Linear focal elastosis in a young black man: A new presentation

Linear focal elastosis in a young black man: A new presentation Ali Moiin, MD, and Ken Hashimoto, MD Detroit, Michigan We report the sixth case oflinear focal elastosis. Our patient is a thin 29-year-old black man who was first seen with rows of papules with a yellow hue distributed symmetrically on the lumbar region of the back. The patient had been aware of these lesions since early childhood, and similar lesions reportedly had occurred in his late father. Light microscopic examination demonstrated dermal thickening but no change in the epidermis. The elastic tissue stain revealed fragmented elastic fibers throughout the dermis. This case meets previously defined diagnostic criteria for linear focal elastosis, which has been reported as isolated cases in elderly white or Japanese men. Our patient is the first young black man with a possible familial association. (J AM ACAD DERMATOL 1994;30:874-7.) In 1989 Burket et a1. 1 described three elderly white men with palpable yellow striae on their backs. Histologically, the lesions showed an increase in fragmented elastic fibers. On the basis ofclinical and histologic findings, the authors proposed the term linear focal elastosis (LFE). In 1991 Hagari et a1. 2 described similar lesions in an elderly Japanese man and described the ultrastructural features. Since then, only two other cases have been reported, both in elderly white men. 3, * We report the first case of extensive LFE in a young black man and review the literature. CASE REPORT A 29-year-old black man had horizontal parallel rows of striae with a yellow hue symmetrically distributed on the lower and upper back (Fig. 1). Striae extended to the buttocks where the parallel lines were formed along the skin cleavage lines. The patient had been aware of these lesions since childhood, and recent weight gain was denied. His medical history and physical examination revealed no evidence of similar disease. The patient did not take any medication and had never been given oral or topical steroids. Similar lesions reportedly had occurred From the Department of Dermatology and Syphilology, Wayne State University School of Medicine. Reprint requests: Ali Moiin, MD, Department of Dermatology, 5E-UHC, 4201 81. Antoine, Detroit, MI 48201. Copyright@ t994 by the American Academy of Dermatology, Inc.

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*Palmer L, Madison S, Linear focal elastosis. Presented at the Fifty-first Annual Meeting of the American Academy of Dermatology, Sun Francisco, Calif., Dec. 5-10, 1992, Gross and Microscopic Presentation.

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in his late father, who also had these lesions since childhood. Results of laboratory examination showed normal complete blood count, urine analysis, and serum cortisol levels. A biopsy specimen demonstrated dermal thickening and focal degeneration of connective tissue producing multiple, strongly eosinophilic foci and tissue defects but no change in the epidermis (Fig. 2). Verhoeff stain for elastic fiber revealed that abnormal elastic fibers were increased in these foci of degeneration and pale-staining regions (Fig. 3). The elastic fibers were arranged in a wavy pattern between collagen bundles. In the mid and reticular dermis elastic fibers were not distributed evenly, but were aggregated, clumped, curled, or fragmented (Fig. 4). There were areas where no elastic fibers were found. Oxytalanfiber in the papillary dermis appeared normal. Electron microscopic examination showed that the elastic fibers were primarily short, lumpy, and branched. The components of elastic fibers, such as elastofibrils, skeleton fibrils, and elastin deposition, were normal in most fibers. In some fibers a dense amorphous material was noted at the periphery (Fig. 5). DISCUSSION The first three cases of LFE were reported by Burket et al. l Each occurred as an incidental finding in elderly white men. The authors described layered, symmetric, palpable yellow lines on the back. Light microscopy showed a thickened, pale-staining dermis. The elastic fiber stain revealed the palestaining material in the dermis to be wavy, fragmented, and agglutinated. Electron microscopy confirmed the presence of numerous fragmented elastic fibers. Hagari et al. 2 studied the fine structure of

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Fig. 1. Rows of striae with yellow hue on lower and upper portions of the back.

Fig. 2. Note multiple eosinophilic foci throughout dermis; also note normal epidermis. (Hematoxylin-eosin stain; X200.)

LFE and reported three additional features with electron microscopy. First, they observed granular or electron-dense materials widely distributed in the matrix. elastin. Second, the elastic fiber microfibrils appeared to be continuous, with intracytoplasmic filaments of fibroblasts as has been described in normal elastogenesis. 4 Third, sequential maturation of elastic fibers was seen. These findings indicated that active elastogenesis still exists in the lesions. On the basis of the features of increased elastic tissue, focality, and linearity in these lesions, Burket et al. l coined the term linear focal elastosis. In normal skin elastic fibers are only visible by special stains; the fibers are thin and wavy, measure I to 3 p,m in diameter, and are concentrated in the reticular dermis. Elastic fibers in the papillary dermis become thinner as they reach the epidermis. In the papillary dermis the elastic fibers run either parallel to the dermal-epidermal junction, where they are referred to as oxytalan fibers, or perpendicular and ascending in the papillary dermis, where they

are termed elauninfibers. 5 Electron microscopy of elastic fibers shows that they consist of two components: microfibrils and matrix. elastin. Microfibrils constitute 15% of mature elastic fibers, and the remainder is composed of matrix.. 5 The greater the immaturity of elastic fibers, the more predominant the microfibrils, as in young children in whom microfibrils constitute more than 50% of elastic fibers. 5 There is a gradual decrease in the proportion of microfibrils to elastin by age 30, and by age 60 microfibrils are diminished, and the elastic fiber consists mainly of elastin. The presence of dense elastin in our patient and the patient of Hagari et a1. 2 may indicate a premature aging of elastic fibers. Clinically LFE resembles striae distensae that result from weight gain, hormonal changes, steroid treatment, or pregnancy. 5 The striae are usually located in abdomen, thighs, arms, or breast. In contrast, lesions of LFE are located on the back and are raised. None of the patients had any hormonal or weight change. Striae distensae also show a flattened

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Fig. 3. Abnormal elastic fiber in eosinophilic foci. (Verhoeff stain, x400.) Fig. 4. Curled clumps of aggregated elastic fibers in mid and reticular dermis. (Verhoeff stain; X400.)

Fig. 5. Electron microscopy shows short, lumpy, branched elastic fibers with amorphous material. (X21,000.)

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epidermis with alteration of the collagen bundles without any changes in elastic tissue. 6 These findings differ in patients with LFE. We report the first case of LFE in a young black man. The history of a similar disease in his late father should promptfamilial studies in future patients with LFE.

REFERENCES 1. Burket JM, Zelickson A, Padilla RS. Linear focal elastosis (elastotic striae). JAM ACAD DERMATOL 1989;20:633-6.

Porter et at. 2. Hagari Y, Mihara M, Shimao S. Linear focal elastosis: an ultrastructural study. Arch Dermatol 1991;127:1365-8. 3. White GM. Linear focal elastosis: A degenerative or regenerative process of striae distensae? J AM ACAD DERMATOL 1992;27:468. 4. Hashimoto K, DiBella RJ. Electron microscopic studies of normal and abnormal elastic fibers of skin. J Invest Dermatol 1967;48:405-23. 5. Lever WF, Schaumburg-Lever G. Histopathology of the skin. 7th ed. Philadelphia: JB Lippincott, 1990;312. 6. Breathnach AS. Ultrastructure of epidermis and dermis in striae atrophicae. In: Moretti GR, Rebora A, eds. Striae distensae. Milan: Brocades, 1976:35.

Oral features of a family with benign familial neutropenia S. R. Porter, BSc, PhD, MBChB, FDS, RCS, RCSE,a J. Luker, PhD, FDS, RCSE,a C. Scully, MD, MDS, PhD, FDSRCPS, FFDRCSI, FDSRCS, FRCPath,a and A. Oakhill, MD, FRCp b Bristol, United Kingdom The oral features of three members of a family with familial benign neutropenia (a mother and two children) are detailed. Prepubertal periodontitis, oral ulceration, and angular stomatitis were the principal features. (J AM ACAD DERMATOL 1994;30:877-80.) Neutropenias are characterized by a transient or chronic decrease in the peripheral blood neutrophil count to 2 or more standard deviations below the normal mean. I Affected patients can have a spectrum of clinical manifestations ranging from health to recurrent, possibly life-threatening bacterial infections. 1 Mucosal ulceration and increased susceptibility to gingivitis and periodontitis are the main oral manifestations of neutropenias. Although numerous reports have been made of the oral aspects of cyclic neutropenia,2-9 little information is available about the oral features of chronic neutropenias. This report details the orofacial features of a family with benign familial neutropenia.

From the Centre for the Study of Oral Disease, University Department of Oral Medicine, Pathology, and Microbiology, Bristol Dental School and Hospital,' and the Institute for Child Health, Bristol Children's HospitaLb Reprint requests: S. R. Porler, Centre for the Study of Oral Disease, Bristol Dental School and Hospital, Lower Maudlin Street, Bristol, BSI 2LY, United Kingdom. Copyright ® 1994 by the American Academy or Dermatology, Inc. 0190-9622/94 $3.00 + 0

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CASE REPORTS

Case 1 A 3V2-year-old girl had severe gingivitis and stained upper incisors. Although she had developed normally she had a history of recurrent furuncles, otitis media, eczema, and superficial oral ulceration. The girl's mother was known to have benign familial neutropenia. Laboratory studies revealed a slightly low hemoglobin level (9.5 gmj dl) and a normal platelet count and white blood cell count (5 X 109jL), but she had neutropenia (0.15 X 109jL) with monocytosis (0.9 X 109 /L), lymphocytosis (3.3 X 109jL), and eosinophilia (0.9 X 109 jL). A bone marrow aspirate revealed normal erythropoiesis but little granulopoiesis beyond the metamyelocyte stage. Levels of serum IgA, IgG, and IgM were raised, probably as a result of chronic infection, and there were elevated titers of antibodies to herpes simplex virus, varicella zoster virus, and cytomegalovirus. The child had a normal female karyotype. A diagnosis of benign familial neutropenia was made and she was given an iron supplement and 1% hydrocortisone cream for the mild eczema, Examination revealed that the child was above the 50th percentile for height, weight, and normal intelligence. She had bilateral blepharitis (Fig. 1) but no lymphadenopathy. The patient had no oral mucosal lesions, but oral hy-

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