Giant Melanocytic Nevus with Multiple Hemangiomas of the Scalp and Recurrent Lobular Capillary Hemangioma : Indian Journal of Paediatric Dermatology

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Giant Melanocytic Nevus with Multiple Hemangiomas of the Scalp and Recurrent Lobular Capillary Hemangioma

Vijay, Atul; Saini, Shivani1; Agarwal, Shail1,

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Indian Journal of Paediatric Dermatology 23(4):p 330-332, Oct–Dec 2022. | DOI: 10.4103/ijpd.ijpd_170_21
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Dear Madam,

A 4-year-old female child born out of nonconsanguineous marriage by normal vaginal delivery at term to a primigravida, with a normal and uneventful antenatal history, presented to our skin outpatient department with progressively extensive and pruritic hyperpigmented skin lesion persistent since birth and recurrent red-colored growths over head and neck, conjunctiva, and oral mucosa for the past 15 days of age, which used to bleed on touch or minor trauma.

The pigmented lesion was progressive proportionate to the growth of the child. The red-colored raised lesion initially appeared in multiple mustard size, round, firm lesions, which progressed to almost pea-sized pedunculated lesions in 2–5 days and bled on touch/slightest trauma. They were recurring in nature as they both appeared and disappeared with the progression of time in the head and neck region. There was no family history of similar lesions. All milestones were achieved on time. There were no systemic symptoms associated.

Cutaneous examination revealed a large, well-defined, irregular, unequally pigmented (dark brown to black colored) patch covering almost 85% of the trunk, including the entire back, 50% of the chest, and entire abdomen, with few small satellite lesions of 1–2 cm size. The hairs over the patch were coarse, darker, and longer than normal skin.

There was the presence of a raised solid skin-colored lesion of size 2 cm and rubbery consistency over the right posterior parietal scalp diagnosed clinically as infantile hemangioma. It started a few days after birth and was very slowly progressive in nature, now looking like residual fibrofatty tissues with overlying lax atrophic skin [Figure 1a-c].

F1
Figure 1:
(a) Clinical appearance of giant congenital melanocytic nevus. A large, irregular, well-defined, and unequally pigmented rough patch covering almost 85% of the trunk. (b) Lobular capillary hemangioma, seen as multiple firm, round, red raised lesions on the face (purple arrow). (c) Infantile hemangioma seen as a appears as 2 cm raised solid skin-colored lesion of size 2 cm, covered with lax atrophic skin overlying the right posterior parietal scalp region

Biopsy of the truncal black patch showed irregularly hyperplastic squamous epithelium with dermis containing a nest of melanocytic cells. These melanocytic cells also surrounded the fatty tissue, and the presence of melanin pigments confirmed the diagnosis of congenital melanocytic nevus (CMN). Biopsy of the bleeding red lesions (pyogenic granuloma) from the scalp showed polypoidal mass lined by keratotic and flattened squamous epithelium with underlying lobules of variable-sized capillaries, consistent with lobular capillary hemangioma [Figure 2a and b].

F2
Figure 2:
(a) Histopathology from darker patch showing keratotic irregularly hyperplastic squamous epithelium with dermis containing nests of melanocytic cells, also surrounded by fatty tissue, melanophages, and melanin pigment, suggestive of congenital melanocytic nevus (H and E stain, ×10). (b) Biopsy from red lesion from scalp showing a polypoidal mass lined by keratotic and flattened squamous epithelium with underlying lobules of variable-sized capillary suggestive of lobular capillary hemangioma (H and E stain, ×10)

No other congenital malformation was observed. An examination of the heart, lungs, abdomen, and extremities did not reveal any significant anomaly. Computed tomography scan of the head was normal, but magnetic resonance imaging (MRI) brain showed multiple scalp hemangiomas, the largest being in the right posterior parietal region (size 16 mm × 6 mm), while underlying skull vault appears normal [Figure 3]. Ultrasound of the abdomen, X-ray of the spine, and fundus examination of the eye were normal.

F3
Figure 3:
This section of magnetic resonance imaging brain showed multiple scalp hemangiomas, the largest being in the right posterior parietal region (size 16 mm × 6 mm), while underlying skull vault appears normal

There was no evidence of malignant transformation.

CMNs are brown to black-colored neurocristopathy, present at birth or shortly thereafter, commonly overlying back and thighs. CMNs are classified according to the maximum diameter expected to be reached by adulthood into small (<1.5 cm), medium (1.5–19.9 cm), and large or giant (>20 cm).[1] Giant CMNs (GCMNs) are rare and seen in 1:20,000–1:500,000 new-borns.[2] They are usually limited to skin but occasionally may invade deeper structures. GCMNs have been described to be associated with spina bifida occulta, neurofibromatosis, lipomatosis, and various other disorders.[23]

CMNs originate from a combination of epidermal and dermal-derived nevus cells. GCMNs are believed to arise from a spontaneous mutation during fetal development but may be genetically inherited in some families. The risk of malignant melanoma is 6%.[3] Malignant transformation should be suspected in cases of focal growth, pruritus, pain, bleeding, ulceration, or significant pigmentary changes. The risk of malignancy is increased in patients with larger lesions (>50 cm), axial lesions, multiple satellite lesions, and presence of nodular, dark patches; junctional activity, deep dermal component, or a blue nevus component on histopathology.[45]

MRI of the brain is warranted to evaluate melanocytic deposition in the central nervous system.

Our case report represents a case of GCMN with recurrent lobular capillary hemangioma, which is unique because there has not been any case reporting with this combination as far. Its concurrent occurrence cannot be explained as these are incidental findings.

We found severe anemia in this patient, which was assumed due to the recurrent nature of pyogenic granuloma, which causes blood loss.

Therapeutic options for CMN vary from surgical resection to laser therapy. Treatment is individualized based on the age of the presenting patient, size and location of the lesion, and risk of melanoma and neurocutaneous melanosis. The procedures commonly used are serial excision and reconstruction with skin grafting, tissue expansion, local rotation flaps and free tissue transfer, phenol peels, dermabrasion, and curettage.[6] Carbon dioxide laser and Er: YAG laser has been used recently to resurface deeper pigmentation and better cosmetic outcomes.[7] However, if deeper tissues such as leptomeninges are involved, excision may not eliminate the risk of development of malignant melanoma.[8]

We explained patient’s guardians about the nature of CMN. Most of the pyogenic hemangiomas were treated with radiofrequency and simple excision with suturing because of their recurring nature even after the use of topical timolol and oral propranolol previously, which was given by another dermatologist, according to guardians. For the management of oral pyogenic hemangioma, the patient was referred to a pediatric surgeon and dentist, and for conjunctival pyogenic granuloma, the patient was referred to an ophthalmologist. Other modalities for pyogenic hemangiomas that can also be used are intralesional corticosteroids, sclerosants, bleomycin, etc., Complications for the same may vary as ulceration, hemorrhage, secondary infections, and cosmetic disfigurement.

Declaration of consent

The authors certify that they have obtained all appropriate consent forms, duly signed by the parent(s) of the patient. In the form the parent(s) has/have given his/her/their consent for the images and other clinical information of their child to be reported in the journal. The parents understand that the names and initials of their child will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1. Arneja JS, Gossin A Giant congenital melanocytic nevi Plast Reconstr Surg 2007 120 26e 40e
2. Rhodes AR Melanocytic precursors of cutaneous melanoma. Estimated risks and guidelines for management Med Clin North Am 1986 70 3 37
3. Habit TP Clinical Dermatology:A color guide to Diagnosis and Therapy Mosby 2004 776 7
4. Rhodes AR, Melski JW Small congenital nevocellular nevi and the risk of cutaneous melanoma J Pediatr 1982 100 219 24
5. Foster RD, Williams ML, Barkovich AJ, Hoffman WY, Mathes SJ, Frieden IJ Giant congenital melanocytic nevi:The significance of neurocutaneous melanosis in neurologically asymptomatic children Plast Reconstr Surg 2001 107 933 41
6. Whang KK, Kim MJ, Song WK, Cho S Comparative treatment of giant congenital melanocytic nevi with curettage or Er:YAG laser ablation alone versus with cultured epithelial autografts Dermatol Surg 2005 31 1660 7
7. Park SH, Koo SH, Choi EO Combined laser therapy for difficult dermal pigmentation:Resurfacing and selective photothermolysis Ann Plast Surg 2001 47 31 6
8. Mark GJ, Mihm MC, Liteplo MG, Reed RJ, Clark WH Congenital melanocytic nevi of the small and garment type. Clinical, histologic, and ultrastructural studies Hum Pathol 1973 4 395 418
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