMC Hospital from 1982 to 2008 were retrospectively analyzed.

RESULTSNeck and upper back lesions were detected in all patients, but no hand or foot involvement was noted. Among 54 patients who received serum immunoglobulin examination, 19 (35.2%) showed abnormal serum immunoglobulin results. Among 67 patients, 22 (32.8%) had concomitant diabetes mellitus. Alcian blue staining was performed in 35 patients, among whom 23 (65.7%) had positive results and 12 (34.3%) had negative results.

CONCLUSIONSScleredema may have systemic involvements in addition to skin lesions. Patients with scleredema also tends to have concomitant diabetes mellitus. Alcian blue staining is not sufficient to differentiate scleredema and scleroderma.

Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Female ; Humans ; Immunoglobulins ; blood ; Male ; Neck ; pathology ; Retrospective Studies ; Scleredema Adultorum ; complications ; diagnosis ; pathology ; Skin ; pathology ; Young Adult