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238


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

Squamous-cell carcinoma in situ

Rothman, Lisa R; Mir, Adnan; Meehan, Shane A; Mitchell, Wendy Long
We present a 30-year-old woman with atopic dermatitis and ichthyosis vulgaris and a one-year history of an erythematous, scaly plaque on the dorsal surface of her right hand, which developed three years after an accidental exposure to prolonged ultraviolet C (UVC) radiation in a laboratory accident. The plaque, which was initially treated as eczematous dermatitis, was eventually identified histopathologically as squamous-cell carcinoma in situ. Although causation is not definitive, this case is the first to describe development of non-melanoma skin cancer (NMSC) in an area of skin known to be acutely exposed to UVC radiation. As UVC radiation becomes a more frequently used anti-microbial technology, UVC radiation may become a more commonly identified risk factor in the development of NMSC.
PMID: 26990329
ISSN: 1087-2108
CID: 2051222