Neutrophilic eccrine hidradenitis (NEH) has been described in a variety of clinical settings but is most often seen in leukemic patients receiving chemotherapy. We have recently encountered 6 healthy individuals, of whom 5 were children, who developed NEH localized to the feet. The patients were from 9 to 21 years of age. The presenting complaint was rapid development of tenderness of the feet with varying degrees of morbidity. At clinical evaluation, tender, erythematous papules and nodules were confined to the feet, primarily the plantar surfaces. The major differential diagnoses were erythema nodosum and vasculitis. Laboratory studies were non-contributory. Histologically, the findings were generally similar to those of NEH with certain exceptions, most notably the absence of syringosquamous metaplasia and the presence, in most cases, of neutrophilic abscesses in eccrine coils. Inflammatory and degenerative changes involved primarily the eccrine duct (coiled and dermal), and tended to spare the secretory apparatus. Stains for microorganisms were negative. There were brief recurrences in some of the patients, but those followed over time have remained well. We suggest the term idiopathic plantar hidradenitis for this condition.
Eccrine angiomatous hamartoma (EAH) is a rare, benign cutaneous lesion histologically defined as a proliferation of eccrine glands within a closely associated vascular stroma. Typically EAH presents as a solitary flesh-colored, hyperhidrotic, painful papule or plaque appearing at birth or during childhood. Only two previously reported cases have been described involving multiple, symmetrically located lesions. The occurrence of knuckle pads in patients with EAH has not been reported. We present an instance of multiple painful EAH lesions occurring symmetrically on the extensor wrists in a 14-year-old girl with knuckle pads.
Myocardial sinusoids communicating with the coronary systems occur in pulmonary atresia with intact ventricular septum. To test the hypothesis that the extent of ventriculocoronary connections correlates with the degree of right ventricular outflow obstruction as evidenced by clinical, angiographic and gross anatomic findings, a serial section study of six human autopsy hearts representing a spectrum of hypoplastic right heart was undertaken. Slides were evaluated for the presence and extent of ventriculocoronary connections, associated developmental abnormalities and secondary changes in the ventricular walls. Whereas extensive blind-ended deep sinusoids were a feature of all five cases with unrelieved obstruction, ventriculocoronary connections were identified in three. Changes that suggested ongoing remodeling provide new evidence for the postnatal temporal evolution of these anomalous communications. The regional distribution of myofiber disarray in hypoplastic right heart supports the concept that vascularization parallels myocardial organization in the developing human heart.
Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome characterized by uncontrolled activation and proliferation of proinflammatory cytokines. Initial presentation commonly includes fever, hepatosplenomegaly, and pancytopenia; 6 to 65% of cases also have a concurrent cutaneous eruption. We present the case of a 6-day-old premature infant boy with congenital severe thrombocytopenia, anemia, and hepatosplenomegaly who presented with several cutaneous violaceous papules and nodules and was found to have HLH.
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