History

Fact Explanation
Often presents with parents Anxious parents, present to a healthcare provider, after noticing an unusual mark on the child's skin.
References

Examination

Fact Explanation
Nevi appearing at birth/or in neonatal period: congenital melanocytic nevi Features: brown or black hyper pigmented lesions, flat or elevated surfaces, size appears small less than 2cm but a giant congenital melanocytic nevus will be larger than 20cm. Common sites are lower trunk, upper back, head and extremities. There is an localized increase melanocytes, in these patients.[1] The cause is a genetic factor resulting in abnormal growth of melanoblasts (precursors of melanocytes). [2] There is increased risk of melanoma( specially giant type) at the median age of 7 years.[7] This can be associated with leptomeningeal involvement resulting in hydrocephalus, seizures and mental retardation.[7]
Nevi appearing at birth/or in neonatal period: dermal melanocytosis (Mongolian spot) Features: bluish pigmented macules, lumbosacral area. This condition is caused by melanocytes residing in the dermis. [1],[3]
Nevi appearing at birth/or in neonatal period: nevus of Ota Features: unilateral blue-brown facial macule, often involves the ipsilateral sclera, palate,Nasal mucosa and has a raised surface.
Nevi appearing at birth/or in neonatal period: nevus of It Features: involves the upper back and shoulder and has a mottled surface. There is mid dermal melanocytosis.[7]
Present at birth or in early life: epidermal nevi Features: found on trunk and limbs, linear pattern of hyper pigmentation, scaling and unilateral. There is hamartomatous growth in the epidermis.It can include other adenaxal skin structures as well. [12] There may be associated developmental abnormalities. [7]
Nevi present in infancy: nevus sebaceous Features: well circumscribed plaques, linear arrangement, hairless, yellow and waxy, become verrucous (wartlike) with age and appear on the scalp, face and upper body. Epidermal naevi are hamartomas of the epithelium. Cell types include keratinocytes, the sebaceous gland, and eccrine and apocrine glands.[3]
Nevi present in infancy and childhood: speckled lentiginous nevus Features: brown colored macules, variable size, can occur any where in the body. There are melanocytes at dermo epidermal junction.[7]
Nevi acquired in childhood: blue nevus Features: dark blue colour in color, found on the scalp, face, hands and feet, small and the surface is flat or elevated. The blue color is due to the pigment cells (melanocytes) being situated deeper in the skin.[1],[7]
Nevi acquired in childhood: spitz nevus Features: solitary, pink or brown papule, about 1cm diameter, common sites are the face or a limb, initial growth can be rapid. This is a type of melanocytic nevus. [4] Although they are benign, they have some histopathologic features that overlap with melanoma.[7]
Nevi acquired in childhood: halo nevus Features: a rim of pale skin around the nevus, pink or less pigmented, the nevus fades disappears with time. Destruction of the nevus is an immune mediated process. A circulating antibody against the melanocytes destroys the nevus. [7]
Nevi acquired in childhood or in adolescents: atypical nevi Features: size is greater than 5 mm diameter, poorly defined borders and varies in color. There is a familial type (One or more first-degree or second-degree relative with malignant melanoma; more than 50 nevi with some being atypical)[6] and a sporadic type.
Vascular Nevi: hemangioma Features: appear as telangiectatic macule, skin ulceration, appears on head, neck, trunk and extremities, bright-red in the superficial variety, well-defined nodules that is slightly raised, deep seated hemangiomas are bluish nodules. [9]
Vascular nevi: nevus flammeus Features: flat lesion, dark red to purple, apparent at birth, does not fade over time, may also develop varicosities, nodules, or granulomas. [8] Capillary ectasias involve both superficial capillaries deeper blood vessels of the dermis and subcutaneous tissue.[9] They may be associated with Sturge-Weber syndrome( seizures, mental retardation,Hemiplegia and glaucoma.[9]
Vascular nevi: nevus simplex Features: occur over the eyes, scalp, and neck, and blanch when compressed, are bilateral and symmetrical.[8] Telangiectasias in the dermis is the cause for this. [8]
References
  1. WELLER Richard et al ed.Clinical dermatology.4th ed.Oxford:Blackwell publishing Ltd.2008.pp293-298
  2. VIANA AC, GONTIJO B, BITTENCOURT FV. Giant congenital melanocytic nevus An Bras Dermatol [online] 2013, 88(6):863-878 [viewed 26 May 2014] Available from: doi:10.1590/abd1806-4841.20132233
  3. RYAN E, WARREN L. Birthmarks--identification and management. Aust Fam Physician [online] 2012 May, 41(5):274-7 [viewed 26 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/22558616
  4. TLOUGAN BROOK E., ORLOW SETH J., SCHAFFER JULIE V.. Spitz Nevi. JAMA Dermatol [online] 2013 March [viewed 26 May 2014] Available from: doi:10.1001/jamadermatol.2013.1124
  5. BOMM L, CARVALHO RV, LIMA FF, OLIVEIRA LN, TOLSTOY F, LOBãO D. Retrospective analysis of melanocytic lesions in children at the National Cancer Institute-RJ. An Bras Dermatol [online] 2014 Apr, 89(2):369-71 [viewed 26 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/24770528
  6. SILVA JH, DE Sá BC, DE ÁVILA AL, LANDMAN G, NETO JP. Atypical mole syndrome and dysplastic nevi: identification of populations at risk for developing melanoma - review article Clinics (Sao Paulo) [online] 2011 Mar, 66(3):493-499 [viewed 27 May 2014] Available from: doi:10.1590/S1807-59322011000300023
  7. KLIEGMAN, Robert M. et al ed.Nelson textbook of PEDIATRICS.19 th ed.vol 2.Philadelphia:Saunders - Elsevier.2011.pp.2231-2235
  8. MCLAUGHLIN MR, O'CONNOR NR, HAM P. Newborn skin: Part II. Birthmarks. Am Fam Physician [online] 2008 Jan 1, 77(1):56-60 [viewed 29 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/18236823
  9. WIRTH FA, LOWITT MH. Diagnosis and treatment of cutaneous vascular lesions. Am Fam Physician [online] 1998 Feb 15, 57(4):765-73 [viewed 29 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/9490999

Differential Diagnoses

Fact Explanation
Melanoma Features such as recent enlargement,change in pigmentation,alterations in shape and surface,inflammation,itch, ulceration and bleeding can be in the presentation.[1]
Ephelides Freckles are brownish macular of less than 3 mm in diameter. They become darker in skin that is exposed to ultraviolet radiation (UV), People with red hair, or fair skin develop this condition.[1],[2]
Lentigines simplex These are Brown-black macule with irregular edges .Their size is variable size(small to more than 10 mm)
Smooth muscle hamartomas This is a differential diagnosis considered in congenital melanocytic nevi. This is observed at birth.there will be pigmented plaques with hypertrichosis on trunk or limbs. Contractions can be elicited by stroking on the surface.[3]
Cafe au lait pacthes They are light brown colored, oval macules that develop in the first year of life.They are common in Neurofibromatosis Type 1 (together with axillary freckling, skin neurofibromas) but can also be seen in type 2 (associated with acoustic neuromas, meningiomas and gliomas). [Cafe au lait patches are a differential diagnosis for nevi such as congenital melanocytic nevi][3]
References
  1. WELLER,Richard.et al ed.Clinical dermatology.4th ed.Oxford:Blackwell publishing Ltd.2008.pp293-298
  2. Melanoma and pigmented lesions.Clinical Knowledge summaries.NICE[online].National Institute for Health and Care Excellence.2011[viewed 26 May 2014].Available form:http://cks.nice.org.uk/melanoma-and-pigmented-lesions
  3. KLIEGMAN, Robert M. et al ed. Nelson textbook of PEDIATRICS.19th ed.vol 2. Philadelphia:Saunders - Elsevier.2011.pp.2231-2235

Investigations - for Diagnosis

Fact Explanation
Biopsy of the lesion If the suspicion of melanoma is present, then a biopsy and histology should be done.[1,2] Excision in primary care setting should be avoided.
References
  1. Melanoma and pigmented lesions.Clinical Knowledge summaries.NICE[online].National Institute for Health and Care Excellence.2011[viewed 26 May 2014].Available form:http://cks.nice.org.uk/melanoma-and-pigmented-lesions
  2. KLIEGMAN, Robert M. et al ed.Nelson textbook of PEDIATRICS.19 ed.vol 2.Philadelphia:Saunders - Elsevier.2011.pp.2231-2235

Investigations - Screening/Staging

Fact Explanation
Lymph node biopsy This is done in metastatic melanoma.[1]
Serum LDH This is a marker and an indicator of prognosis [Note:The variability in specificity and accuracy can be problematic in using LDH but it is the most used marker up to now.[2]
Imaging Ultrasound scans, CT scans and PET scans help to identify metastatic deposits in areas which are inaccessible to biopsy. [1]
MRI In giant congenital melanocytic nevi in head and midline lesions, MRI demonstrates leptomeningial involvement. [3]
CSF cytology This is also done to detect malignancy involving the brain and meninges.[3]
References
  1. THOMPSON JF, SCOLYER RA, KEFFORD RF. Melanoma - a management guide for GPs. Aust Fam Physician [online] 2012 Jul, 41(7):470-3 [viewed 27 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/22762064
  2. VEREECKEN P, CORNELIS F, VAN BAREN N, VANDERSLEYEN V, BAURAIN JF. A synopsis of serum biomarkers in cutaneous melanoma patients. Dermatol Res Pract [online] 2012:260643 [viewed 27 May 2014] Available from: doi:10.1155/2012/260643
  3. KLIEGMAN, Robert M. et al ed.Nelson textbook of PEDIATRICS.19th ed.vol 2.Philadelphia:Saunders - Elsevier.2011.pp.2231-2235

Management - General Measures

Fact Explanation
Educate the parent/patient(older children and adolescents) Nevi are benign conditions.Some can develop in to malignancy, so attention to changes of the nevi are important. (recent enlargement, satellite lesions,change in pigmentation,alterations in shape and surface,inflammation,itch, ulceration and bleeding)[1] In this situation,referral to a specialist is important.[2] Educate the parent of photographic mole mapping and inspect for changes every 3-4 months.(Specially conditions such as atypical nevi)[3]
Complete skin examination every 6-12 months This is done in patients with increased risk of melanoma such as atypical nevi, and congenital melanocytic nevi.[3]
Avoiding sunburn Parent should be advised on the importance of sun protection in conditions with increased risk of melanoma(atypical nevi).[3] Ultraviolet light can increase the risk of nevi formation as well as malignant transformation. Advice the patient on avoiding sun burn by; using clothing to cover the skin, using a broad-spectrum sunscreen.[2]
References
  1. WELLER,Richard.et al ed.Clinical dermatology.4th ed.Oxford:Blackwell publishing Ltd.2008.pp293-298
  2. Melanoma and pigmented lesions.Clinical Knowledge summaries.NICE[online].National Institute for Health and Care Excellence.2011[viewed 26 May 2014].Available form:http://cks.nice.org.uk/melanoma-and-pigmented-lesions
  3. KLIEGMAN, Robert M. et al ed.Nelson textbook of PEDIATRICS.19 ed.vol 2.Philadelphia:Saunders - Elsevier.2011.pp.2231-2235

Management - Specific Treatments

Fact Explanation
Managing Melanociyic Nevi: Surgical removal Most nevi are benign. In these instances follow up can be advised. [6] Reasons for early excision are: cosmetic unacceptability, parents wishes[6], risk of melanoma( in giant congenital nevi, atypical nevi)[6] and change in the nevus.[4] Surgical removal can be done by using a scalpel or by the electrosurgical procedures[2]
Cryotherapy This is used to induce necrosis by altering temperatures in which cells can't sustain its functions.
CO2/ Erbium YAG laser therapy A technique of laser resurfacing.
Retinoic acid/Salicylic acid These drugs induce keratinolysis and aids in controlling pruritus and scaling in epidermal nevi. [6]
Refferal Urgently refer (within 2 weeks) to a dermatologist, plastic surgeon, or other suitable specialist with experience of melanoma diagnosis if: the lesion is suggestive of a malignant melanoma, new nodules with pigmentation, vascularity and nail changes. Routinely refer for risk estimation, (people with increased risk are: those with giant congenital pigmented nevi, a family history of 3 or more patients with melanoma, more than 100 nevi, atypical nevi. [5]
Management of vascular nevi: flash-lamp pumped pulsed dye laser (FPDL) Nevus Simplex: Watchful waiting and if therapy needed, flash-lamp pumped pulsed dye laser (FPDL) is the treatment of choice [7] Nevus Flammeus: Treatment is recommended during infancy. (prevent the soft tissue and bony abnormalities). Can be treated with flash-lamp pumped pulsed dye laser FPDL, (minimal scarring). As an adjunct to therapy, opaque make-up[7] Hemangiomas: They require no specific therapy other than patient education and reassurance. Treatment with FPDL and topical antibiotics may hasten recovery.[7]
Management of vascular nevi: corticosteroids Corticosteroids, inhibit fibrinolysis in vessel walls, reduce plasminogen activators, inhibit angiogenesis and induce regression of vessels.[7]
Management of vascular nevi: Interferon Alfa-2a This is used in life- or sight-threatening hemangiomas that are resistant to corticosteroids. These agents inhibit angiogenesis and prevent platelet trapping.[7]
Management of vascular nevi: laser therapy The neodynium:YAG (Nd:YAG) This type is used in the treatment of rapidly growing mixed or deep hemangiomas.[7] The carbon dioxide (CO2) laser is useful in managing subglottic hemangiomas not responding to corticosteroids This method can reduce the need of tracheostomy in airway obstruction. There is a increased risk of scarring , so not recommended for initial treatment. [7]
Management of vascular nevi: surgical excision Surgical excision is indicated in the management of visceral or ocular lesions not responding to corticosteroids.[9] Embolization is done preoperatively to reduce blood loss.[7]
Management of vascular nevi: anti-fibrinolytics Aminocaproic acid (Amicar) and tranexamic acid (Cyclokapron) are anti-fibrinolytic agents. These drugs act by inhibiting plasminogen activator and plasmin. [7]
References
  1. RUSSAK JE, DINULOS JG. Pigmented Lesions in Children Semin Plast Surg [online] 2006 Aug, 20(3):169-179 [viewed 27 May 2014] Available from: doi:10.1055/s-2006-949120
  2. HAINER BL. Electrosurgery for the skin. Am Fam Physician [online] 2002 Oct 1, 66(7):1259-66 [viewed 27 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/12387438
  3. KIM YJ, WHANG KU, CHOI WB, KIM HJ, HWANG JY, LEE JH, KIM SW. Efficacy and Safety of 1,064 nm Q-switched Nd:YAG Laser Treatment for Removing Melanocytic Nevi Ann Dermatol [online] 2012 May, 24(2):162-167 [viewed 27 May 2014] Available from: doi:10.5021/ad.2012.24.2.162
  4. Congenital melanocytic naevi.DermNet NZ [online].DermNet New Zealand Trust.2013.[viewed 26 May 2014].Available form:http://www.dermnetnz.org/lesions/congenital-naevus.html
  5. Melanoma and pigmented lesions.Clinical Knowledge summaries.NICE[online].National Institute for Health and Care Excellence.2011[viewed 26 May 2014].Available form:http://cks.nice.org.uk/melanoma-and-pigmented-lesions
  6. KLIEGMAN, Robert M. et al ed.Nelson textbook of PEDIATRICS.19 ed.vol 2.Philadelphia:Saunders - Elsevier.2011.pp.2231-2235
  7. WIRTH FA, LOWITT MH. Diagnosis and treatment of cutaneous vascular lesions. Am Fam Physician [online] 1998 Feb 15, 57(4):765-73 [viewed 29 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/9490999