Sweet syndrome-like neutrophilic infiltrate as initial presentation of acute myelogenous leukemia.

Sweet syndrome (SS) is a neutrophilic dermatosis that may be associated with malignancies, especially hematological. We describe the case of a 53-year-old woman with a clinical presentation suggestive of SS, accompanied by pancytopenia and a hypercellular marrow with signs of myelodysplasia. The histopathological findings were characterized as an SS-like cutaneous neutrophilic infiltrate with atypical myeloid cells, myeloperoxidase, and BCR-ABL+, which were absent in peripheral blood and bone marrow aspirate. The patient was treated with systemic corticosteroids with resolution of symptoms and relapse 3 months later when we tried drug withdrawal. Eight months later, the patient was admitted to hematology for a mature acute myelogenous leukemia with an FLT3 mutation. The patient successfully underwent medullar allotransplant and is now asymptomatic (5-month follow-up). This case describes a patient with an acute myelogenous leukemia presenting initially with heralding SS-like cutaneous neutrophilic infiltrate with atypical BCR-ABL+ myeloid cells, as a form of aleukemia cutis. Early recognition of this so-called aleukemic leukemia cutis may allow clinicians to intervene earlier, initiating effective treatment.

Secondary bilateral striopallidodentate calcinosis associated with generalized pustular psoriasis (Von Zumbusch).

Bilateral striopallidodentate calcinosis (BSPDC) mentioned in the literature as Fahr's disease (a misnomer), is characterized by symmetrical and bilateral intracerebral calcifications located in the basal ganglia with or without deposits in the dentate nucleus, thalamus, and white matter. This entity is usually asymptomatic but may be manifested by neurological symptoms. Idiopathic BSPDC can occur either as sporadic or autosomal dominant familial forms. Secondary presentations of BSPDC are associated with infections, neoplastic diseases, toxicological or traumatic factors, and metabolic disorders. We describe a case of generalized pustular psoriasis associated with secondary BSPDC owing to pseudohypoparathyroidism. Laboratory tests revealed hypocalcemia, hyperphosphatemia, and a normal serum level of parathormone. The correction of the phosphorus-calcium metabolism disorder produced clinical improvement.

Actinomycetoma of the chest wall attributed to Nocardia nova after reconstructive surgery.

A 29-year-old man, presented with multiple ulcers, nodules, abscesses, fistulae, and atrophic scars, over the right chest wall. Six years prior, the patient had a car accident, which resulted in skin loss of the right arm, shoulder, thoracic wall. In addition, he suffered a supracondylar fracture; orthopedic surgery and skin grafts were required. Material discharging from sinus tracts was obtained for mycological and bacteriological studies. Direct microscopic examination revealed small white grains. Cultures on Sabouraud and Lowenstein-Jensen media isolated orange-white colonies suggestive of Nocardia. PCR assay identified Nocardia nova. Thoracic and right upper limb CT showed signs of chronic osteomyelitis. Treatment with imipenem/cilastatin for 8 weeks, followed by amoxicillin clavulanate for 6 months, resulted in healing of lesions and improvement in the patient's general health. Primary cutaneous nocardiosis remains a diagnostic challenge. Nocardia are soil-borne filamentous gram-positive bacteria. Identification of characteristic granules on examination of discharge smears from discharge or tissue biopsy is essential for diagnosing mycetoma. Because grain discharge is discontinuous, multiple clinical specimens should be submitted for microscopic examination and culture. Sulfonamides have been the mainstay of Nocardia actinomycetoma treatment. However, our patient's strain was resistant to Co-trimoxazole. Therefore, treatment with imipenem followed by amoxicillin clavulanate was favored, with good clinical and analytical response.

Malignancy associated with dermatomyositis - a retrospective single-center study with 33 patients.

BACKGROUND: Dermatomyositis is a multisystemic inflammatory disease that has been associated with neoplastic disease. OBJECTIVES: To assess the clinical/ laboratory data in a series of patients with dermatomyositis (DM) and identify associations with malignancy. MATERIAL AND METHODS: Retrospective study of DM inpatient files between 1965 and 2011. The patients were included if fulfilled at least four of the five Bohan and Peter diagnostic criteria. DM was considered associated with malignancy only if its diagnosis had preceded or followed the DM diagnosis by up to three years. Data was analyzed with a significance level of 5%. RESULTS: Our results have a retrospective nature and represent only a single-center experience. Between 1965 and 2011, 33 patients fulfilled the inclusion criteria, with a median age of 56 years old (s= 14.329) and female to male ratio of 1.36:1. Neoplastic disease was associated to DM in 30% of the patients and men had significantly more cases of associated neoplasia than women (p=0.042 in Fisher´s exact test). In 70% of these patients, malignancy was diagnosed simultaneously or in the first year after DM diagnosis. CONCLUSIONS: In our series, DM was more frequent in women but, when associated with malignancy, it was more frequent in men, in contrast to the female predominance reported in Western series. Malignancy was found in about one third of DM patients and, in terms of target organs, did not differ from the general population. Therefore, as the majority of malignancies were detected at the time of or in the year following DM diagnosis, screening should be performed for the most frequent neoplasms at the time of diagnosis and repeated one year later.

"Half-half" blisters in bullous pemphigoid successfully treated with adjuvant high-dose intravenous immunoglobulin.

Bullous pemphigoid is a rare, autoimmune blistering disease. Its clinical presentation is tense blisters that may arise on normal-appearing or erythematous skin. Bullous pemphigoid refractory to systemic corticosteroids in combination with immunosuppressants such as azathioprine and mycophenolate mofetil may benefit from adjuvant high-dose intravenous immunoglobulin (IVIg). We describe a particular case with an unusual clinical presentation unresponsive to systemic corticosteroids plus azathioprine, in which the addition of high-dose IVIg was successful. The combined therapy of systemic corticosteroids and azathioprine plus high-dose IVIg can be an option in refractory cases due to its efficiency and tolerability.

Erythema nodosum leprosum associated with minocycline.

Erythema nodosum leprosum is defined by the appearance of tender skin nodules, which can be accompanied by fever, joint pain, neuritis, edema, malaise and/or lymphadenopathy. The authors describe the case of a 19-year-old Angolan black woman, resident in Portugal for the last 10 years, diagnosed with Hansen's disease at the age of 12, irregular with follow-up and non-compliant with treatment. She was referred to our clinic with painful nodules and pustules on the upper limbs, diffuse facial infiltration with pustules and fever, after initiating minocycline with the intention of treating acne. Diagnosis of erythema nodosum leprosum was confirmed by the presence of acid-fast bacilli in the skin smear and also in skin biopsy. Minocycline was suspended and the patient was treated with systemic steroids, with prompt clinical improvement. Our case is reported to alert clinicians to this unusual presentation of erythema nodosum leprosum in a patient treated with highly bactericidal drugs that were not intended to treat Hansen's disease.