Neutrophilic dermatosis after azathioprine exposure.

Importance: Azathioprine hypersensitivity syndrome can present clinically and histopathologically like Sweet syndrome. Shared clinical features include fever, constitutional symptoms, prompt response to systemic corticosteroid therapy, neutrophilia, and abrupt onset of erythematous cutaneous lesions. Histologically, both azathioprine hypersensitivity syndrome and Sweet syndrome are rich in neutrophils.

Neuropilin-2 as a useful marker in the differentiation between Spitzoid malignant melanoma and Spitz nevus.

Background: Spitzoid malignant melanoma (SMM) shares many histopathologic features with Spitz nevus (SN). The distinction between SMM and SN remains one of the most difficult diagnostic problems in dermatopathology. Neuropilin-2 (NRP2) is a cytoplasmic/cell surface protein that is a mediator of melanoma-endothelial cell interaction.

Perifollicular fibroma in Birt-Hogg-Dubé syndrome: an association revisited.

Background: Mutation in the folliculin gene in Birt-Hogg-Dubé (BHD) syndrome leads to a spectrum of benign tumors of the hair follicle, classically including both fibrofolliculoma and trichodiscoma. In addition, lesions clinically indistinguishable from fibrofolliculoma/ trichodiscoma may show histopathologic findings of perifollicular fibroma or angiofibroma. Although some consider perifollicular fibroma to be a variant of angiofibroma, the specific histopathologic findings of perifollicular fibroma are uncommon.

Light microscopic hair shaft analysis in ectodermal dysplasia syndromes.

The objective of the study was to catalog hair shaft abnormalities in individuals with ectodermal dysplasia (ED) syndromes using light microscopy and to compare findings with those in unaffected controls. Light microscopy was performed in a nonblinded manner on hair shafts from 65 participants with seven types of ED (hypohidrotic ED, ED-ectrodactyly-cleft lip or palate, ankyloblepharon-ectodermal defects-cleft lip and palate, Clouston syndrome, Goltz syndrome, Schopf-Schulz Passarge syndrome, and oculodentodigital dysplasia) and 41 unaffected controls. Hair donations were collected at the 28th Annual National Family Conference held by the National Foundation for Ectodermal Dysplasia.

Nevi of special sites.

Melanocytic nevi can have a wide range of histologic appearances. Within the spectrum of nevi, there exists a group that presents in certain anatomic locations with histologically worrisome features but nonetheless benign behavior. This group of nevi has been broadly categorized as nevi of special sites.

Nevi of Special Sites.

Melanocytic nevi can have a wide range of histologic appearances. Within the spectrum of nevi, there exists a group that presents in certain anatomic locations with histologically worrisome features but nonetheless benign behavior. This group of nevi has been broadly categorized as nevi of special sites.

Treatment of acne conglobata with modern external beam radiation.

Like other diseases of the follicular occlusion tetrad, acne conglobata can be difficult to treat. We describe the successful use of modern external beam radiation for acne conglobata in the case of a 53-year-old man with long-standing and disfiguring acne conglobata and hidradenitis suppurativa. For patients with severe acne conglobata resistant to more accepted therapies, modern external beam radiation may be a viable alternative on the therapeutic ladder.

Cutaneous blastomycosis: a diagnostic challenge.

Primary cutaneous inoculation blastomycosis occurs less commonly than secondary blastomycosis, in which cutaneous lesions most often originate from a primary pulmonary infection which disseminates through the blood or lymphatics to involve the skin. In secondary cutaneous blastomycosis, the primary pulmonary infection is frequently subclinical at the time cutaneous lesions manifest. Here we report two cases that illustrate the difficulty in distinguishing between primary and secondary cutaneous involvement.

Nevirapine-induced Stevens-Johnson syndrome in a pediatric patient.

Nevirapine is a non-nucleoside reverse transcriptase inhibitor commonly used in human immunodeficiency virus-1 multidrug regimens and associated with life-threatening cutaneous reactions. Here, we report the successful use of intravenous immunoglobulin in a pediatric patient with Stevens-Johnson syndrome and highlight the risk of nevirapine usage in human immunodeficiency virus postexposure prophylaxis.

Primary systemic amyloidosis associated with multiple myeloma: a case report and review of the literature.

Amyloidosis is a broad and complex class of diseases that comprises several etiologies, many manifestations, and a diversity of outcomes. We discuss a patient with primary systemic amyloidosis associated with multiple myeloma that illustrates many of the typical and atypical features of the disease process. Despite more in-depth assessment and accurate classification, survival for patients with primary systemic disease remains poor.