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Chapter 48 Genetic Epidermal Syndromes: Disorders of Aging Nancy Burton Esterly, Nancy Burton EsterlySearch for more papers by this authorEulalia Baselga, Eulalia BaselgaSearch for more papers by this authorPeter M. H. Chan, Peter M. H. ChanSearch for more papers by this authorBeth A. Drolet, Beth A. DroletSearch for more papers by this authorAnita P. Sheth, Anita P. ShethSearch for more papers by this authorCindy L. Lamerson, Cindy L. LamersonSearch for more papers by this author Nancy Burton Esterly, Nancy Burton EsterlySearch for more papers by this authorEulalia Baselga, Eulalia BaselgaSearch for more papers by this authorPeter M. H. Chan, Peter M. H. ChanSearch for more papers by this authorBeth A. Drolet, Beth A. DroletSearch for more papers by this authorAnita P. Sheth, Anita P. ShethSearch for more papers by this authorCindy L. Lamerson, Cindy L. LamersonSearch for more papers by this author Book Editor(s):James J. Nordlund, James J. Nordlund Dermatologist and Professor Emeritus, Group Health Associates, Cincinnati, OH, USASearch for more papers by this authorRaymond E. Boissy, Raymond E. Boissy Professor of Dermatology and Cell Biology, University of Cincinnati College of Medicine, Cincinnati, OH, USASearch for more papers by this authorVincent J. Hearing, Vincent J. Hearing Chief, Pigment Cell Biology Section, Laboratory of Cell Biology, National Institutes of Health, Bethesda, MD, USASearch for more papers by this authorRichard A. King, Richard A. King Director, Genetics Division, Department of Medicine, Institute of Human Genetics, University of Minnesota, Minneapolis, MN, USASearch for more papers by this authorWilliam S. Oetting, William S. Oetting Associate Professor, Genetics Division, Department of Medicine Institute of Human Genetics, University of Minnesota, Minneapolis, MN, USASearch for more papers by this authorJean-Paul Ortonne, Jean-Paul Ortonne Professor of Dermatology and Chairman, Department of Dermatology, University of Nice-Sophia Antipolis, Nice, FranceSearch for more papers by this author First published: 25 April 2006 https://doi.org/10.1002/9780470987100.ch48 AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Summary This chapter contains sections titled: Acrogeria Metageria Progeria Xeroderma Pigmentosum Werner Syndrome The Pigmentary System: Physiology and Pathophysiology, Second Edition RelatedInformation

Chapter 51 Hypermelanosis Associated with Gastrointestinal Disorders Eun Ji Kwon, Search for more papers by this authorVictoria P. Werth, Search for more papers by this authorJames J. Nordlund, Search for more papers by this authorJoerg Albrecht, Search for more papers by this authorNancy Burton Esterly, Search for more papers by this authorEulalia Baselga, Search for more papers by this authorBeth A. Drolet, Search for more papers by this author Eun Ji Kwon, Search for more papers by this authorVictoria P. Werth, Search for more papers by this authorJames J. Nordlund, Search for more papers by this authorJoerg Albrecht, Search for more papers by this authorNancy Burton Esterly, Search for more papers by this authorEulalia Baselga, Search for more papers by this authorBeth A. Drolet, Search for more papers by this author Book Editor(s):James J. Nordlund, Dermatologist and Professor Emeritus, Group Health Associates, Cincinnati, OH, USASearch for more papers by this authorRaymond E. Boissy, Professor of Dermatology and Cell Biology, University of Cincinnati College of Medicine, Cincinnati, OH, USASearch for more papers by this authorVincent J. Hearing, Chief, Pigment Cell Biology Section, Laboratory of Cell Biology, National Institutes of Health, Bethesda, MD, USASearch for more papers by this authorRichard A. King, Director, Genetics Division, Department of Medicine, Institute of Human Genetics, University of Minnesota, Minneapolis, MN, USASearch for more papers by this authorWilliam S. Oetting, Associate Professor, Genetics Division, Department of Medicine Institute of Human Genetics, University of Minnesota, Minneapolis, MN, USASearch for more papers by this authorJean-Paul Ortonne, Professor of Dermatology and Chairman, Department of Dermatology, University of Nice-Sophia Antipolis, Nice, FranceSearch for more papers by this author First published: 25 April 2006 https://doi.org/10.1002/9780470987100.ch51 AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinked InRedditWechat Summary This chapter contains sections titled: Porphyria Cutanea Tarda Cronkhite-Canada Syndrome Hemochromatosis and Hemosiderosis Primary Biliary Cirrhosis Inflammatory Bowel Disease and Pigmentation Pellagra Peutz-Jeghers Syndrome The Pigmentary System: Physiology and Pathophysiology, Second Edition RelatedInformation

Pediatric DermatologyVolume 23, Issue 1 p. 84-86 Large Plantar Mass in a Newborn Anne W. Lucky M.D., Anne W. Lucky M.D.Search for more papers by this authorJulie S. Prendiville M.B., M.R.C.P.I., F.R.C.P.C., Julie S. Prendiville M.B., M.R.C.P.I., F.R.C.P.C.Search for more papers by this authorSheila S. Galbraith M.D., Sheila S. Galbraith M.D. Departments of Dermatology and PediatricsSearch for more papers by this authorDavid M. King M.D., David M. King M.D. Orthopedic Surgery, Medical College of Wisconsin, Milwaukee, WisconsinSearch for more papers by this authorBeth A. Drolet M.D., Beth A. Drolet M.D. Departments of Dermatology and PediatricsSearch for more papers by this authorNancy B. Esterly M.D., Nancy B. Esterly M.D. Departments of Dermatology and PediatricsSearch for more papers by this author Anne W. Lucky M.D., Anne W. Lucky M.D.Search for more papers by this authorJulie S. Prendiville M.B., M.R.C.P.I., F.R.C.P.C., Julie S. Prendiville M.B., M.R.C.P.I., F.R.C.P.C.Search for more papers by this authorSheila S. Galbraith M.D., Sheila S. Galbraith M.D. Departments of Dermatology and PediatricsSearch for more papers by this authorDavid M. King M.D., David M. King M.D. Orthopedic Surgery, Medical College of Wisconsin, Milwaukee, WisconsinSearch for more papers by this authorBeth A. Drolet M.D., Beth A. Drolet M.D. Departments of Dermatology and PediatricsSearch for more papers by this authorNancy B. Esterly M.D., Nancy B. Esterly M.D. Departments of Dermatology and PediatricsSearch for more papers by this author First published: 24 January 2006 https://doi.org/10.1111/j.1525-1470.2006.00179.xCitations: 2 Address correspondence to Sheila Galbraith, M.D., Department of Dermatology, Medical College of Wisconsin, 9200 W. Wisconsin Avenue, Milwaukee, WI 53226, or e-mail: sgalbrai@mcw.edu. Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Citing Literature Volume23, Issue1January 2006Pages 84-86 RelatedInformation

We report four infants born with necrotic caput succedaneum that led to a scarring alopecia with ongoing inflammation and persistent scale-crust. These lesions did not significantly improve with topical or oral antibiotics, but did respond somewhat to topical corticosteroids. Alopecia with chronic erosive scale-crust and a moderate response to topical corticosteroids are findings consistent with a diagnosis of erosive pustular dermatosis of the scalp.

Odonto-onycho-dermal dysplasia is an ectodermal dysplasia (ED) described once previously in two families who exhibited atrophic malar patches, sparse hair, conical teeth, dystrophic nails and hyperkeratosis of the palms and soles. We describe a boy who developed a blistering malar rash soon after birth. When examined at 11 months, and then at 27 months of age, he had persistent atrophic malar plaques that reddened with heat. He also showed nail dystrophy, sparse hair, lingual concavity of the incisors, a bifid maxillary incisor, a live-cusped molar, and hyperhidrosis of the palms and soles. In addition he had chronic tearing, photophobia, blepharitis, and a mild keratitis.After reviewing EDs with atrophic or scar-like skin changes, we believe this child most resembles the patients with odonto-onycho-dermal dysplasia, although his eye findings are unique.

Although inflammatory bowel disease ( IBD) typically presents with gastrointestinal complaints, mucocutaneous lesions are commonly associated and can precede gastrointestinal symptoms, thereby alerting the clinician to the diagnosis of IBD before the onset of gastrointestinal symptoms. Nine children are reported who had no gastrointestinal symptoms suggestive of IBD but presented with mucocutaneous findings of IBD and were subsequently diagnosed with Crohn's disease or ulcerative colitis based on characteristic features on gastrointestinal endoscopy and/ or biopsies. The majority of the patients had oral and perianal lesions. We believe that IBD is a common etiology for persistent oral lesions in the pediatric population. In addition to a good history, children with unexplained oral mucous membrane lesions should have an examination of the rectal and genital mucosa as well as tests for complete blood count, iron levels, sedimentation rate, albumin, and occult blood in the stool with endoscopy and biopsies to rule out IBD if indicated.

Aplasia cutis of the scalp is often a sporadic condition, but familial occurrences with an autosomal dominant inheritance have been documented. Aplasia cutis of the scalp may be seen in two main clinical variants: oval-shaped membranous aplasia cutis and irregular, larger defects. We report six families in whom more than one member has aplasia cutis of the scalp, all of them with large irregular defects located over the vertex or anterior to the vertex along the sagittal suture. We review previous reports of this entity with clinical pictures and note that in most instances, the defects are of the nonmembranous variant.

Two children with chronic and asymmetric skin indurations are presented. The clinical and pathologic features are suggestive of asymmetric childhood scleredema and stiff skin syndrome. The key features of scleredema and stiff skin syndrome are discussed.

Background: Dermatofibrosarcoma protuberans (DFSP) is an uncommon low-grade fibrohistiocytic tumor that usually occurs on the trunk or proximal extremities and typically appears during the second to fifth decade of life. It most,commonly begins as a red-blue plaque that grows slowly and ultimately becomes nodular. The tumor is associated with a high recurrence rate but low metastatic potential. It rarely presents in childhood and is even more rarely present at birth. The clinical diagnosis of DFSP in infancy or childhood may be difficult because, in its early stages, the tumor often resembles a vascular birthmark.Observations: We studied 6 patients with congenital DFSP who were initially thought to have other diagnoses, highlighting the potential clinical variability in presentation. Half of the cases in this series occurred in areas of the body outside of the typically reported distribution pattern of acquired DFSP and in locations that, therefore, may not arouse suspicion of congenital DFSP.Conclusions: Given the aggressive local potential and high recurrence rate of DFSP, early diagnosis is preferable to facilitate appropriate excision. We recommend that any infant or child presenting with a cutaneous plaque or nodule, even congenital, that does not have characteristic or diagnostic clinical features undergo tissue biopsy for histologic evaluation.

A 14-year-old girl was admitted to the hospital because of persistent throat pain, fever, fatigue, 25 pound weight loss, and leukopenia. On physical examination she was thin, ill-appearing, and had necrotic papules on the face and palpable cervical lymph nodes. Presumptive differential diagnosis included occult malignancy and infection. Numerous investigative procedures failed to elucidate a source. Vasculitis was eventually appreciated after repeat skin biopsy. Numerous serologic studies were performed and were notable for a very low level of the second component of complement without direct evidence of lupus erythematosus (LE) or other autoimmune conditions. A diagnosis of C2 deficiency-associated vasculitis was made. She was treated with high-dose prednisone and cyclophosphamide with resolution of her symptoms. Two years later she returned with marked malar erythema. Antinuclear and Smith antibodies were then detected and a diagnosis of LE was made. She was treated with hydroxychloroquine and sun-avoidance measures with clearance of the malar rash.

Morrell, Dean S. M.D.; Challgren, Eric M.D.; Eapen, Mary M.B.B.S, M.S.; Esterly, Nancy B. M.D. Author Information

Background: Hemophagocytic lymphohistiocytosis is a rare, rapidly progressive, and potentially fatal disorder of activated histiocytes. The initial clinical presentation commonly includes fever, hepatosplenomegaly, and pancytopenia. Skin eruptions are described in up to 65% of patients. Information regarding the morphological features, configuration, and distribution of these eruptions is lacking and is typically reported as nonspecific and "maculopapular." The aim of this report is to better delineate the cutaneous manifestations of the disorder to assist in differentiating the process from other systemic diseases.Observation: A case report of a neonate with hemophagocytic lymphohistiocytosis with generalized purpuric macules is described. The clinical features of 5 other patients with hemophagocytic lymphohistiocytosis at Children's Hospital of Wisconsin, Milwaukee, are summarized. Clinical images of 1 additional neonatal patient with hemophagocytic lymphohistiocytosis are presented as well. These observations demonstrate the varied cutaneous manifestations of hemophagocytic lymphohistiocytosis:erythroderma, generalized purpuric macules and papules, and morbilliform eruptions.Conclusion: Awareness of cutaneous involvement can assist in the initial diagnosis of hemophagocytic lymphohistiocytosis and potentially signify recurrences.

An 11-year-old white girl with Down syndrome was referred for evaluation of numerous white cutaneous papules. The lesions were first noticed on the dorsal aspects of the hands at 4 years of age. New lesions developed on her palms, the tops of her feet, and periorbital/perioral areas over the next several years. There was no history of trauma to the involved areas. The lesions were asymptomatic and many spontaneously resolved without scarring. Past medical history was significant for an atrial-septal defect repaired at 15 months of age, tonsillectomy and adenoidectomy in 1993, cardiac valve surgery in 1996, and sinus surgery in June 2000. The patient had no history of renal failure/ stones, chronic abdominal pain, or skeletal fractures. She was taking no medications or supplements. On examinations, the patient had multiple 1–2 mm, firm, white papules on the dorsal surfaces of her hands and feet, with a few similar lesions on her palms and periorally (Figs. 1 and 2). There was mild erythema surrounding a few lesions, but no livedo reticularis, tissue necrosis, or ulceration. Skin examination was otherwise unremarkable. Serum calcium, phosphorus, parathyroid hormone, 25-hydroxyvitamin D, and 24hour urinary calcium levels were all within normal limits. A shave biopsy of one of the papules was obtained prior to referral to our clinic. WHAT IS YOUR DIAGNOSIS?