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.

Ehlers-Danlos syndrome type IV: a single base substitution of the last nucleotide of exon 34 in COL3A1 leads to exon skipping.

The Ehlers-Danlos syndrome has been classified into nine phenotypic presentations. Type IV is a variant of particular importance because people affected with this genodermatosis are at great risk of spontaneous hemorrhage from vascular rupture or bowel perforation. Recent molecular advances have identified mutations in the gene for type III procollagen as responsible for Ehlers-Danlos syndrome type IV. We report a case of a 14-year-old male with a typical presentation of the type IV variant who was found to have markedly dilated fibroblast cisternae and varying collagen fibril diameter on ultrastructural study. A novel genetic defect was noted by polymerase chain reaction and DNA sequencing of genetic material isolated from skin fibroblast cultures. Analysis of the gene for type III procollagen revealed a single base mutation in the last nucleotide of exon 34. The mutation led to abnormal RNA splicing and skipping of exon 34 on the mRNA level.

Androgenetic alopecia: an autosomal dominant disorder.

A hereditary, androgen-driven disorder, androgenetic alopecia is the most common form of alopecia in humans: its prevalence is 23-87%. Central alopecia is more severe in men; women are more likely to experience diffuse thinning. The acute onset of alopecia in those with inflammatory diseases of the scalp suggests a variety of etiologies, including the impact of inflammatory cells, release of cytokines, presence of growth factors, and increased interaction of stromal cells. Therapeutic modalities, which are most effective when used in combinations, utilize hair growth promoters, antiandrogens, and androgen blockade agents.

Malignant melanoma and Charcot-Marie-Tooth disease.

Two patients with Charco-Marie-Tooth disease subsequently developed cutaneous malignant melanoma. This constellation of diseases may be due to chance, but raises the possibility of a shared neural crest defect or genetic linkage.

Relapsing polychondritis. Immunomicroscopic findings in cartilage of ear biopsy specimens.

Two cases of relapsing polychondritis are reported. Direct immunofluorescence examination of ear biopsy specimens in both patients showed the presence of granular deposits of immunoglobulins and the C3 component of complement at the chondrofibrous junction. These findings suggest that immunomicroscopic examination of ear cartilage could be diagnostically useful in this disease.

A histochemical approach to the differentiation of lichen planus from lupus erythematosus.

A histochemical technic utilizing a maleimide-derived fluorochrome for the detection of disulfide bonds in subepidermal hyaline bodies is described. Lichen planus and the skin lesions of lupus erythematosus, which may have similar clinical, histologic, and immunofluorescent presentations, tend to demonstrate different patterns of reactivity with this reagent. This technic, then, may be used to distinguish between these two cutaneous disorders.

Globular deposits of immunoglobulins and complement in the papillary dermis. Clinical significance.

Globular deposits of immunoglobulins in the papillary dermis have been reported to occur in certain dermatoses, particularly in lichen planus. The clinical correlation of these deposits in 52 skin biopsy specimens reviewed by light and fluorescent microscopy was studied. These cases included five of lichen planus, 24 of lupus erythematosus or related diseases, four of dermatitis herpetiformis, three of drug eruption; two each of bullous pemphigoid, erythema nodosum, porphyria cutanea tarda; one each of vitiligo, pyoderma gangrenosum, neurodermatitis, erythema multiforme, granuloma annulare, vasculitis, epidermolysis bullosa simplex, Rothmund-Thompson syndrome, and four of unspecified dermatoses. Using an arbitrary scale of 1-4 based on the frequency of deposits, 3+ and 4+ deposits were identified in all five cases of lichen planus, as well as in six cases of lupus erythematosus, one of drug eruption, one of bullous pemphigoid, one of erythema nodosum, the vitiligo, vasculitis, and Rothmund-Thompson syndrome cases, and two cases of unspecified dermatoses; other cases showed only 1+ and 2+ deposits. In all five cases of lichen planus, the deposits contained IgM and C3, and in addition, IgA was present in four, IgG and fibrinogen in three. Among non-lichen planus cases, C3 was detected in 11 of 49, and fibrinogen in only four of 49. These findings indicate that globular deposits of Ig in the dermis, though suggestive, are not pathognomonic of lichen planus.

Tumor cell characterization of histiocytic medullary reticulosis.

Tumor cells from a 10-year-old boy was suspected histiocytic medullary reticulosis were examined and were found to have receptor sites for complement, the ability to phagocytize latex beads, and the electron microscopical picture of immature histiocytes. These findings strongly support the diagnosis of histiocytic medullary reticulosis. This disorder and the method of examination of the tumor cells are discussed.

Perforating granuloma annulare in a patient with acquired immunodeficiency syndrome. Immunohistologic evaluation of the cellular infiltrate.

The inflammatory infiltrate found in the lesions of perforating granuloma annulare in a patient with acquired immunodeficiency syndrome was analyzed. Double labeling with fluorescent antibody tags fluorescein isothiocyanate-Leu 4 (peripheral T cells) and phycoerythrin-Leu 2a (cytotoxic/suppressor) revealed the infiltrate to consist predominantly of cytotoxic/suppressor T cells. This finding is a reflection of the patient's altered immune status and calls into question the role of specific T-cell subsets in the pathogenesis of granuloma annulare.

Mucous gland basement membrane immunofluorescence in cicatricial pemphigoid.

Three patients are described with cicatricial pemphigoid and positive immunofluorescence findings in the basement membrane zone of mucous glands of the pharynx, mouth, and nose. These findings appear to be unique to cicatricial pemphigoid.

Pretibial mucin. Histologic patterns and clinical correlation.

BACKGROUND AND OBJECTIVE: We identified several patients with a histologic diagnosis of pretibial myxedema in whom thyroid disease was not found. The purpose of this study was to investigate if histologic characteristics can distinguish between pretibial mucinosis secondary to Graves' disease and that unassociated with thyroid disease. METHODS: Biopsy specimens interpreted as compatible with pretibial myxedema were reviewed; these included 12 cases of pretibial mucinosis with documented Graves' disease, and six cases of pretibial mucinosis without evidence of Graves' disease. Ten specimens interpreted as compatible with stasis dermatitis were also evaluated for histologic characteristics, including the possible presence of mucin. RESULTS: Features that distinguish between pretibial mucinosis associated with Graves' disease and pretibial mucinosis without Graves' disease included preservation of a zone of normal-appearing collagen in the superficial papillary dermis (12/12 with Graves' disease, 0/6 without), mucin deposition in the reticular dermis (12/12 with Graves' disease, 0/6 without), lack of mucin deposition in the superficial papillary dermis (11/12 with Graves' disease, 1/6 without), angioplasia (2/12 with Graves' disease, 6/6 without), and the presence of hemosiderin (2/12 with Graves' disease, 6/6 without). Mucin deposition in the papillary dermis was found in six of 10 specimens interpreted as stasis dermatitis. CONCLUSIONS: There are patients with pretibial mucinosis in whom there is no thyroid disease. Specimens from patients without Graves' disease have features of stasis dermatitis in addition to mucinosis. We conclude that pretibial mucinosis may result from stasis or Graves' disease and that histologic differences allow for accurate differentiation.

Cutaneous macroglobulinosis.

A patient with Waldenström's macroglobulinemia had firm, translucent papules and nodules on the extremities. Clinically, the skin lesions were suggestive of amyloidosis cutis. Histologically, a homogeneous eosinophilic material was observed in the upper dermis and encasing the hair follicles. Special histochemical stains, electron microscopy, and immunofluorescence microscopy identified the material as IgM.

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.

Alopecia: histologic changes.

The major types of alopecia that can be diagnosed histologically are androgenic alopecia, alopecia areata, trichotillomania, inflammatory scarring alopecias, pseudopelade, and anagen and telogen effluviums. Important to recognize are the common histologic features in chronic alopecias, which include follicular plugging, decreased numbers of hair follicles, frequent increased fibrous tracts, and superficial changes of actinic damage. Specific helpful changes to be observed are (1) the presence of actinically damaged skin that would reveal a chronic alopecic condition, (2) the presence and site of the dermal inflammation, (3) whether there is a decrease in the numbers of hair follicles and the presence of a reversal of the anagen/telogen ratio, (4) miniaturization of the hair follicle, (5) evidence of involution, and (6) the presence of scarring as demonstrated with elastic fiber stains. By assessing these histologic changes, the dermatopathologist can make a reasonably specific diagnosis of alopecia.