Elastin staining patterns in primary cicatricial alopecia.

BACKGROUND: Most biopsy specimens of cicatricial (scarring) alopecia can be readily subclassified as lymphocytic versus neutrophilic, but specific diagnosis remains difficult, particularly when a late stage of the disease is sampled. OBJECTIVE: We sought to document patterns of scarring highlighted by elastic tissue staining in primary cicatricial alopecia. METHODS: We documented Verhoeff elastic van Gieson staining patterns in 58 routinely embedded (vertical) biopsy specimens of cicatricial alopecia. Patterns of fibrosis included perifollicular (wedge-shaped vs broad tree trunk-shaped) and diffuse. The patterns were compared against the diagnosis obtained by independent expert clinical review, including central centrifugal cicatricial alopecia (CCCA), lichen planopilaris, traction alopecia, frontal fibrosing alopecia, discoid lupus erythematosus, and tufted folliculitis. RESULTS: Wedge-shaped perifollicular fibrosis was seen in lichen planopilaris but also in CCCA. Broad tree trunk-shaped perifollicular fibrosis was most commonly encountered in CCCA. LIMITATIONS: The retrospective nature of the study precluded temporal staging of the disease process. CONCLUSIONS: Patterns of fibrosis highlighted by elastin staining in primary cicatricial alopecia appear to be disease specific. Superficial wedge-shaped perifollicular fibrosis is associated with but may not be specific for lichen planopilaris. Broad tree trunk-like perifollicular fibrosis is specific for CCCA but not present in many cases. Elastin staining represents a useful ancillary study for the evaluation of late-stage scarring alopecia in routinely oriented punch biopsy specimens.

Shiitake mushroom-induced flagellate erythema: A striking case and review of the literature.

Ingestion of raw or undercooked shiitake mushrooms is associated with a distinctive flagellate erythema. We describe a 61-year-old Caucasian man who presented with a pruritic, erythematous eruption of multiple linear streaks on the trunk and extremities starting 1 day after eating raw shiitake mushrooms. His symptoms and skin lesions resolved with minimal hyperpigmentation within approximately 1 week after treating with topical steroids and oral antihistamines. Skin biopsy showed non-specific findings, including a sparse perivascular and interstitial dermatitis as well as focal vacuolar interface changes. Our case illustrates that this condition is a visibly striking dermatitis with a self-limited course. The pathomechanism of the skin eruption remains unclear.

Current knowledge in inflammatory dermatopathology.

The complex and fascinating spectrum of inflammatory skin disease, and the comprehension of it, is ever expanding and evolving. During the first decade of the 21st century, numerous advances in the understanding of inflammatory disease mechanisms have occurred, particularly in psoriasis and atopic dermatitis. Continuation of this trend will assure a future in which molecular tests for biomarkers of immediate clinical relevance are used in routine patient care, not only for diagnosis but also for prognosis and management. This article focuses on selected recent or noteworthy developments that are clinically relevant for the histologic diagnosis of inflammatory skin diseases.

Assessment of the 'no eosinophils' rule: are eosinophils truly absent in pityriasis lichenoides, connective tissue disease, and graft-vs.-host disease?

Eosinophils are often present in the inflammatory infiltrate of an interface dermatitis, but the diagnostic specificity of eosinophils in interface dermatitis has not been formally evaluated. We retrospectively identified 97 examples of interface dermatitis with clinically confirmed diagnoses, including lupus erythematosus (LE), lichen planus, pityriasis lichenoides (PL), graft-vs.-host disease (GVHD), dermatomyositis (DM) and drug reaction. Diagnoses were clinically confirmed by at least two dermatologists. Slides were reviewed in a blinded fashion by at least two dermatopathologists. The average eosinophil count per 10 ×200 (×20 objective) fields was lowest for PL (0.2), DM (0.3), GVHD (0.4), and LE (0.5) [defined as Group 1] and was higher for lichen planus, drug reactions, erythema multiforme (major and minor) and viral exanthems [defined as Group 2]. Distinction between Group 1 and Group 2 was maximized using an eosinophil count cutoff of 1.1. In conclusion, eosinophils are usually rare to absent in PL, DM, most forms of LE and GVHD. While final interpretation requires a composite assessment of all features, our results suggest that the presence of even a single eosinophil within nine or ten ×20 fields argues against a diagnosis of PL, DM or LE.

Unilateral calciphylaxis in a patient with systemic lupus erythematosus, chronic kidney disease, and hemodialysis-associated steal syndrome.

Calciphylaxis is a rare but life-threatening disease characterized by deposition of calcium within small and medium sized vessels, with subsequent thrombosis, cutaneous ischemia, and necrosis. Because of its systemic nature, calciphylaxis is typically a symmetrical, bilaterally-distributed phenomenon. Here, we present an unusual case of unilateral leg calciphylaxis in the setting of relative chronic arterial insufficiency of the affected extremity secondary to steal syndrome.

Rare tumors through the looking glass: an examination of malignant cutaneous adnexal tumors.

OBJECTIVE: To identify prognostic factors related to malignant cutaneous adnexal tumors. DESIGN: Population-based study using the Surveillance, Epidemiology, and End Results database from January 1, 1988, through December 31, 2006. SETTING: Seventeen population-based cancer registries. PARTICIPANTS: A total of 4032 patients with malignant cutaneous adnexal tumors. MAIN OUTCOME MEASURES: Overall survival (OS) and disease-specific survival (DSS). RESULTS: Ten-year OS and DSS rates were 54% and 97%, respectively. Unfavorable factors for OS were increasing age (hazard ratio [HR] 1.08; 95% confidence interval [CI], 1.07-1.09; P < .001), distant metastases (3.26; 2.34-4.53; P < .001), no surgical procedure (1.27; 1.01-1.59; P = .04), grade IV tumors (1.97; 1.18-3.28; P = .009), nodal metastases (2.19; 1.40-3.43; P = .001), and T3 tumors (1.37; 1.00-1.87; P = .04). Favorable factors for OS were a wide surgical excision (HR, 0.78; 95% CI, 0.68-0.89; P < .001), female sex (0.73; 0.65-0.82; P < .001), malignant eccrine spiradenoma (0.72; 0.53-0.99; P = .04), and histologic findings of sweat duct carcinoma (0.63; 0.44-0.90; P = .01). Unfavorable factors for DSS included N1 status (HR, 6.77; 95% CI, 2.11-21.68; P < .001), distant metastases (12.24; 6.03-24.85; P < .001), histologic findings of malignant eccrine spiradenoma (5.62; 1.25-25.34; P = .02), and no surgical procedure (2.81; 1.09-7.23; P = .03). Favorable factors for DSS included female sex (HR, 0.52; 95% CI, 0.30-0.91; P = .02). CONCLUSIONS: Five-year survival among patients with malignant cutaneous adnexal tumors is good in the absence of distant metastases. Wide resection may be preferable to less aggressive excision. The prognostic importance of lymph node metastases warrants consideration of lymph node basin staging.

Lupus erythematosus-like imiquimod reaction: a diagnostic pitfall.

Imiquimod (AldaraTM), a Toll-like receptor 7 agonist (TLR7), is known for its unique properties of being an immune response modifier and stimulator. Upon topical application, this TLR7 agonist triggers a cell-mediated immune response predominantly expressed by dendritic cells and monocytes. Local skin irritation at the application site involving erythema, pain, crusting and erosions is common and well documented. On the contrary, the specific histopathologic features associated with these treatment site reactions is not. Herein reported is a case where historical omission of imiquimod use for actinic keratosis complicated the histologic interpretation. We highlight a lupus erythematosus-like microscopic pattern and explore histopathologic features that could help in avoiding a diagnostic pitfall, as well as the relationship between TLR activation, cell-mediated immunity and skin histology.

Subcutaneous intravascular pyogenic granuloma: a case report and review of the literature.

Pyogenic granuloma (PG), also known as lobular capillary hemangioma, is a common vascular proliferation that often occurs after minor injury or infection of the skin. Typically these lesions occur in the superficial dermis; although rare, subcutaneous and intravascular lesions can occur. We present a case of PG with the unusual features of being both a deep subcutaneous and intravascular lesion localized to the forehead without antecedent trauma. We also review the literature on PG and discuss the differential diagnosis.

Pyoderma gangrenosum masquerading as necrotizing fasciitis.

Necrotizing fasciitis is a fulminant advancing soft tissue infection characterized by widespread fascial necrosis, which can result in significant morbidity and even death. This condition requires prompt diagnosis and aggressive management with extensive surgical debridement and appropriate antibiotic coverage. Pyoderma gangrenosum, in contrast, is a noninfectious inflammatory condition of the skin that typically does not require surgical management. Both conditions can present with extensive ulceration and tissue necrosis, and close clinical-pathologic correlation is required to make the appropriate diagnosis. We present a case in which the diagnosis of pyoderma gangrenosum was initially elusive and serves to illuminate the diagnostic pitfalls in dealing with these entities.

Nonbullous neutrophilic dermatosis: Sweet's syndrome, neonatal lupus erythematosus, or both?

We describe a 5-day-old infant who fulfilled the diagnostic criteria for Sweet's syndrome, and the concurrent histologic and autoantibody features supporting the diagnosis of neonatal lupus. To our knowledge, this is the youngest case of Sweet's syndrome reported in the literature. Importantly, our findings further support the hypothesis that lupus erythematosus should be considered in the differential diagnosis of a nonbullous neutrophilic dermatosis, as it may represent the initial manifestation of the disease.

Mycobacterium marinum infection simulating interstitial granuloma annulare: a report of two cases.

We report two cases of Mycobacterium marinum infection that histologically simulated interstitial granuloma annulare (GA). In one case, an infectious etiology was not suspected in histologic sections, but a tissue culture performed during the patient's clinic visit identified M. marinum, and a subsequent Fite stain revealed mycobacteria. Interstitial granulomatous dermatitis is a rare presentation for cutaneous nontuberculous mycobacteria and has yet to be attributed specifically to M. marinum. In both immunocompetent and immunosuppressed patients, infection with M. marinum should be considered in lesions histologically resembling interstitial GA, particularly when there is clinical suspicion for an infectious process.