SKIN & MISCELLANEOUS DISORDERS

SKIN & MISCELLANEOUS DISORDERS

A. Pilonidal Sinus Disease

Pilonidal sinus disease (PSD) is the result of a chronic inflammatory reaction of the subcutaneous tissue of the low back from midline skin orifices that ingest hair shafts in hirsute buttocks. PSD arises from hair follicles in the skin, appears after puberty and affects mostly males adolescents. Male sex, adolescence and a familial disposition are associated with the development of PSD. Minor local trauma (Jeep Disease' in military personnel) and overweight are the most important factors for development of symptomatic PSD. Primary closure after excision of PSD has frequently been complicated by wound breakdowns. Sepsis and hematoma formation are the main causes of wound breakdowns. PSD is best treated in the acute situation with follicle removal and lateral drainage. The method is suited to outpatient management and gives minimal disability and good long-term control. In the chronic situation conservative therapy  (meticulous hair control by natal cleft shaving, perineal hygiene, and limited lateral incision/drainage for abscess) effectively controls PSD in the nonoperative outpatient setting while promoting near-normal work status preferred over excisional operations. 

B. Ingrown Toe Nails

Onychocryptosis is the medical term use to describe ingrown toe nails (ITN). Congenital hyperplasia of the nail bed leads to this condition in infants and children. Adolescent and athletes are susceptible to develop ITN. Other conditions associated with ingrown nails are hallux valgus, claw toes, gout, diabetes, arthritis, or fungus infections of the nails. Initial management should be conservative inserting cotton wool pledgets moistened with an antiseptic under the ingrowing nail edge along with oral antibiotics if there are signs of infection. Insertion of the pledget under anesthesia improves the results and chronicity does not adversely affect the outcome though the procedure is time consuming. Other alternatives are segmental total nail avulsion, nail edge excision, nail matrix phenol cauterization or wedge resections. Total nail avulsion and nail edge excision carries the highest recurrence rate. Nail matrix phenolization is an effective short procedure that can be done under the presence of an infection with a low recurrence rate. Radical wedge resection is simple, has a low recurrence rate, leaves patients with an intact, pain-free, cosmetically acceptable nail-bearing toe, and permits the wearing of normal shoes within a short period. In children conservative treatment is more promising than in adults. If unsuccessful the next reliable surgical approach is wedge resection. Attentions to hygiene and always cutting nails transversely are important preventing factors.

C. Ganglion Cysts

Ganglion cysts are synovial cysts that appear in the wrist or foot after minor trauma or stress. Most occur in the dorsal wrist area. Presence of a colorless to pale-yellow gelatinous material in the aspirate is pathognomonic of ganglion cysts. FNA smears are monotonous showing abundant mucoid material, single cells resembling histiocytes, a few tight clusters of cells, some collagen fibers, and some red blood cells with altered shapes. Serial microscopic studies have shown evidence of a one way valve like system between the affected joint and the ganglion. Diagnosis is physical, though ultrasound findings (cysts with a mean diameter of 1.4 cm and projection into the joint or tendon) are of help. Management consists of excision. Recurrence rates are high ranging between 10 and 35%. Ganglion cysts in the volar aspect of the wrist have a higher incidence of postoperative complications (nerve and radial artery damage). Intralesional injection of hyaluronidase has been found a safe, fast, well accepted and cost-effective alternative to surgical excision.

D. Anal Warts

Warts in the perianal region of prepubertal infant or child most commonly are the result of human papilloma virus (HPV) infestation. Main clinical manifestations of anal warts are cauliflower-like Condylomata Acuminata that usually involves moist surfaces, keratotic and smooth papular warts usually on dry surfaces, and subclinical flat warts that can be found on any mucosal or cutaneous surface. Mode of transmission can occur from an infected maternal birth canal (perinatally), by autoinoculation or heteroinoculation from common hand warts, through sexual abuse and possibly indirect transmission via fomites. Often, the mode of transmission is unknown. HPV-DNA typing is a useful technique that helps identify the genital types involved (6 or 11, 16 or 18) alerting the physician to proceed with a careful assessment for sexual abuse. Predisposing factors for anal warts include social problems, lack of hygiene, promiscuity, diabetes and ammoniacal erythema. The appearance of the papilloma is diagnostic. Management includes cytotoxic agents (podophyllin, podophyllotoxin and fluorouracil), destructive procedures (scissor excision, cryotherapy, electrocautery, and laser photocoagulation) and recently topical interferon hydrogel. In case of transmission by sexual abuse child protection is warranted.

E. Gynecomastia

Gynecomastia refers to abnormal breast enlargement in males. In children, gynecomastia can be classified as simple pubertal, pathological, general obesity and pectoral muscle hypertrophy. Most cases of gynecomastia are simple pubertal associated to a transient or permanent disturbance in steroid hormone physiology occurring when the male breast is exposed to a decreased ratio of androgen to estrogen. Pubertal gynecomastia can be managed non-operatively since breast enlargement start one year after the onset of puberty and subside two years later. Pathological gynecomastia is associated with drug use (steroids, digitalis, spironolactone, and marijuana), chronic liver disease or malignancy (Leydig cell tumor of the testis). General obesity is associated with fat deposition surrounding breast tissue that lends itself to weight reduction. Diagnosis is by history and physical exam. Routine endocrine work-up is not cost effective. Persistent pain, uncertain diagnosis and cosmetic reasons (embarrassment or distress) are the major reasons for operation. Subcutaneous mastectomy through a periareolar incision gives the best cosmetic results. Common late postoperative sequelae are inverted areolae, hypopigmentation and hypoesthesia of the areolar region.

F. Bites & Stings

It is estimated that more than 1.5 million bites occur yearly in the United States. Most of them within the home setting in children younger than five years of age. When managing bites in children three general concepts must be observed: 1) Determine if the attacking animal has injected venom into its victim. For this to occur the history must provide the exact identification of the animal and type of venom. Once identified an specific antivenom can be utilized. 2) All bites wounds are considered contaminated wounds. This includes rabies and tetanus. Prophylaxis in either case should be provided. Furthermore the bite can produce significant tissue loss and destruction. Initial management should include thorough mechanical cleansing and debridement along with systemic antibiotics. 3) An assessment should be made of the long-term outlook for both disfigurement and loss of function of the wound. Wounds of the face do fairly better with primary closure no matter the inciting initial agent. Significant loss of tissue might need conservative management followed by reconstruction when adequate granulation tissue appears. Ischemic extremity from compartment syndrome might need fasciotomy. Fortunately we don't see snake bites in Puerto Rico. Few cases of spider bites has been reported. Dog bites (pit bull) are the most common bite seen in the emergency room affecting the extremity or face of young infants. Cat bites or scratch can produce a febrile condition associated with lymphadenopathy. Human bites carry the risk of HIV and hepatitis infestation.

The insects that inflict more venous stings than any other in children are the bees and ants. Stings from bees and wasps produce a local tissue reaction with a wheal and flair. Symptoms develop within twenty minutes of the sting and include urticaria, syncope and respiratory distress. Most serious sequelae is anaphylaxis which occur when the child has been previously inoculated. More than 500 stings are needed to cause death in a child. Management is local and systemic. The venom can be removed if the event has less than 20 minutes. Cold compresses will reduce pain associated with the sting and baking soda helps with the itching. Systemic support includes airway control, alpha agonists medication, inhaled beta agonist for bronchospasm and calcium for muscle spasms. Best prophylaxis is reducing exposure. Fire ants' sting can produce edema, pruritus, erythema, pain and burning with a characteristic wheal. Wound is cleaned with soap and water. Rarely systemic management is needed.

G. Perianal Abscess

Perianal abscess is a not so rare condition seen almost exclusively in infants less than two years of age. Most cases are seen in males infants. The infant presents with a history of increasing irritability, fever, erythema and induration of the perianal skin. In a period of 48 to 72 hours the area becomes fluctuant. Oral antibiotics are ineffective in controlling the infectious process. It is theorized that a perianal abscess arises from a developmental anomaly in the deep crypts of Morgagni which trap bacterias initiating a cryptitis that proceed to a perianal abscess. This abscess may open or not to become later a fistula in ano. Gut derived organisms are isolated from most cases of perianal abscess. Most abscess are located laterally equally divided between right and left. Perianal abscesses in children are best treated by incision, drainage and systemic antibiotics. A proportion of patients with perianal abscess later develop a fistula in ano. This fact has led some researchers to propose that primary treatment of perianal abscess in childhood involve a careful search for a coexisting fistula and treatment of this by fistulotomy. Long term recurrence is very rare. Recurrence should prompt a search for associated disorders such as Crohn's, immunodeficiency and autoimmune neutropenia.

H. Keloids

Excessive scarring is deposition of collagen out of the range of normal after surgery. Two types of excessive scarring seen in children and adults are hypertrophic scars and keloids. Hypertrophic scars are slight elevation of the scar restricted to the boundaries of the original wound, regress with time and rarely cause functional impairment. Hypertrophic scars rarely need to be removed. Keloids extend beyond the borders of the original wound, cause functional impairment, contain mast cells that release histamine and produce pruritus. Familial cases have been seen. Keloids do not regress over time and recur after surgical excision. Keloids contain large thick collagen fibers composed of numerous fibrils closely packed together. In contrast hypertrophic scars exhibit modular structures in which fibroblastic cells, small vessels, and fine, randomly organized collagen fibers are present. Earlobe piercing is the main etiological factor of earlobe keloids. Keloids removal is indicated in cases of functional impairment of a joint or for cosmetic reasons. Excision with intraoperative local injection of triamcinolone or alpha-2b interferon has produced some therapeutic advantage in severe cases. Early single postoperative fraction radiotherapy claims effectiveness in reducing keloid recurrences.

I. Felons

A felon is defined as a painful fusiform abscess (swelling) in the distal fat pad of the phalanx with increased tenderness over the flexor aspect and erythema (redness and cellulitis) distributed uniformly along the entire flexor aspect of the pulp space. Felon is very different from a paronychia. Paronychia is a more localized painful swelling on the extensor aspect of the distal digit located at the proximal extent of the sulcus. Initial management consists of antibiotics, warm soaks and elevation of the affected extremity. If the child does not improve rapidly with medical treatment, surgical incision and drainage are indicated to avoid involvement of the bony distal phalanx due to rapid extension of the infections process through synovial-lined spaces. Felons are managed with digital anesthetic block and incision along the ulnar aspect of the digit to produce drainage of the closed space infection. The incision is made along the ulnar aspect of the phalanx to avoid the possibility of a painful scar on the radial side of the phalanx which is the pinching side of the distal phalanx.  Cultures are routinely taken during surgery. Staphylococcus aureus is a common offending organism. Penrose drain for 48 hours is recommended.

J. Latex Allergy

Allergy to natural rubber latex is an increasing common condition in both children and health care workers. Almost 20 to 40% of children with spina bifida are allergic to latex. Other affected persons are health care workers, latex industry workers, immune compromised individuals, children with bladder exstrophy, anorectal anomalies, and persons with positive risk factors such as multiple surgical procedures. Chemical additives in latex gloves can cause an irritant or allergic contact dermatitis. Latex proteins are responsible for most of the immediate IgE-mediated hypersensitivity allergic reactions. Symptoms range from rhinitis, conjunctivitis and urticaria to intraoperative anaphylaxis and death. Skin prick testing with natural rubber latex and glove tests are safe diagnostic procedure. In children with spina bifida significant and independent risk factors identified for latex sensitization are multiple interventions and higher levels of total serum IgE. The only currently available treatment is complete avoidance of latex. For children with known history of latex allergy premedication with antihistamines and steroids is in order.

K. Nevus Sebaceous

Nevus sebaceous of Jadassohn is a congenital hamartomatous skin lesion occurring mostly on the scalp, face and neck. At physical exam the lesion is well circumscribe with a yellow-orange smooth plaque appearance. Borders become irregular with puberty and hormonal changes. Malignant transformation has been reported in 10% of cases occurring only after puberty. The nevus sebaceous can transform into a basal or squamous cell carcinoma. The lesion will not go away spontaneously. It is uncommon for malignancy to develop in a sebaceous nevus before puberty. Due to the risk of malignant transformation and the difficulty in follow-up of these children with time, early complete excision for prophylaxis is recommended in cases of nevus sebaceous. Excision must encompassed clean deep and lateral margins of resection to be effective. Large lesion will benefit from use of tissue expanders.

L. Polydactylism

Polydactylism (accessory finger or toe) is one of the most common congenital anomaly of hands or feet in children. The prevalence of polydactylism is almost two cases for every each 1000 born alive newborns. Familial occurrence is associated in almost 20% of cases with variable gene penetrance. Polydactylism of the hand is more common than in the foot. In the hand the ulnar accessory finger predominates. A few cases undergo traumatic intrauterine amputation, a condition known as rudimentary polydactylism. Different genes are involved in the pathomorphogenesis of postaxial polydactylism. Polydactyly may be preaxial (medial, thumb side) or tibial (hallux-side), postaxial (lateral) or ulnar (side of the little finger or toe), and central (middle fingers or toes). The duplication may appear at the distal and medial phalanges or at the whole digit. Surgical amputation of the affected finger or toe is indicated for cosmetic reasons or for functional disturbances in wearing shoes respectively. Careful clinical and radiographic evaluation should be made prior to treatment to achieve good functional and cosmetic results. Polydactylism can be diagnosed prenatally and when isolated is associated with good perinatal outcome. Primary suture ligation of accessory digits in infancy can be associated with later development of neuroma in the stump. Identification and high transection of the accessory digital nerve is essential in the treatment of pedunculated supernumerary digits.

M. Giant Pigmented Nevus

Giant Pigmented Nevus (GPN) refers to a congenital melanocytic nevi characterized by a diameter of 20 cm or greater in adulthood, or a lesion occupying greater than 2% of the body surface area of an infant or child. Generally, melanocytic nevi is solitary, but smaller satellite lesions may be present within a GPN. The mode of inheritance of GPN is probably multifactorial with a 2:1 female predominance. The lesion is typically brown pigmented, varies in size and shape, contains hair and can be found anywhere on the face, neck, trunk or extremity of the child. GNP extensively involving the extremities can result in reduced growth of the affected limb. GPN has a higher incidence of malignant transformation (melanoma formation) due to the increase number of nevus cells at risk for such transformation. Lifetime incidence of malignant transformation is four to 6%. This capacity of GPN to develop into malignant melanoma is the main reason for opting for prophylactic surgical excision as treatment of choice. Tissues expansion is the primary modality for excision and reconstruction in the face and scalp since it can be started early in life and repeated as required. For the trunk excision with abdominoplasty or skin graft is preferred. In the extremity excision and skin grafting is best option. Dermabrasion or laser therapy cannot ensure complete removal of nevus cells.

N. Chronic Granulomatous Disease of Childhood

Chronic Granulomatous Disease (CGD) is a very rare inherited primary immunodeficiency disease of childhood. Due to defective respiratory burst, phagocytic cells of children with CGD are not able to kill certain bacterias and fungi despite normal chemotaxis and phagocytosis. The proper functioning of the NADPH oxidase of the phagocytic cell is impaired. Children with CGD are rendered susceptible to infection by a group of catalase positive microorganisms due to the inability of phagocytic cells to reduce molecular oxygen and create reactive oxygen metabolites necessary for normal intracellular killing. Catalase positive organisms affecting children with CGD include staph aureus, Escherichia Coli, Serratia, Salmonella, Candida and Aspergillus. Children with CGD present clinically with recurrent abscess involving skin, soft tissue (most common site), lymph nodes, lung, bone and liver that responds poorly to antibiotics needing surgical drainage. Palisading granulomas with central necrosis can be seen in histologic samples. Though four different types of CGD have been described, most cases are X-linked defects. Diagnosis of CGD is made by showing the inability of neutrophils from the patient to undergo respiratory burst after phagocytosis (NBT Test). Fungal infections account for most deaths. Children with CGD should receive long-term Trimethoprim-Sulfamethoxazole prophylaxis. Gamma interferon reduces the number of infection requiring hospitalizations.

O. Congenital Melanocytic Nevus

Skin nevus found in infants and children can be a source of concern to both parents and physician dealing with pediatric patients. Congenital melanocytic nevus (CMN) is a characteristic pigmented nevus with mild raised borders and a strong skin discoloration caused by melanin deposits. Incidence of CMN is 0.2% with more than 90% small nevi (< 1.5 cm). Congenital melanocytic nevus may cause cosmetic defects and represent a risk of malignant transformation, namely melanoma. The incidence of developing melanoma is associated with location and size of the CMN; those identified in the face and neck or covering large areas of the body (larger than 5 cm) has a higher incidence of malignant transformation. Not always there is a correlation between pre and postoperative diagnosis after prophylactic excision of a nevi. Digital videomicroscopy using polarize light can help increase the diagnostic yield of CMN. A high index of suspicion for cutaneous melanoma is needed by clinicians assessing melanocytic lesions in children and adolescents for early diagnosis taking into consideration change in color, growth rate, and bleeding, For large lesions and those in exposed areas of the body, surgical excision is the treatment of choice. Medium and small lesions can be managed with Ruby laser treatment. Ruby laser treatment does not result in scarring, mutilation, or functional impairment.

P. Hyperhydrosis

Primary idiopathic hyperhydrosis is the occurrence of perspiration in excess of that required for body cooling. Secondary hyperhydrosis can be the result of conditions such as hyperthyroidism, severe obesity, pheochromocytoma or anxiety. Primary hyperhydrosis can involve the hands, axillae, or trunk. Sweat is secreted by eccrine glands innervated by sympathetic cholinergic fibers. Eccrine glands consist of tubules which extend deeply in the dermis becoming coiled and surrounded by myoepithelial cells. The majority of children seek help due to palmar hyperhidrosis. The condition can be socially debilitating causing psychological and emotional upset. Conservative management consists of antiperspirants, iontophoresis and systemic medication (anticholinergics). If the condition does not improve with conservative measures, surgical sympathectomy is indicated. The aim of surgery is to disrupt the sympathetic supply to palms by destroying relevant ganglions in the upper thoracic sympathetic chain (T2 and T3). The sympathectomy can be done supraclavicular, limited posterior thoracotomy, transaxillary thoracotomy or using thoracoscopy. Results are excellent. The most serious and uncommon complication is Horner's syndrome.

Q. Preauricular Tags

Preauricular skin or chondrocutaneous branchial remnant is not a rare finding in infants. Preauricular tags occur in five of every 1000 live births. They are congenital malformations that should be taken care for aesthetic reasons. Most preauricular tags are from branchial origin and contain cartilage. Excision is indicated after the age of six months under general endotracheal anesthesia as an ambulatory procedure. Cosmetic results are very good. Less than 5% of all preauricular tags are associated with other malformations of the ear/face region, most of them conductive type hearing impairment. Physical examination under such circumstances provides the first clue of a hearing disorder. Possibility increases if there is familial history of hearing impairment. When in doubt, audiometry is indicated. Another association with preauricular tag is the presence of a urinary tract anomaly which occurs in 8% of all cases. Such renal anomaly includes hydronephrosis caused by ureteropelvic obstruction or vesicoureteric reflux, and horse-shoe kidney. Some workers recommend that urinary tract ultrasonography be conducted in the routine assessment of infants with preauricular tags and multiple congenital anomalies. Specific disorders associated with tags that increase the yield of finding a renal disorder includes CHARGE association, Townes-Brocks syndrome, branchio-oto-renal syndrome, Nager syndrome, and diabetic embryopathy. Routine renal ultrasonography is not necessary in the evaluation of children with isolated preauricular tags.