NSF may present with a spectrum of clinical abnormalities, and exhibit overlapping histopathological features resembling cicatrix and other dermal reparative/regenerative processes. NSF may in fact to be a disorder of aberrant extracellular matrix remodeling in patients with renal insufficiency.
A 15- year-old Caucasian girl presented with a 1-week history of necrotic ulcers on her bilateral lower extremities, associated with fever and malaise. She had had similar ulcerations on and off over the past 3 years. She had a medical history of Graves' disease, diagnosed in 1999, for which she was taking methimazole (MMI). On physical examination, there were multiple necrotic ulcers with elevated, inflamed borders and an overlying eschar on the bilateral distal extremities (Figure 1). There was one hemorrhagic, flaccid bulla on the lateral aspect of the ankle. Her complete blood count, comprehensive metabolic panel, prothrombin time, and international normalized ratio were within normal limits. Histopathology revealed a neutrophilic vasculitis involving small as well as medium-sized blood vessels. The overlying epidermis and upper dermis showed necrosis (Figure 2). Antinuclear antibody (ANA) was positive at 1:160. Perinuclear (p-) antineutrophil cytoplasmic antibody (ANCA) was positive at 1:320. Cytoplasmic ANCA was negative. The patient did not meet any clinical criteria for a diagnosis of lupus erythematosus. Direct immunofluorescence revealed IgM, complement C3, and fibrin in small- and medium-caliber blood vessels in the dermis, consistent with a leukocytoclastic vasculitis of small- and medium-caliber blood vessels (Figure 3). MMI was stopped and the patient was given prednisone, tapered over 5 days. Her thyroid function remained stable, and she did not develop new necrotic ulcers. One month later, the patient developed urticarial lesions on her lower extremities. Histopathology revealed a lymphocytic vascular reaction. No eosinophils or neutrophils were seen. These newer urticarial lesions resolved. The patient underwent debridement of the thick, necrotic ulcerative eschars. To date, her lesions have resolved (Figure 4). An ANA drawn 5 months after withdrawal of MMI was negative, and p-ANCA was 1:67.
A 37-year-old African American female with a diagnosis of acute myelogenous leukemia (AML) being treated with chemotherapy presented with a lesion on her lower back within the confines of a newly inked tattoo. Five days after tattoo placement, she developed an oozing, indurated, necrotic plaque at the site. Four days later, she developed chills, fever, and neutropenia. A skin biopsy was performed and was consistent with pyoderma gangrenosum (PG) or neutrophilic dermatoses. PG is an inflammatory skin disease associated with both cutaneous trauma and systemic disease, including hematologic malignancy. PG after tattoo placement, in both healthy patients and those with hematologic malignancies, has, to our knowledge, not yet been described in the literature. While further studies are necessary to investigate the link between PG and tattooing, oncologists may wish to counsel patients with leukemia to refrain from obtaining new tattoos.
This is a case report of a 63-year-old man post renal transplant who developed eruptive keloids, many of which developed with no history of previous trauma. Reports of spontaneous keloids are rare in the literature, and given his history of renal failure, we have considered a connection with nephrogenic systemic fibrosis (NSF). With no history of prior keloid formation, the patient developed a large number of lesions with a wide distribution, which then subsequently healed without keloid formation. Additionally, the lesions stained positive for CD68 and factor XIIIa, both of which are correlated with the lesions of NSF. However, the patient did not present with the classic clinical presentation of NSF, had no history of exposure to gadolinium, and staining for CD34 was negative. In conclusion, NSF is a relatively new disease, the details of which have yet to be clearly established. This case may represent a variant of NSF.
Sweet syndrome (acute f ebrile neutrophilic dermatosis) is a disorder of unknown etiology. It has been associated withautoimmuneprocesses, malignancies, infections, drug reactions, and gastrointestinal disorders suchas inflammatory bowel disease. Wedescribe the case of a 51-yearold man who presented with severe pain in his tongue and throatand referred pain in his rightear, alongwith odynophagia.fever; and hoarsenessof 48 hours'duration. An oral and oropharyngeal examination revealed the presence of aphthous ulcerations, as wellas a 3 x 3-cm raised inflammatory lesion on the right anterior lateral tongue and a 5 x 5-mm bulla on the hard palate in the . midline. In addition, erythematous papules and macules were notedon hisface, neck, and extremities. Cultures, a biopsy, and laboratory tests yielded a diagnosis of Sweet syndrome. The patient was prescribed oral prednisone, and his signs and symptoms resolved within 2 months. AlthoughSweetsyndrome is uncommon, even in dermatology practice, itsheadand neckand oral manifestations and its association with paraneoplastic disease warrant theneedfor otolaryngologists tobeaware ofthecondition.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.