BACKGROUND: Scleredema adultorum of Buschke is a rare disease characterized by firm and non-pitting edema of the skin. The condition is rare with unknown etiology. Diagnosis is made on the basis of clinical findings and skin biopsy.
METHODS: Here, we describe a 14-year-old Iranian girl presenting with non-pitting edema and woody thickening of the skin that progressed within a month. She was evaluated for possible underlying malignancy or connective tissue disorders, which were excluded by multiple laboratory workups. She underwent a skin biopsy which confirmed the diagnosis of scleredema, and she was successfully treated with intravenous immunoglobulin and mycophenolate mofetil.
CONCLUSIONS: While scleredema adultorum of Buschke is a rare disease with no definite treatment, our effort through this report was to highlight the possible benefits of treatment by intravenous immunoglobulin and mycophenolate mofetil.

BACKGROUND: IgG4-related diseases are characterized by marked infiltration and IgG4-positive plasma cells and fibrosis, and involve multiple organs. However, IgG4-related mastitis is rare. We report a case of mastitis associated with IgG4-related disease.
METHODS: A 78-year-old woman visited our hospital with a complaint of multiple subcutaneous nodules. A biopsy of a dorsal subcutaneous mass was performed but did not yield a definitive diagnosis. However, blood tests showed a high level of IgG4, thus she was referred to the department of collagen disease for further examination. Computed tomography (CT) showed a thickening of the skin of the right breast, and the patient was referred to our department. On physical examination, a large area of thickened skin was observed in the right breast without inflammatory breast cancer-like redness, and no mass was palpable. A needle biopsy was performed on an indistinct hypoechoic area in the breast, and she was diagnosed with mastitis associated with IgG4-related disease. Systemic steroid therapy was then administered and the symptoms of multiple skin nodules and mastitis improved.
CONCLUSIONS: We reached the diagnosis based on a biopsy of the mammary gland enabling the patient to begin treatment for IgG4-related disease. This case was characterized by breast skin thickening, which is different from inflammatory breast cancer.

Scleredema is a rare cutaneous mucinosis characterized by diffuse swelling and non-pitting induration. A 63-year-old man reported a 5-year history of skin thickening of the trunk and a 3-week history of dyspnea. Echocardiography revealed diffuse hypokinesis. Skin biopsies obtained from the waist showed thickened dermis with mucin. Myocardial biopsies showed alcian blue-stained tissue between the muscle fibers. The patient was referred to a dermatologist for phototherapy. Cardiomyopathy should be considered in patients with scleredema. Scleredema usually has a good prognosis; however, the mortality risk could be high when accompanied by cardiomyopathy.

Pachydermoperiostosis is a rare condition representing a primary form of hypertrophic osteoarthropathy. It presents in different stages. Patients often overlook early symptoms, because they are benign. The most common manifestations are clubbing of the fingers and toes, skin thickening with characteristic folds on the face and head and widening of joints accompanied by radiological changes. Surgical treatment is not often needed, and, consequently, there are no strict guidelines on surgical management, which is mainly based on case report ana-lysis. This paper presents a case of surgical management of pachydermoperiostosis.