Faculty Bibliography

We present a 21-year-old primigravida woman with a several-week history of pruritic, edematous, targetoid plaques that appeared initially on the abdomen, flanks, and legs and that progressed to involve the inner aspects of the upper arms and lateral aspects of the chest. The histopathologic findings showed perivascular and interstitial dermatitis with eosinophils and vacuolar changes with linear C3 deposition at the basement-membrane zone on direct immunofluorescence study. A diagnosis of pemphigoid gestationis was made. Pemphigoid gestationis is a rare, bullous dermatosis of pregnancy that may be associated with prematurity and small-for-gestational age birth weights. The diagnosis is often made with direct immunofluorescence studies of perilesional skin. Oral glucocorticoids remain the gold standard of therapy in moderate-to-severe cases. The edematous papules and plaques of pemphigoid gestationis may be particularly difficult to distinguish from polymorphic eruption of pregnancy; therefore, immunofluorescence studies are prudent. Prompt recognition and appropriate management may reduce morbidity of this disease, which often recurs with subsequent pregnancies.

A 35-year-old man initially was referred for management of recalcitrant urticaria. Owing to his long history of arthritis and sensorineural hearing loss, genetic testing was performed. The test showed a D305N heterozygous mutation in the NLRP3 gene, which is consistent with the diagnosis of Muckle-Wells syndrome. We discussed the rationales behind the use of the interleukin-1 antagonist anakinra in this autoinflammatory disorder.

A 57-year-old woman with systemic lupus erythematosus and Sjogren syndrome presented with blue-grey hyperpigmentation of the face, upper back, and dorsal aspects of the feet after seven years of therapy with hydroxychloroquine. We present an unusual case of drug-induced hyperpigmentation.

BACKGROUND: One of the most common types of combined melanocytic nevus is that of a blue nevus with ordinary melanocytic nevus. Blue nevi have also been described in association with non-melanocytic cell types, such as those of neural or mesenchymal derivation. Although there are rare descriptions in the literature of blue nevi with myomatous structures, the specific association of combined blue nevi with smooth muscle hyperplasia has not been reported METHODS: We review the clinicopathological features of 12 cases of combined blue nevi with smooth muscle hyperplasia. RESULTS: The majority of these lesions occurred on the back of middle-aged patients and were clinically interpreted as melanocytic nevi or melanoma. Histopathologic examination revealed a combined population of 'common' and blue nevus melanocytes with accompanying smooth muscle hyperplasia. In addition to a lentiginous proliferation of melanocytes at the dermal-epidermal junction with variable basilar hyperpigmentation, there were varying degrees of epidermal acanthosis and follicular induction (three cases). CONCLUSION: We present an unusual hamartoma with features of combined blue nevus and smooth muscle hyperplasia, which has not been previously described.

A 14-year-old boy presented with a 2-month history of recurrent crops of tender, erythematous papules. Cultures for infectious organisms (viral and bacterial) were negative. Biopsies revealed a neutrophil-dense infiltrate with numerous enlarged mononuclear cells reactive for CD30, CD3, and CD4 with a small subset reactive for CD56, TIA1, and GranzymeB. Special stains for fungal and bacterial microorganisms were negative. A diagnosis of neutrophilrich (pyogenic) lymphomatoid papulosis (LyP) was made. Classic LyP is rare in children, and the neutrophil-rich variant has not been described extensively in the literature. In this histopathologic setting, the atypical lymphocytes may be obscured but made more apparent with immunohistochemistry. Cases of neutrophil-rich anaplastic large cell lymphoma have been described and have a similar histologic appearance to our case but are generally in adults and present as solitary tumors on the face. The histopathologic differential diagnosis includes benign processes with CD30+ mononuclear cells in addition to infection processes and neutrophilic dermatoses

Detection of acute kidney injury is undergoing a dynamic revolution of biomarker technology allowing greater, earlier, and more accurate determination of diagnosis, prognosis, and with powerful implication for management. Biomarkers can be broadly considered as any measurable biologic entity or process that allows differentiation between normal function and injury or disease. The ADQI (Acute Dialysis Quality Initiative) had its Ninth Consensus Conference dedicated to synthesis and formulation of the existing literature on biomarkers for the detection of acute kidney injury in a variety of settings. In the papers that accompany this summary, ADQI workgroups fully develop key concepts from a summary of the literature in the domains of early diagnosis, differential diagnosis, prognosis and management, and concurrent physiologic and imaging measures.

Pityriasis rubra pilaris (PRP) is an inflammatory condition of uncertain etiology. We present a case of PRP that demonstrates acantholysis on histopathologic examination, which is a characteristic feature that increasingly is recognized to aid in the diagnosis. We also review the clinical presentations and treatment options for this condition.