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38


Sarcoidosis

Martires, Kathryn; Shvartsbeyn, Marianna; Brinster, Nooshin; Ramachandran, Sarika; Franks, Andrew G Jr
We present a 28-year-old man with a one-year history of cutaneous lesions in old scars and tattoos with concomitant subcutaneous nodules and myopathy. A skin biopsy specimen showed cutaneous sarcoidosis. We discuss the multiple aspects of this case, which represent unique presentations of this systemic disease as well as review isomorphic and isotopic responses.
PMID: 26990326
ISSN: 1087-2108
CID: 2051202

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

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

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

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

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

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
PMID: 26990342
ISSN: 1087-2108
CID: 2051352

Mycobacterium avium complex infection

Vedvyas, Chetan; Shvartsbeyn, Marianna; Brinster, Nooshin; Femia, Alisa
We report the case of a 30-year-old woman with a three-year history of papules, nodules, and multiple ulcers of the left lower extremity. A skin biopsy specimen showed dermal fibrosis and patchy granulomatous inflammation in the upper-to-mid dermis that was associated with a perivascular, lymphohistiocytic infiltrate along with granulomas and necrosis in the subcutaneous fat. Tissue culture showed Mycobacterium avium complex infection. Clinical manifestations of cutaneous nontuberculous mycobacterial infections are discussed as well as their potential association with pedicures.
PMID: 26990341
ISSN: 1087-2108
CID: 2051342

Multiple eruptive dermatofibromas

Callahan, Shields; Matires, Kathryn; Shvartsbeyn, Marianna; Brinster, Nooshin
We report a 34 year-old woman with psoriasis, systemic lupus erythematosus (SLE), and recent anti-TNFa therapy, who presented with multiple, eruptive dermatofibromas (MEDF). Although the pathogenesis of MEDF remains unknown, there is substantial evidence that this phenomenon represents an aberrant immune response. Like the more common presentation of solitary dermatofibromas, these lesions are benign, and no treatment is required. However, MEDF is increasingly recognized as a sign of immune dysregulation and an appropriate work-up should be initiated to identify an underlying cause in patients without a known trigger.
PMID: 26990339
ISSN: 1087-2108
CID: 2051322

Extragenital bullous lichen sclerosus

Quatrano, Nicola A; Shvartsbeyn, Marianna; Meehan, Shane A; Pomerantz, Rhonda; Pomeranz, Miriam Keltz
Lichen sclerosus (LS) is a chronic, inflammatory dermatosis that is characterized by pruritic, white, atrophic plaques that classically affect the anogenital region of postmenopausal women. Extragenital involvement also may occur with several reported morphologic variants. Extragenital bullous LS is a rare variant, which presents as flaccid bullae that favor the trunk and proximal aspects of the extremities. The treatment of extragenital bullous LS is similar to that of genital LS. However, extragenital LS is often less responsive and may present a therapeutic challenge. We describe a 65-year-old woman with a two-year history of vulvar and extragenital LS, who developed a bullous eruption within a pre-existing patch of lichen sclerosis on the breast. We review the clinical and histopathologic features of extragenital bullous LS and discuss current treatment options, which include those for recalcitrant cases.
PMID: 26990331
ISSN: 1087-2108
CID: 2051242