REVIEW

Umbilical and periumbilical dermatoses Christopher R. Kieliszak, DO,a Tess H. Pollinger, DO,b Megha M. Tollefson, MD,c,d and John R. Griffin, MDe,f,g Washington, District of Columbia; Bethesda, Maryland; Rochester, Minnesota; and Dallas, Texas The umbilicus may be the site of congenital and acquired malformations and may harbor clinical clues to the diagnosis of potentially fatal inherited disorders, primary skin conditions, and a variety of infectious diseases. Both benign and malignant tumors may involve the umbilicus, and some are unique to this site. Finally, cutaneous signs localized to this anatomic location may be found in diverse systemic diseases. ( J Am Acad Dermatol http://dx.doi.org/10.1016/j.jaad.2015.02.1100.)

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n utero, the umbilical cord lies adjacent to critical embryologic fusion planes of the abdominal wall and is responsible for vascular flow between the growing fetus and the placenta. The umbilicus is formed by the separation of the umbilical stump, which generally occurs 1 week after birth.1 This article will review congenital and acquired anatomic abnormalities, cutaneous diseases, neoplasms, infectious diseases, and foreign material involving the umbilicus and periumbilical skin.

CONGENITAL MALFORMATIONS Omphalomesenteric duct malformations During embryologic development, the omphalomesenteric duct connects the yolk sac to the midgut and typically involutes by 6 weeks’ gestation.2 Failure to involute allows a direct connection between the umbilicus and the ileum. Malformations of the omphalomesenteric duct typically present at birth with umbilical drainage of intestinal contents, but can rarely become symptomatic in adolescence. Other signs and symptoms include a pink to red glistening umbilical nodule, described as a ‘‘raspberry tumor,’’3 melena, anemia, abdominal pain, intussusception, or intestinal obstruction.2 Surgical excision is recommended.4,5 Persistence of the most external portion of the omphalomesenteric duct is responsible for umbilical polyps, cysts, or sinuses. From the Division of Otolaryngology,a Head and Neck Surgery, George Washington University, Washington, DC; Department of Dermatology,b Walter Reed National Military Medical Center, Bethesda; Departments of Dermatologyc and Pediatrics,d Mayo Clinic, Rochester; Division of Dermatology,e Baylor University Medical Center, and the Departments of Internal Medicinef and Pathology and Laboratory Medicine,g Texas A&M University Health Science Center, Dallas. Dr Kieliszak is currently affiliated with the Department of Otolaryngology, Head and Neck Surgery, OhioHealth Doctors Hospital, Columbus, Ohio

Abbreviations used: ACD: AGA: C3: CMPI: DIF: IgA: IgG: LAD: SMJN:

allergic contact dermatitis alfa-galactosidase A complement C3 cow’s milk protein intolerance direct immunofluorescence immunoglobulin A immunoglobulin G leukocyte adhesion deficiency Sister Mary Joseph nodule

Urachal malformations The urachus connects the fetal bladder to the umbilicus and involutes with normal development. A patent urachus and an umbilicaleurachal sinus are malformations that typically present in infancy. Clinical findings include drainage of urine from the umbilicus, an umbilical sinus with recurring periumbilical dermatitis,6 a painful mass inferior to umbilicus, and recurrent urinary tract infections. Partial involution may present with umbilical drainage and recurrent periumbilical dermatitis or cellulitis, or as a painful mass just inferior to the umbilicus. Surgery is required to prevent secondary infection and potential development of adenocarcinoma.7 Abdominal wall defects Exstrophy of the bladder, more common in males, is a rare, complex abnormality consisting of an Funding sources: None. Conflicts of interest: None declared. Accepted for publication February 11, 2015. Reprint requests: John R. Griffin, MD, Division of Dermatology, Baylor University Medical Center, 3900 Junius St #145, Dallas, TX 75246. E-mail: [email protected]. Published online April 1, 2015. 0190-9622/$36.00 Ó 2015 by the American Academy of Dermatology, Inc. http://dx.doi.org/10.1016/j.jaad.2015.02.1100

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absent lower abdominal wall inferior to the appears as a skin-colored or pigmented umbilicated umbilicus, separation of the abdominal musculature papule. and pubic rami, and externalization of the bladder epithelium (Fig 1, A).3,5 If left untreated, patients are ACQUIRED ANATOMIC UMBILICAL at increased risk of bladder cancer. ABNORMALITIES Gastroschisis is an abdominal wall defect that is Acquired umbilical hernias are more common in characterized by extrusion of abdominal contents women in the fourth to sixth decades of life18 lacking a membranous and present as a soft covering (Fig 1, C ). The protuberance of tissue at the CAPSULE SUMMARY defect arises to the right of umbilicus. Predisposing conthe umbilicus and is likely ditions include obesity, asciA wide variety of disorders present with caused by vascular comprotes, multiple pregnancies umbilical and periumbilical skin findings. mise.8,9 Surgery is necessary. with prolonged labor, and We provide an update of malformations, An omphalocele is characterlarge intraabdominal tumors. inherited and acquired disorders, ized by partial absence of Surgical treatment is neinfections, neoplasms, and signs of the abdominal wall with cessary because umbilical systemic disease affecting this anatomic externalization of abdominal hernias in adults do not region. contents covered by peritospontaneously involute.19 neal membrane. It can be Knowledge of these clinical entities associated with anomalies Inherited diseases places the dermatologist in a unique of multiple organs, chromoLeukocyte adhesion defiposition to aid in patient care. somal abnormalities, and ciencies (LADs) types I to III BeckwitheWiedemann are autosomal recessive syndrome.5,10 diseases caused by defects in leukocyte adhesion Prune belly syndrome, a triad of hypoplastic or activation and, in type III, platelet activation abdominal musculature, cryptorchidism, and urinary defects.20 Types I and III present in neonatal life tract abnormalities, is named for the characteristic with omphalitis and delay in separation of the finding of wrinkled abdominal skin (Fig 1, D). umbilical stump.21 Other symptoms include Multiorgan system malformations are present and mucositis, periodontitis, and delayed wound healing multidisciplinary care is critical.5 in LAD type I. Immunology referral is critical.20 Fabry disease is an X-linked disorder caused by mutations in the galactosidase alpha (GLA) gene Other umbilical and periumbilical dysplasias resulting in alpha-galactosidase A (AGA) deficiency. A congenital umbilical hernia is caused by incomIt typically presents before puberty with signs plete fascial closure around the umbilical ring. and symptoms of abdominal pain, acroparestheSpontaneous closure is typical by 5 years of age sias, angiokeratoma corporis diffusum, corneal (Fig 1, B).11 The hernia may uncommonly become opacities, hearing loss, hypohydrosis, renal impairincarcerated, strangulated, or rupture, necessitating ment, and cardiovascular and neurovascular disease. surgical consultation. Associated conditions include The abdomen and umbilicus are common sites for the following: low birth weight, African American the angiokeratomas (Fig 2),22 which may be ethnicity, EhlerseDanlos syndrome (dermatospraxis 12 13 type), BeckwitheWiedemann syndrome, mucoarranged as ‘‘periumbilical rosettes.’’3 The diagnosis 14 15 polysaccharidoses, cutis laxa, and congenital can be confirmed by GLA gene sequencing.23 16 hypothyroidism. Pseudoxanthoma elasticum, caused by mutations of the ABCC6 gene, is inherited in an autosomal An umbilical granuloma, or granulation tissue recessive fashion but may demonstrate pseudodothat develops after cord separation,17 is the most minance. It presents in the second decade of common cause of an umbilical mass in neonates. It life and is characterized by fragmentation and manifests as a glistening, red umbilical papule and is calcification of elastic fibers leading to cutaneous, treated with topical silver nitrate; if there is no ocular, and vascular sequelae.24 Skin findings improvement, obtaining a biopsy specimen is indicated. Surgical referral is indicated if ectopic include yellow to orange papules on the lateral tissue, such as pancreas or liver, is noted on the aspect of the neck and flexures, giving a ‘‘plucked biopsy specimen. chicken skin’’ appearance. Rarely, these papules are Polythelia (supernumerary nipple) presents along localized to the periumbilical region with or without the embryonic milk lines passing on each side of the systemic manifestations.25 The typical patient umbilicus from the inguinal folds to the axillae. It without systemic manifestations is a multiparous, d

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Fig 1. A, Exstrophy of the bladder in a female neonate. B, Congenital umbilical hernia in a young child. C, Gastroschisis in a neonate—note the lack of membranous covering and location just right of the umbilicus. D, Prune belly syndrome. A newborn with wrinkled abdominal skin and atonic abdominal musculature. (A-C, Courtesy Candace Granberg, MD. D, Reprinted from Nelson Textbook of Pediatrics, 19th ed. Philadelphia [PA]: Elsevier/Saunders; 2011. Chapter 534. Used with permission. Copyright Elsevier 2011.)

obese, African American woman26; a proposed term for this presentation is ‘‘perforating calcific elastosis.’’27 Primary skin diseases Periumbilical allergic contact dermatitis (ACD) is prevalent among children and adolescents. It is commonly caused by nickel found in belt buckles and jean snaps (Fig 3, A)28,29 or dibenzyldithlocarbamate, a rubber component found in elastic waistbands that reacts with bleach when bleaching undergarments or pants.30 Psoriasis (Fig 3, B) and seborrheic dermatitis have a predilection for the umbilicus. If involvement is limited to the umbilicus,

tinea corporis or Bowen disease should be excluded.31 Clear cell papulosis is a rare disease of young children and is speculated to represent a precursor of extramammary Paget disease.32,33 Lesions may result from a functional defect in melanogenesis or melanin distribution34 and are characterized by hypopigmented macules or barely palpable flat papules34 on the lower abdomen or along the milk lines in close proximity to the umbilicus. Immunobullous disease Chronic bullous disease of childhood,35 or linear immunoglobulin A (IgA) bullous dermatosis, is the

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Fig 2. Angiokeratoma corporis diffusum in a male patient with Fabry disease. (Courtesy of Edward Cowen, MD, MHSc.)

most common acquired immunobullous disease in children, with onset before 5 years of age. Pruritus may precede the development of clear to hemorrhagic vesicles and bullae superimposed on erythematous or normal skin (Fig 4). Bullae arranged in rosettes are described as ‘‘clusters of jewels.’’ Lesions are typically located around the umbilicus and on the buttocks, thighs, and the oral mucosa. Direct immunofluorescence (DIF) of skin biopsy material showing linear deposition of IgA at the basement membrane zone is diagnostic. Pemphigoid gestationis is an umbilical or periumbilical eruption of pruritic urticarial plaques (Fig 5, A and B) arising in the second or third trimester of pregnancy.36 This immunobullous disorder typically resolves within months postpartum. Histopathology reveals a subepidermal bulla containing eosinophils and neutrophils; DIF reveals linear C3 or IgG or both.3 There is a small risk of transient fetal blistering disease caused by the acquisition of maternal circulating antibodies. Pruritic urticarial papules and plaques of pregnancy (PUPPP) is the most common specific dermatosis of pregnancy36; it manifests as urticarial papules and plaques within striae on the abdomen (Fig 5, C ). In distinction to pemphigoid gestationis, PUPPP tends to spare the umbilicus and DIF on skin biopsy specimens is negative.

INFECTIOUS DISEASES AFFECTING THE UMBILICUS Neonates are particularly susceptible to infection of the umbilicus or omphalitis. Devitalized tissue of the umbilical cord stump provides a medium

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for bacterial growth, and thrombosed blood vessels act as a portal for microorganisms to enter the bloodstream.17,37 Omphalitis may present solely with periumbilical erythema or with a purulent discharge and surrounding induration, tenderness, and yellow crusting.3,5 It carries a mortality rate between 7% and 15%.38 Necrotizing fasciitis complicating omphalitis carries mortality rates of 60% to 85%.39 Suspicion for omphalitis warrants broad-spectrum antibiotic treatment5,40; surgical omphalectomy is indicated for resistant infections. Strongyloides stercoralis is an intestinal helminth most commonly seen in Latin American countries,41 but also in the southeastern United States. Larvae penetrate skin and superficial veins causing a serpiginous urticarial eruption known as ‘‘larva currens.’’ Patients may manifest periumbilical ‘‘thumbprint purpura’’ in the hyperinfection syndrome (Fig 6), which carries a poor prognosis and can lead to septic shock and acute respiratory failure.42-45 A skin biopsy specimen may aid in the diagnosis. Nematodes infesting humans as dead end hosts cause cutaneous larva migrans. The characteristic ‘‘creeping eruption’’ is often confined to the feet and lower extremities,46 but may also be found on the abdomen. Enteric fever, caused by Salmonella typhi and Salmonella paratyphi, is endemic in Africa, Asia, and Central and South America. It is acquired by eating food contaminated by fecal matter containing the organism. ‘‘Rose spots’’—light pink macules on the trunk and abdomen, with a predilection for the umbilicus47—are present in 5% to 30% of cases,48,49 along with fever, abdominal pain, hepatosplenomegaly, anorexia, and or constipation. Scabies manifests as erythematous papules, pustules, and grayish burrows with a predilection for the umbilicus, wrists, areolae, and finger web spaces. Diagnosis can be confirmed by detection of the mite, eggs, or scybala under light microscopy.50 Infrequently, condyloma lata of secondary syphilis may affect the umbilicus.51,52 These lesions appear as skin-colored to brown coalescing verrucous flat papules.

BENIGN TUMORS OF THE UMBILICUS Benign tumors of the umbilicus are described in Table I.3,53-56 Endometriosis manifests as a dome-shaped, bluish-black or reddish-brown umbilical nodule in \1% of patients.3,54 It can be painful, with cyclic variations in size and concurrent bleeding corresponding with menses.22 Dermoscopy reveals a regularly distributed homogenous

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Fig 3. A, Allergic contact dermatitis caused by nickel in a belt buckle. Instructing the patient in the use of commercially available dimethylglyoxime to test products for nickel will aid in avoidance of ongoing contact. B, Plaque psoriasis favoring the abdomen and umbilicus.

of the literature reported that SMJN was discovered before the diagnosis of the primary tumor in 41% of cases.60 Management requires obtaining a biopsy specimen for accurate diagnosis.

Fig 4. Linear immunoglobulin A bullous dermatosis. Note the annular arrangement of vesicles inferiorly. (Courtesy of Brooke Army Medical Center.)

red coloration fading to the periphery, with interspersed deeper red globular structures (‘‘red atolls’’).57 Treatment is by excision and screening for pelvic endometriosis.

MALIGNANT TUMORS OF THE UMBILICUS A number of malignant tumors may involve the umbilicus either primarily or secondarily (Table I).3,31,58 Sister Mary Joseph nodule (SMJN) describes an umbilical nodule representing tumor spread from an underlying intraabdominal or pelvic malignancy and is more common in women.59 Clinically, ulcerated or fissured nodules lined with fibrous tissue and irregular margins are noted (Fig 7).59 They can be painful or pruritic and exhibit a bloody, purulent, or serous discharge. One review

Foreign material Omphalith is an accumulation of keratinous and sebaceous material in the umbilicus, commonly resulting from poor hygiene.3 Young children may place foreign bodies, such as beads or peanuts, in the umbilicus. They typically present with the sudden onset of tenderness and induration. Gentle probing and removal is generally performed.

CUTANEOUS SIGNS OF SYSTEMIC DISEASE Several cutaneous signs of systemic disease61-68 are unique to this anatomic location (Table II). The ‘‘red umbilicus’’ was identified in 1 study that revealed that 9% of patients with cow’s milk protein intolerance (CMPI) had umbilical erythema.62 The erythema subsided on elimination of cow’s milk protein from the diet, but returned within 24 hours of rechallenge.62 An umbilical ecchymosis, referred to as a ‘‘Cullen sign,’’ is seen in 3% of patients with hemorrhagic pancreatitis.66 This nonspecific sign, representing a collection of blood in the umbilical subcutaneous tissue, may indicate retroperitoneal bleeding from other causes, such as ruptured ectopic pregnancy.66

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Fig 5. A, Pemphigoid gestationis. An unusual case mimicking pruritic urticarial papules and plaques of pregnancy (PUPPP) in that the urticarial plaques on the abdomen spare the umbilicus and preferentially involve striae. B, Pemphigoid gestationis. The same patient also developed acral bullae and the diagnosis was confirmed following skin biopsy. C, PUPPP. Note the prominent sparing of the umbilicus. (A and B, Courtesy David Wetter, MD. C, Courtesy of Brooke Army Medical Center.)

Table I. Primary neoplasms affecting the umbilicus

Fig 6. Periumbilical thumbprint purpura of Strongyloides hyperinfection in a South American patient who was chronically immunosuppressed on prednisone. (Courtesy Marc Grossman, MD.)

Rarely, skin involvement can be seen in primary systemic amyloidosis (amyloid light [AL] chain/AL amyloidosis), manifesting as umbilical petechiae, purpura, and ecchymoses.61 These findings result from deposition of amyloid in blood vessel walls, which can be demonstrated with special stains on a skin biopsy specimen.61

Benign Acrochordon53 Condyloma acuminata3 Dermatofibroma3 Desmoid tumor3 Endometriosis3,54 Epidermal nevus3 Epidermoid cyst3 Granular cell tumor3 Keloid3 Lipoma3 Melanocytic nevi (ie, blue, compound, intradermal, junctional, ‘‘special site’’)3,55 Neurofibroma3 Pyogenic granuloma Seborrheic keratosis3 Syringoma (including eruptive)56 Teratoma3 Umbilical polyp3 Malignant Adenocarcinoma of urachal elements3 Basal cell carcinoma3 Extra mammary Paget disease58 Malignant melanoma3 Mycosis fungoides Squamous cell carcinoma3,31

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Fig 7. Sister Mary Joseph nodule. (Courtesy of Brooke Army Medical Center.)

Table II. Umbilical and periumbilical cutaneous signs of systemic disease Systemic disease

Umbilical manifestation

Amyloidosis61

Umbilical petechiae, purpura, and ecchymosis Umbilical erythema Umbilical fistula, pyoderma gangrenosum, and ‘‘metastatic’’ disease Umbilical erythema Periumbilical ecchymosis/discoloration Dilatation of veins radially around umbilicus Periumbilical ecchymosis/discoloration

Cow’s milk protein intolerance62 Crohn disease63,64

Gangrenous bowel/peritonitis65 Hemorrhagic pancreatitis66 Portal hypertension67 Ruptured ectopic pregnancy68

In conclusion, familiarity with the clinical findings of various malformations and disease processes involving or localized to the umbilicus and periumbilical skin is important for prompt and accurate diagnosis and optimal patient care. REFERENCES 1. Mullany LC, Darmstadt GL, Khatry SK, LeClerq SC, Katz J, Tielsch JM. Impact of umbilical cord cleansing with 4.0% chlorhexidine on time to cord separation among newborns in southern Nepal: a cluster-randomized, community-based trial. Pediatrics. 2006;118:1864-1871. 2. Moore TC. Omphalomesenteric duct malformations. Semin Pediatr Surg. 1996;5:116-123. 3. Powell FC, Su WP. Dermatoses of the umbilicus. Int J Dermatol. 1988;27:150-156. 4. Disorders of the umbilicus in infants and children: a consensus statement of the Canadian Association of Paediatric Surgeons. Paediatr Child Health. 2001;6:312-313. 5. Kliegman R, Nelson WE. Nelson textbook of pediatrics. Philadelphia (PA): Elsevier/Saunders; 2011.p. lxvii, 2610. 6. Cox GA, Chan I, Lloyd J, Witherow RO, Leonard JN. Urachal sinus presenting as periumbilical dermatitis. Br J Dermatol. 2007;157:419-420. 7. Ashley RA, Inman BA, Routh JC, Rohlinger AL, Husmann DA, Kramer SA. Urachal anomalies: a longitudinal study of urachal remnants in children and adults. J Urol. 2007;178:1615-1618. 8. Hoyme HE, Higginbottom MC, Jones KL. The vascular pathogenesis of gastroschisis: intrauterine interruption of the omphalomesenteric artery. J Pediatr. 1981;98:228-231.

Clinical term

Comment

Rare reports of papular eruptions Red umbilicus Lesions may be contiguous with bowel or separated

Cullen sign Caput medusae Associated with rare hemorrhagic complications Cullen sign

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44. von Kuster LC, Genta RM. Cutaneous manifestations of strongyloidiasis. Arch Dermatol. 1988;124:1826-1830. 45. Ronan SG, Reddy RL, Manaligod JR, Alexander J, Fu T. Disseminated strongyloidiasis presenting as purpura. J Am Acad Dermatol. 1989;21:1123-1125. 46. Heukelbach J, Feldmeier H. Epidemiological and clinical characteristics of hookworm-related cutaneous larva migrans. Lancet Infect Dis. 2008;8:302-309. 47. Geddes AM. Imported infections. Unexplained fever. Br Med J. 1974;4:397-398. 48. Litwack KD, Hoke AW, Borchardt KA. Rose spots in typhoid fever. Arch Dermatol. 1972;105:252-253. 49. Geddes AM. A new look at infectious diseases: enteric fever, salmonellosis, and food poisoning. Br Med J. 1973;1:98-100. 50. Griffin JR, Newman CC. Clinical, dermatoscopic, and microscopic findings of infestation with Sarcoptes scabiei var hominis. Mayo Clin Proc. 2011;86:e47. 51. Fiumara NJ. Unusual location of condyloma lata. A case report. Br J Vener Dis. 1977;53:391-393. 52. Tham SN, Lee CT. Condyloma latum mimicking keratoacanthoma in patient with secondary syphilis. Genitourin Med. 1987;63:339-340. 53. Banik R, Lubach D. Skin tags: localization and frequencies according to sex and age. Dermatologica. 1987;174:180-183. 54. Jaime TJ, Ormiga P, Leal F, Nogueira OM, Rodrigues N. Umbilical endometriosis: report of a case and its dermoscopic features. An Bras Dermatol. 2013;88:121-124. 55. Mason AR, Mohr MR, Koch LH, Hood AF. Nevi of special sites. Clin Lab Med. 2011;31:229-242. 56. Ghanadan A, Khosravi M. Cutaneous syringoma: a clinicopathologic study of 34 new cases and review of the literature. Indian J Dermatol. 2013;58:326. 57. De Giorgi V, Massi D, Mannone F, Stante M, Carli P. Cutaneous endometriosis: non-invasive analysis by epiluminescence microscopy. Clin Exp Dermatol. 2003;28:315-317. 58. Vincent J, Taube JM. Pigmented extramammary Paget disease of the abdomen: a potential mimicker of melanoma. Dermatol Online J. 2011;17:13. 59. Khan AJ, Cook B. Metastatic carcinoma of umbilicus: ‘‘Sister Mary Joseph’s nodule’’. Cutis. 1997;60:297-298. 60. Dubreuil A, Dompmartin A, Barjot P, Louvet S, Leroy D. Umbilical metastasis or Sister Mary Joseph’s nodule. Int J Dermatol. 1998;37:7-13. 61. Kumar S, Sengupta RS, Kakkar N, Sharma A, Singh S, Varma S. Skin involvement in primary systemic amyloidosis. Mediterr J Hematol Infect Dis. 2013;5:e2013005. 62. Iacono G, Di Prima L, D’Amico D, Scalici C, Geraci G, Carroccio A. The ‘‘red umbilicus’’: a diagnostic sign of cow’s milk protein intolerance. J Pediatr Gastroenterol Nutr. 2006;42: 531-534. 63. Veloso FT, Cardoso V, Fraga J, Carvalho J, Dias LM. Spontaneous umbilical fistula in Crohn’s disease. J Clin Gastroenterol. 1989;11:197-200. 64. McLelland J, Griffin SM. Metastatic Crohn’s disease of the umbilicus. Clin Exp Dermatol. 1996;21:318-319. 65. Chang YT, Lin JY. Red umbilicus as a clinical manifestation in a 19-month-old girl with typhoid colonic perforation. Kaohsiung J Med Sci. 2007;23:80-83. 66. Parikh RP, Upadhyay KJ. Cullen’s sign for acute haemorrhagic pancreatitis. Indian J Med Res. 2013;137:1210. 67. Qureshi WA, Harshfield D, Shah H, Netchvolodoff C, Banerjee B. An unusual complication of paracentesis. Am J Gastroenterol. 1992;87:1209-1211. 68. Linkletter AM, Moore FC. Cullen’s sign in ruptured ectopic pregnancy. Can Med Assoc J. 1961;85:1003-1004.

Umbilical and periumbilical dermatoses.

The umbilicus may be the site of congenital and acquired malformations and may harbor clinical clues to the diagnosis of potentially fatal inherited d...
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