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IgA vasculitis (Henoch-Schonlein purpura)
Farhadian, Joshua A; Castilla, Carmen; Shvartsbeyn, Marianna; Meehan, Shane A; Neimann, Andrea; Pomeranz, Miriam Keltz
We report the first case of direct immunoflourescence-proven immunoglobulin A (IgA) vasculitis associated with influenza infection in an adult patient. IgA vasculitis, which was previously known as Henoch-Schonlein purpura, is the most common systemic vasculitis in children but rarely occurs in adults. Disease onset often occurs after upper respiratory tract infections that are caused by adenovirus or enterovirus. The American College of Rheumatology defines IgA vasculitis by the presence of any two of the following four criteria: age = 20 years at disease onset, palpable purpura, acute abdominal pain, and a biopsy specimen that shows granulocytes in the walls of small arterioles or venules. Purpura, abdominal pain, and arthralgia comprise the classic triad. Renal involvement may be severe, especially in adults. Treatment is most often supportive but glucocorticoids and/or immunosuppressive agents are recommended in severe or refractory cases.
PMID: 26990342
ISSN: 1087-2108
CID: 2051352
Lupus miliaris disseminatus faciei
Cymerman, Rachel; Rosenstein, Rachel; Shvartsbeyn, Marianna; Meehan, Shane A; Kornreich, Craig
Lupus miliaris disseminatus faciei (LMDF) is a rare, inflammatory condition, which is characterized by red-brown and yellow-brown papules on the face, with characteristic involvement of the eyelids and with histopathologic findings of suppurative and granulomatous folliculitis and dermatitis. The etiology of this disease is not known, but retinoids, anti-inflammatory, immunosuppressive, and antimicrobial medications are utilized to treat the condition with variable results. We present the case of a patient with LMDF that has thus far been refractory to treatment.
PMID: 26990343
ISSN: 1087-2108
CID: 2051362
Nodular amyloidosis
White, Forrest; Shvartsbeyn, Marianna; Meehan, Shane A; Ramachandran, Sarika
Nodular amyloidosis is the rarest form of primary cutaneous amyloidosis. We report the case of a 74-year-old woman with an eight-year history of asymptomatic, hyperpigmented plaques on the pretibial areas. A skin biopsy specimen showed deposits of amorphous eosinophilic material that extended throughout the dermis with apple-green birefringence with a Congo-red stain, which established a diagnosis of nodular amyloidosis. Patients with nodular amyloidosis should be evaluated for systemic disease and followed appropriately due to a small risk of progression to systemic amyloidosis.
PMID: 26990345
ISSN: 1087-2108
CID: 2051372
Primary diffuse macular amyloidosis
Kanchanapoomi, Melissa; Rosenstein, Rachel; Shvartsbeyn, Marianna; Meehan, Shane A; Dolly, Naomi; Femia, Alisa
We present a 53-year-old woman with diffuse macular amyloidosis. We discuss the clinical manifestations, pathophysiologic mechanisms, and associations of cutaneous macular amyloidosis.
PMID: 26990346
ISSN: 1087-2108
CID: 2051382
Linear atrophoderma of Moulin
Ahearn, Ian M; Gittler, Julia; Shvartsbeyn, Marianna; Meehan, Shane A; Pomeranz, Miriam Keltz
We present a 40-year-old woman with asymptomatic, linear, hyperpigmented atrophic plaques in a Blaschkoid distribution on the right back and right upper extremity that is consistent with a diagnosis of linear atrophoderma of Moulin. Clinical lesions developed with a biphasic pattern in late adolescence and in adulthood. The pathogenesis of this acquired, progressive Blaschkolinear dermatosis may hold insight into the pathogenesis of this rare dermatologic condition, as well as other dermotoses, which include those resulting from post-zygotic genetic mosaicism.
PMID: 26990347
ISSN: 1087-2108
CID: 2051392
Generalized granuloma annulare in a folliculocentric distribution
Rothman, Lisa R; Mir, Adnan; Meehan, Shane A
We present a 69-year-old man with type 2 diabetes mellitus and a five-year history of an eruption of follicular pustules, papules, and nodules, which was identified histopathologically as folliculocentric granuloma annulare (GA). Folliculocentric generalized GA is a rarely reported variant of GA, in which the the palisading histiocytes form focal granulomas in a follicular pattern. In this case, the GA may represent an isotopic phenomenon, with lesions developing in hair follicles that were previously affected by a suppurative folliculitis.
PMID: 26990334
ISSN: 1087-2108
CID: 2051272
Benign familial pemphigus ( Hailey-Hailey disease)
White, Forrest; Shvartsbeyn, Marianna; Meehan, Shane A; Urbanek, Richard W
A 56-year-old man presented with a 15-year history of scaly red plaques on the trunk and axillae. Skin biopsy was consistent with Hailey-Hailey disease. His condition was refractory to multiple therapies, which included topical and oral antibiotics and topical, intralesional, and oral glucocorticoids. Treatment with subcutaneous botulinum toxin type A at the axillae and on the back led to a nearly complete resolution of plaques in those areas. Botulinum toxin type A should be considered in patients with extensive Hailey-Hailey disease who are fail traditional therapies.
PMID: 26990332
ISSN: 1087-2108
CID: 2051252
Angiolymphoid hyperplasia with eosinophilia
Farhadian, Joshua A; Shvartsbeyn, Marianna; Meehan, Shane A; Urbanek, Richard W
Angiolymphoid hyperplasia with eosinophilia is a rare, benign, vascular proliferation that presents as dome-shaped, light-pink-to-red-brown papules or subcutaneous masses that lack distinguishing surface changes. The condition typically presents as a single lesion or multiple lesions that involve contiguous areas. The pathogenesis is poorly understood. Angiolymhpoid hyperplasia with eosinophilia has been associated with antecedent trauma, T-cell proliferation, infection, and hormone imbalance. This report details a case of widespread angiolymphoid hyperplasia with eosinophilia that flared while the patient was pregnant.
PMID: 26990330
ISSN: 1087-2108
CID: 2051232
Acrokeratoelastoidosis
Mu, Euphemia W; Mir, Adnan; Meehan, Shane A; Nguyen, Nathalie
Acrokeratoelastoidosis is a rare palmoplantar keratoderma. We report the case of a 32-year old man with multiple keratotic papules and plaques on the peripheral margins of his hands and feet. Histopathologic evaluation showed compact orthokeratosis; Verhoeff van Gieson stain showed thin and fragmented elastic fibers. The differential diagnosis includes focal acral hyperkeratosis, which has a similar presentation but with normal elastic fibers. Treatment of acrokeratoelastoidosis is often disappointing, with minimal response to topical glucocorticoids, keratolytics, retinoids, cryotherapy, and lasers.
PMID: 26990327
ISSN: 1087-2108
CID: 2051212
Eosinophilic dermatitis of hematologic malignancy
Penn, Lauren; Ahern, Ian; Mir, Adnan; Meehan, Shane A
Eosinophilic dermatosis of hematologic malignancy is a rare, paraneoplastic phenomenon that presents as a pruritic papular or vesicular eruption that is clinically and histopathologically similar to insect bites. We present a 56-year-old man with multiple relapses of diffuse large B cell lymphoma with a typical presentation of pruritic papules and vesicles on the extremities that correlate with a recent relapse of his lymphoma.
PMID: 26990335
ISSN: 1087-2108
CID: 2051282