Clinical, Epidemiologic, Histopathologic and Molecular Features of an Unexplained Dermopathy

BACKGROUND: Morgellons is a poorly characterized constellation of symptoms, with the primary manifestations involving the skin. We conducted an investigation of this unexplained dermopathy to characterize the clinical and epidemiologic features and explore potential etiologies. METHODS: A descriptive study was conducted among persons at least 13 years of age and enrolled in Kaiser Permanente Northern California (KPNC) during 2006-2008. A case was defined as the self-reported emergence of fibers or materials from the skin accompanied by skin lesions and/or disturbing skin sensations. We collected detailed epidemiologic data, performed clinical evaluations and geospatial analyses and analyzed materials collected from participants' skin. RESULTS: We identified 115 case-patients. The prevalence was 3.65 (95% CI = 2.98, 4.40) cases per 100,000 enrollees. There was no clustering of cases within the 13-county KPNC catchment area (p = .113). Case-patients had a median age of 52 years (range: 17-93) and were primarily female (77%) and Caucasian (77%). Multi-system complaints were common; 70% reported chronic fatigue and 54% rated their overall health as fair or poor with mean Physical Component Scores and Mental Component Scores of 36.63 (SD = 12.9) and 35.45 (SD = 12.89), respectively. Cognitive deficits were detected in 59% of case-patients and 63% had evidence of clinically significant somatic complaints; 50% had drugs detected in hair samples and 78% reported exposure to solvents. Solar elastosis was the most common histopathologic abnormality (51% of biopsies); skin lesions were most consistent with arthropod bites or chronic excoriations. No parasites or mycobacteria were detected. Most materials collected from participants' skin were composed of cellulose, likely of cotton origin. CONCLUSIONS: This unexplained dermopathy was rare among this population of Northern California residents, but associated with significantly reduced health-related quality of life. No common underlying medical condition or infectious source was identified, similar to more commonly recognized conditions such as delusional infestation

Spectral characteristics of fibers/materials.

<p><b>A.</b> Photograph of formalin-fixed material with the IR spectral characteristics of cellulose consistent with a cotton fiber. <b>B.</b> Upper panel IR spectrum obtained from unidentified fiber, lower panel spectrum is a cellulose reference.</p

Summary of Case Finding and Study Enrollment Efforts, Unexplained Dermopathy, Calilfornia.

<p>Summary of Case Finding and Study Enrollment Efforts, Unexplained Dermopathy, Calilfornia.</p

Representative skin lesions detected on clinical examination.

<p><b>A.</b> Three erythematous scaly plaques with a fourth more proximal eroded and crusted plaque. <b>B.</b> Close-up of the eroded plaque in image 4A showing blue fibers. <b>C.</b> Excoriated erythematous papules suggestive of arthropod bites, dermatitis or possible excoriated folliculitis. <b>D.</b> Close-up of excoriated lesion in image 4C.</p

Superficial infectious processes identified in impetiginous skin lesions of case patients.

<p><b>A.</b> Superficial and deep perivascular dermatitis with epidermal hyperplasia and prominent scale-crust. A heavy growth of <i>Stenotrophomonas maltophilia</i> was obtained in culture of this site. <b>B.</b> Ulcerated skin with purulent exudates and serum-crust containing numerous colonies of coccoid bacteria (<b>C</b>) that stain intensely by using an immunohistochemical technique for <i>Streptococcus pyogenes </i><b>D</b> and <b>E</b>. Purulent serum-crust from an impetiginous lesion, with abundant colonies of gram-positive coccoid bacteria (<b>F</b>). A heavy growth of <i>Staphylococcus aureus</i> was obtained in culture of this site. Hematoxylin and eosin stain (A, B, C, F), immunoalkaline phosphatase with naphthol fast-red and hematoxylin counterstain (D), and Lillie-Twort stain (F). Original magnifications ×12.5 (A), ×25 (B), ×50 (C), ×100 (D, E), and ×158 (F).</p

Prevalence of Fair or Poor Self-rated Health Among Case-patients Completing Web-based Survey, Unexplained Dermopathy, California.

<p>*Data are from the 1993–2007 Behavioral Risk Factor Surveillance System (BRFSS). All respondents to the BRFSS are non-institutionalized residents, 18 years old or older.</p>+<p>Excludes participants <18 yrs of age.</p><p>**One sided exact 95% confidence interval.</p

Age-and Sex-specific Prevalence Rates of Unexplained Dermopathy, California, July 2006–June 2008.

<p>*rate per 100,000 enrollees.</p><p>**excludes 11 cases not identified by electronic record.</p

Representative histopathologic features of case-patient skin lesions.

<p><b>A.</b> Epidermal hyperplasia with compact orthokeratosis and hypergranulosis and perivascular inflammatory infiltrates in the dermis consistent with lichen simplex chronicus. <b>B.</b> Focal erosion with superficial ulceration and scale-crust consistent with excoriation. <b>C.</b> Mixed perivascular inflammatory cell infiltrates comprised of lymphocytes, neutrophils and eosinophils, suggestive of arthropod bite or drug reaction. <b>D.</b> Suppurative folliculitis comprised of eosinophils and neutrophils. Hematoxylin and eosin stain, original magnifications ×25 (A, B), ×100 (C), and ×50 (D).</p

Distribution of Case-patients by Self-reported year of Onset, California.

<p>Distribution of Case-patients by Self-reported year of Onset, California.</p