Eosinophilic fasciitis in a young male auto mechanic exposed to organic solvents.

We report a case of eosinophilic fasciitis in a teenage auto mechanic who was most likely affected by occupational exposure to organic solvents, including the aromatic hydrocarbons benzene, trimethylbenzene, naphthalene, toluene, and xylene. The patient presented with an 8-month history of painful induration of his extremities and an abnormal gait. A deep excisional biopsy of the fascia was obtained, demonstrating subcutaneous fibrosis with perivascular and interstitial inflammation, with lymphocytes and plasma cells spilling into the sclerosed fascia, and focal fibrinoid necrosis. Treatment was begun with intravenous pulse doses of methylprednisolone, prednisone (20 mg daily), and subcutaneous methotrexate (25 mg weekly), and the patient's painful induration had resolved and gait had normalized at the 6-month follow-up. Our case suggests that exposure to organic solvents could be implicated in the pathogenesis of eosinophilic fasciitis and highlights the importance of a thorough occupational history to prevent repeat exposures to potentially causative agents.

Comorbidities in pediatric alopecia areata.

BACKGROUND: Comorbidities are associated with higher health care costs, complex management, and poorer health outcomes. Identification and treatment of comorbid conditions in paediatric alopecia areata (AA) patients could provide an opportunity to improve health outcomes. OBJECTIVES: To determine the prevalence of comorbidities among paediatric patients with AA using a large de-identified aggregated patient database. METHODS: This is a cross-sectional study using aggregated health record data through April 1, 2019. Patients ≤18 years of age, with alopecia areata (n = 3510) and without alopecia areata (n = 8 310 710) were identified. The primary outcome was the prevalence of comorbidities among AA patients. RESULTS: Of the 8 314 220 paediatric patients, 3510 (1570 males and 1940 females) had a diagnosis of alopecia areata. The most common comorbidities included atopic dermatitis (17.4% vs. 2.2% controls, OR 9.2, 95% CI 8.55-10.18, P < 0.001), anaemia (7.7% vs. 2.4% controls, OR 3.4, 95% CI 3.06-3.92, P < 0.001), obesity (5.7% vs. 1.1% controls, OR 5.6, 95% CI 4.76-6.34, P < 0.001), vitamin D deficiency (5.1% vs. 0.4% controls, OR 14.7, 95% CI 13.5-18.1, P < 0.001), hypothyroidism (2.6% vs. 0.2% controls, OR 12, 95% CI 10.73-15.9, P < 0.001), vitiligo (1.4% vs. 0.04% controls, OR 32.2, 95% CI 24.01-42.1, P < 0.001), psoriasis (1.4% vs. 0.07% controls, OR 20.6, 95% CI 15.55-27.2, P < 0.001), hyperlipidemia (1.4% vs. 0.2% controls, OR 5.9, 95% CI 4.4-7.7, P < 0.001), and depression (2.6% vs. 0.6% controls, OR 4.8, 95% CI 5.09-9.45, P < 0.001). CONCLUSIONS: Findings from this study suggest that children with AA are more likely to have certain autoimmune and metabolic disorders than the general paediatric population. Paediatric AA patients display a severe burden of autoimmune and metabolic diseases, thus in daily practice, dermatologists might consider multidisciplinary management in these patients.

Comorbid conditions in lichen planopilaris: A retrospective data analysis of 334 patients.

BACKGROUND: Lichen planopilaris (LPP) is a rare, cicatricial, lymphocyte-mediated alopecia that is thought to have an autoimmune pathogenesis and possibly related to other autoimmune diseases. However, data are limited and studies that examine comorbid conditions are lacking. OBJECTIVES: We sought to determine the prevalence of systemic comorbid conditions, nutritional deficiencies, psychological problems, and skin cancers in patients with LPP. METHODS: We identified 334 patients with LPP who were seen in the Department of Dermatology at the Cleveland Clinic Foundation between 2000 and 2016. Patients with LPP were compared with 78 control patients with a diagnosis of seborrheic dermatitis. RESULTS: There were more female patients with LPP compared with the controls (93.1% vs. 79.5%; p < .001) but the average age did not differ (54.77 ± 12.83 vs. 52.19 ± 15.37; p = .12). Conditions positively associated with LPP were Hashimoto's thyroiditis (6.3% vs. 0%; p = .023), hypothyroidism (24.3% vs. 12.8%; p = .028), and hirsutism (11.4% vs. 1.3%; p = .006). Negatively associated conditions were allergic rhinitis (15% vs. 24.4%; p = .046), diabetes mellitus type II (11.7% vs. 21.8%; p = .019), hyperlipidemia (38.6% vs. 52.6%; p = .024), vitamin D deficiency (50% vs. 65.4%; p = .014), depression (15.6% vs. 28.9%; p = .018), and sleep problems (7.5% vs. 29.5%; p < .001). CONCLUSIONS: Our study further emphasizes that dermatologists should screen patients with LPP for autoimmune disorders that are associated with LPP and complete a full metabolic workup to avoid missing other abnormalities. The importance of atopy, autoimmune disorders, endocrine disorders, nutritional deficiencies, psychological problems, and skin cancers in patients with scarring alopecia should be better understood.

Cutaneous Adnexal Cylindroma of Breast: Epithelial Immunoreactivities for GATA-3, Mammaglobin, and E-Cadherin Do Not Equate to a Mammary Ductal Neoplasm.

Cylindromas are benign epithelial neoplasms derived from cutaneous eccrine adnexal structures. These tumors are most commonly encountered on the head, neck, and scalp of older women. In rare instances, solitary cylindromas may arise at other body sites. In the current case, a cylindroma of the skin of the breast was diagnosed by complete excision. Immunohistochemical studies confirmed the tumor cells to be immunoreactive with cytokeratin AE1/3, cytokeratin 5/6, cytokeratin 7, p63, and SOX10. The neoplastic cells were also noted to be immunoreactive with markers typically expected to be positive in ductal epithelium of the breast including GATA3, mammaglobin, and E-cadherin. The case emphasizes the importance of correlating clinical setting, imaging studies, patient history, and careful microscopic evaluation in arriving at an accurate diagnosis. This case also illustrates the point that not all "breast" tumors that are confirmed to be positive for GATA3, mammaglobin, and E-cadherin are derived from mammary ducts.

The utility of cytokeratin 5/6 in the recognition of cutaneous spindle cell squamous cell carcinoma.

BACKGROUND: Cutaneous spindle cell squamous cell carcinoma (SSCC) is a challenging diagnosis since it may be difficult to distinguish from spindle cell melanoma, leiomyosarcoma and atypical fibroxanthoma. Furthermore, it may be difficult to demonstrate epithelial differentiation by a traditional immunohistochemical panel. We performed an expanded immunohistochemical evaluation of ultrastructurally documented SSCC to assess its utility in diagnosing this entity. METHODS: We identified 16 cases of SSCC that were composed predominantly of spindle-shaped cells and with ultrastructural evidence of epithelial differentiation (i.e. at least rudimentary cell junctions). Immunohistochemical analysis using antibodies to a variety of cytokeratins (AE1/3, K903, CK5/6) and S-100 protein was performed. The extent of immunostaining was graded on a scale of 0 to 4+ (0: no staining; 1+: < or =25%; 2+: 26-50%; 3+: 51-75%; 4+: >75%). RESULTS: Of the 16 cases, 6 expressed AE1/3 (38%), 8 expressed K903 (50%) and 11 (69%) expressed CK5/6. Six cases were positive for all three CK markers and two cases were positive for both K903 and CK5/6 but negative for AE1/3. Three cases (19%) stained for CK5/6 without any staining for AE1/3 or K903. Five cases (31%) were negative for all epithelial markers. The extent of CK5/6 staining was either similar to or greater than K903 staining in 7 of 8 cases that stained with both markers. All 16 cases were negative for S-100 protein. CONCLUSIONS: Including CK5/6 in the initial battery of immunostains performed on a cutaneous spindle cell neoplasm can help demonstrate epithelial differentiation in SSCC, even in the absence of AE1/3 or K903 staining. However, some cases of cutaneous SSCC can only be confirmed ultrastructurally, as up to one-third may not show evidence of epithelial differentiation using an expanded immunohistochemical panel.

Familial cutaneous mastocytosis.

Cutaneous mastocytosis appears to occur sporadically; however, familial inheritance has been reported in 50 families since the mid-1880s. We report four cases of telangiectasia macularis eruptiva perstans (TMEP) occurring in three generations of a family. Whereas most patients with TMEP manifest in adulthood, all of the members of this family developed initial lesions during childhood. This is the third documented instance of familial mastocytosis affecting members of three generations, and the first report of familial TMEP. Although the inheritance pattern is unknown, the presentation of disease in this family further supports the hypothesis of an autosomal dominant mode of transmission with incomplete penetrance.

Evaluation of a new paraffin-reactive CD7 T-cell deletion marker and a polymerase chain reaction-based T-cell receptor gene rearrangement assay: implications for diagnosis of mycosis fungoides in community clinical practice.

BACKGROUND: T-cell deletion and T-cell receptor (TCR) gene rearrangement studies are helpful in the early diagnosis and subsequent management of mycosis fungoides (MF). However, this often requires fresh-frozen tissue that can be difficult to obtain and evaluate in community clinical practice. A new CD7 antibody, the most sensitive and specific T-cell deletion marker, and a new TCR-gamma gene rearrangement polymerase chain reaction (PCR) assay (TCR-gamma PCR) are now available on routine paraffin-embedded biopsy specimens. OBJECTIVE: Our purpose was to assess the utility of CD7 deletion and TCR-gamma PCR in the diagnosis of MF using routine paraffin-embedded biopsy material. METHODS: Cases of MF (n = 17) with matching frozen tissue immunohistochemistry and benign reactive dermatoses (lichen planus; n = 27) were assessed for CD7 (Clone: CD7-272) deletion and TCR-gamma PCR using paraffin-embedded biopsy specimens. RESULTS: Excellent concordance comparing frozen and paraffin embedded CD7 immunostaining (88%) was observed. CD7 deletion and TCR-gamma PCR was sensitive (94%) and specific (96%) for a diagnosis of MF using paraffin-embedded biopsy specimens. CONCLUSION: In the diagnosis of MF, detection of CD7 deletion and monoclonal TCR rearrangements can be successfully performed in a cost-effective, timely fashion using routine formalin-fixed paraffin-embedded biopsy specimens.

Shedding: how to manage a common cause of hair loss.

Although telogen effluvium, or shedding-the most common type of diffuse hair loss in both women and men-is usually self-limiting, the condition may become chronic if the trigger is not identified and corrected. The authors discuss the physiologic and emotional triggers, clinical presentation, diagnosis, and management strategies, including the importance of patient education and reassurance.

Lipodystrophy.

Congenital lipodystrophy is an uncommon autosomal recessive disorder that occurs mainly in females and is characterized by loss of subcutaneous fat, insulin-dependent diabetes mellitus, and masculinization due to defective metabolism of fat. Acquired lipodystrophy is now most commonly encountered in patients infected with HIV who take protease inhibitors. We present an illustrative case of lipodystrophy and review the presenting signs allowing for an accurate clinical diagnosis.

Hair loss: an overview.

Hair loss is a common problem in men and women. Correct diagnosis of hair disorders is complex and requires evaluation of clinical presentation, history, physical examination, and laboratory tests. Hair loss may be categorized as hair shaft abnormalities, permanent alopecia, or nonpermanent alopecia. Nonpermanent alopecia, the most common type, includes androgenetic alopecia, telogen effluvium, alopecia areata, and traction alopecia. The hallmark of this group is the possibility of complete regrowth with adequate treatment.

Hirsutism in women. Effective therapy that is safe for long-term use.

Hirsutism should be considered part of the androgen-excess syndrome unless another cause (e.g., masculinizing tumor, androgenic-drug use) can be established. Medical evaluation for transient or late-onset androgen excess, polycystic ovary syndrome, and insulin resistance is important because of the risks associated with chronic androgen excess. Treatment of insulin resistance with antiandrogen and/or insulin-lowering therapy can reduce ovarian testosterone levels and hirsutism. Simple laboratory evaluation (i.e., measuring total and free testosterone, DHEAS, and androstenedione) identifies about half of patients with hyperandrogenism. More extensive evaluation and testing are required in the remaining half. Combination therapies, specifically oral contraceptives along with antiandrogen agents, are the most effective. Studies suggest that addition of low-dose GnRH agonist therapy prolongs remission of hirsutism. Most methods produce improvement within 6 months, with continued improvement at 12 months. Successful treatment results in finer hair, decreased rate of growth, decreased need for cosmetic camouflage or removal, and improved appearance. All methods, whether used continuously or intermittently, should be considered long term.

A morphologic study of dermatofibrosarcoma protuberans: expansion of a histologic profile.

UNLABELLED: Dermatofibrosarcoma protuberans (DFSP) is a fibrohistiocytic tumor of intermediate malignancy characterized by a distinctive storiform growth pattern and frequent local recurrences. In this study, we retrospectively reviewed 48 cases of DFSP diagnosed at the Cleveland Clinic Foundation between 1970 and 1999 to determine the prevalence of morphologic variations including the presence of giant cell fibroblastoma (GCF)-like areas, multinucleated giant cells, hypercellular zones and fibrosarcomatous change. RESULTS: The cohort consisted of 42 patients (20 males, 22 females) with a median age at diagnosis of 40 years (range: 10-73 years). Forty-one primary tumors and seven recurrences were evaluated from these 42 patients. Tumor sites included the trunk (22 cases), head and neck (8 cases), upper extremities (7 cases) and lower extremities (6 cases). GCF-like areas were identified in seven (14.6%), multinucleated giant cells in ten (20.8%), hypercellular zones in 12 (25%) and fibrosarcomatous change in six (12.5%) cases, respectively. Combinations included giant cells and GCF-like areas (two cases), giant cells and hypercellular zone (two cases), and GCF-like areas and hypercellular zones (one case). Our findings suggest that DFSP has a wider range of morphologic features, including GCF-like areas, multinucleated giant cells, hypercellular zones and fibrosarcomatous change, than has been previously recognized in the literature.

Cutaneous presentation of posttransplant lymphoproliferative disorder.

Posttransplant lymphoproliferative disorder (PTLD) is a known complication of solid organ and bone marrow transplantation. However, cutaneous manifestation is an extremely rare phenomenon of this disorder. We describe a lung transplantation patient with cutaneous PTLD who presented with bilateral extremity nodules and fever.

Elastic tissue in scars and alopecia.

A recent report suggests that elastic fibers appear in scars in a time-dependent fashion. This observation prompted our investigation, because we have found elastic tissue stains helpful in determining the pattern of scarring in cases of permanent alopecia. We carried out this investigation to determine if the Verhoeff-Van Gieson (VVG) elastic stain can reliably differentiate scarred from non-scarred dermis and to test our hypothesis that elastic stained sections are helpful in distinguishing lichen planopilaris (LPP) from lupus erythematosus (LE), central progressive alopecia in black females ("follicular degeneration syndrome" and "hot comb alopecia" are other terms used to describe this condition) and classic ivory white idiopathic pseudopelade. We studied histological sections from surgical scars of known duration, stained with the VVG elastic stain and VVG-stained sections of scalp biopsies from patients with established lesions of permanent alopecia. In most cases, both vertical and transverse sections were examined. In every case, the VVG stain clearly differentiated scar from the normal surrounding dermis. Distinct patterns of elastic tissue allowed for correct classification in most of the well-established cases of permanent alopecia studied. We determined that the Verhoeff-Van Gieson stain is an excellent stain to evaluate the pattern of scarring in cases of permanent alopecia and elastic tissue stains may be helpful in the histological evaluation of alopecia.

alpha-Hydroxy acid-based cosmetic procedures. Guidelines for patient management.

alpha-Hydroxy acid (AHA) peels and home regimens have recently been recognized as important adjunctive therapy in a variety of conditions including photodamage, actinic damage, melasma, hyperpigmentation disorders, acne, and rosacea. Overall in our experience and in the literature, AHAs have a proven level of safety and efficacy in a variety of skin types. Although their exact mechanism of action is unknown, it has been demonstrated that AHAs improve these disorders by thinning the stratum corneum, promoting epidermolysis, dispersing basal layer melanin, and increasing collagen synthesis within the dermis. In patients with photodamage, AHA peels and topical products are often combined with retinoids and other antioxidants for maximum benefit. Similarly, synergistic effects of fluorouracil and glycolic acid are observed in the treatment of diffuse actinic keratoses. For patients with melasma, AHA peels and combination products containing bleaching agents such as hydroquinone, kojic acid, and glycolic acid seem to have increased efficacy. Acne and rosacea patients can see improved results when standard regimens like antibacterials and topical retinoids are supplemented with AHA peels and lotions. However, care should always be taken prior to commencing treatment with AHA peels and topical products. By obtaining a thorough history and physical examination, the physician will identify any specific factors like medications, prior procedures and medical conditions which can affect the outcome of the peel. During the interview, there should be open discussion of patient questions and concerns so that realistic expectations can be made. Pre- and post-peel regimens should also be reviewed in full as patient compliance is essential to ensure the success of a series of AHA peels.

A lifetime of healthy skin: implications for women.

During her lifetime, a woman faces the possibility of seeking dermatological assistance for a myriad of conditions, including acne, rosacea, striae, photodamage, and skin cancers. It is important for clinicians and patients to be aware of the symptoms of these conditions as well as the most beneficial approaches for prevention, diagnosis, treatment, and management. The life expectancy of women has increased and predictions for the year 2050 estimate the average age at 81 years. This will place women at greater risk for dermatological problems, especially photodamage and skin cancer. In addition, various ethnic groups may manifest these conditions differently. Although acne is most prevalent among teenaged males, most can expect clearing by age 25. Females may continue to experience acne into the adult years, sometimes beyond the age of 40. Although it is not a life-threatening disease, acne may have psychosocial and quality-of-life consequences. Treatments for acne can be topical or systemic, and include retinoids, antibiotics, benzoyl peroxide, azelaic acid, and hormonal therapy. Rosacea is more common in women (especially during menopause) than in men. It is a chronic condition that can cause complications, including telangiectasia, conjunctivitis, and blepharitis. Although there is no cure, rosacea can be managed and controlled with medication. Topical antibiotics, such as metronidazole, and systemic antibiotics, such as tetracycline, clarithromycin, and doxycycline, are used to manage rosacea. Striae, or stretch marks, occur most frequently in pregnant women, adolescents experiencing growth spurts, weight lifters, and the obese. Although not a health threat, they can be psychologically distressing. There are not many treatment options for striae, but topical tretinoin and the pulsed dye laser offer promising results. Intrinsic, or normal, aging of the skin results from the process of chronological aging. Photodamage is skin damage caused by chronic exposure to ultraviolet (UV) light. It is the leading cause of extrinsic aging, or alterations of the skin due to environmental exposure. Estimates indicate that almost half of a person's UV exposure occurs by age 18. Photoaging causes numerous histologic, physiologic, and clinical changes; it also increases the risk for skin cancer. Photodamage can be prevented through the use of sun screens, protective clothing, and avoidance of the sun during peak intensity time. The only product approved by the FDA for the treatment of photodamage (fine wrinkles, mottled hyperpigmentation, and skin roughness), topical tretinoin emollient cream, may help prevent additional photoaging when it is used to treat existing photoaging. Other management options for photodamaged skin include alpha-hydroxy acids, antioxidants, antiandrogens, moisturizers, and exfoliants. In patients with excessive manifestations of photodamage, surgical management may be needed, including dermabrasion, chemical peels, soft tissue augmentation, laser resurfacing, botulism toxin, and Gortex threads. Clinicians must educate their patients about the most appropriate skin care regimen as well as approaches for preventing and treating common afflictions. In this way, women will have the best opportunity for having and maintaining healthy skin.

Pigmented purpuric dermatosis in a young male.

The pigmented purpuric dermatoses (PPD) are a group of disorders that most often occur on the lower extremities of middle-aged adults as asymptomatic, yellow-orange patches with petechiae. The PPD represent a benign, often chronic, capillaritis of unknown cause. We present a case of PPD on the abdomen of a young male and emphasize careful observation in such a patient to reveal possible progression to purpuric mycosis fungoides.