|Year : 2021 | Volume
| Issue : 3 | Page : 179-185
Zosteriform speckled lentiginous nevus with bilateral segmental neurofibromatosis: A rare association
Bhagyashree B Supekar1, Vaishali H Wankhade1, Apoorva D Chopkar1, Rajesh P Singh1, Dharitri Bhat2
1 Department of Dermatology, Venereology and Leprology, Government Medical College and Hospital, Nagpur, Maharashtra, India
2 Department of Pathology, Government Medical College, Nagpur, Maharashtra, India
|Date of Submission||05-Aug-2020|
|Date of Decision||17-Oct-2020|
|Date of Acceptance||10-Apr-2021|
|Date of Web Publication||24-Nov-2021|
Dr. Vaishali H Wankhade
Department of Dermatology, Venereology and Leprology, Government Medical College and Hospital, Nagpur 440003, Maharashtra
Source of Support: None, Conflict of Interest: None
Bilateral segmental neurofibromatosis is a nonfamilial variant of neurofibromatosis without any systemic involvement. Speckled lentiginous nevus (SLN) is a pigmented, light brown to tan patch of varied diameter, speckled with smaller, darker colored macules or papules. SLN has been reported in association with bilateral nevus of Ito, blue nevus, centrofacial lentiginosis, and nevus sebaceous. Its association with bilateral segmental neurofibromatosis has been rarely reported in the literature. We report a rare co-existence of zosteriform nevus spilus with bilateral segmental neurofibromatosis, diagnosed on the basis of clinico-dermoscopic-histopathological features, in a 25-year-old female.
Keywords: Bilateral segmental neurofibromatosis, dermoscopy, rare association, speckled lentiginous nevus, zosteriform distribution
|How to cite this article:|
Supekar BB, Wankhade VH, Chopkar AD, Singh RP, Bhat D. Zosteriform speckled lentiginous nevus with bilateral segmental neurofibromatosis: A rare association. Pigment Int 2021;8:179-85
| Introduction|| |
Segmental neurofibromatosis (SNF, classified as type V by Riccardi) is a nonfamilial type, characterized by neurofibromas and/or Cafe au lait macules (CALMs) limited to an area or segment of the body and without any other cutaneous and/or extracutaneous manifestations of neurofibromatosis (NF). Nevus spilus (speckled lentiginous nevus, SLN) is a rare type of congenital nevus that presents as hyperpigmented macules initially, followed by the formation of more obvious densely pigmented spots later in life. The association of both these conditions has been reported rarely in the literature.
| Case report|| |
A 25-year-old female born out of nonconsanguineous parentage, presented to us with multiple skin-colored, painless raised lesions over the abdomen and back, increasing in size and number since 15 years. She also complained of the appearance of the dark-colored flat lesion over the right side of the chest and back since the age of 10 years, which initially appeared on the chest and gradually progressed to involve the right side of the back, shoulder, upper arm, neck, and lower part of the face. She had no neurologic osculoskeletal complaints. None of her family members had similar complaints.
On cutaneous examination, there were multiple skin-colored, nontender, soft nodules of varying sizes ranging from 0.5 to 3 cm in diameter, confined to T8 to T11 dermatomes bilaterally over back [Figure 1]a,b and abdomen [Figure 1]c,d. Buttonhole sign was elicited on digital pressure in these nodules. No axillary or palmar freckling was observed. There were no CALMs and neurofibromas elsewhere on the body. There was a large tan-colored patch with multiple superimposed discrete hyperpigmented macules, distributed in a zosteriform pattern over the right lower half of face [Figure 2]a,b, the right upper half of chest, shoulder, upper arm [Figure 2]c, neck and back up to the midline [Figure 2]d. Dermoscopy was performed using a 3Gen Dermlite DL4 (CA, USA) 10× polarized mode. Dermoscopy of the nodule over back revealed peripheral halo of brown pigmentation, fingerprint-like structure, and peripheral pigment network, suggestive of neurofibroma [Figure 3]a–c, which was further confirmed on histopathology. Dermoscopy of the tan-colored patch over the chest revealed hyperpigmented brownish reticulate macules [Figure 4]a,b and homogenous macules [Figure 4]c,d suggestive of nevus spilus [Figure 4]a-d, diagnosis of which was further confirmed on histopathology. Histopathological examination of the nodule revealed regularly spaced thin spindle cells with elongated wavy nuclei among thin collagenous strands, suggestive of neurofibroma [Figure 5]a,b. Histopathology of the tan-colored patch over back revealed the presence of melanocytes in contiguity near tips and sides of elongated rete ridges [Figure 6]a,b. General and systemic examination was normal. Routine hematological investigations were normal. No abnormality was detected on ophthalmic and neurological examination. Based on clinical, dermoscopic, and histopathological findings, a diagnosis of bilateral SNF co-existing with zosteriform speckled lentiginous nevus was reached. The patient was counseled regarding the course of the condition and regular follow-up was advised.
|Figure 1 (a–d) Multiple skin-colored, nontender, soft nodules of varying sizes ranging from 0.5 to 3 cm in diameter, confined to T8 to T11 dermatomes bilaterally over back (a and b) and abdomen (c and d).|
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|Figure 2 (a-d) Multiple large tan-colored patches with multiple superimposed discrete hyperpigmented macules, distributed in a zosteriform pattern over the right lower half of face (a and b), right upper half of chest, shoulder, upper arm (c), neck, and back up to the midline (d).|
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|Figure 3 (a–c) Dermoscopy (3Gen Dermlite DL4, 10× Polarized mode) revealed peripheral halo of brown pigmentation (black arrow), fingerprint-like structures (black star; a), peripheral halo of pigmentation (black arrow; b), peripheral pigment network (green arrow) and fingerprint-like structures (black star; c) suggestive of neurofibroma.|
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|Figure 4 (a–d) Dermoscopy (3Gen Dermlite DL4, 10× polarized mode) of tan-colored patch over chest revealed hyperpigmented brownish reticulate macules (black arrow; a and b) and homogenous macules (green arrow; c and d) and, suggestive of nevus spilus.|
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|Figure 5 (a and b) Histopathological examination of nodule revealed regularly spaced thin spindle cells with elongated wavy nuclei among thin collagenous strands, suggestive of neurofibroma (H and E; a: 10× and b: 40×).|
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|Figure 6 (a and b) Histopathology of the tan-colored patch over back revealed presence of melanocytes in contiguity near tips and sides of elongated rete ridges. (H and E; a: 10× and b: 40×).|
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| Discussion|| |
The first reference for segmental neurofibromatosis was given by Gammel in 1931, later on, Crowe et al. described four similar cases of SNF. Miller and Sparkle proposed the term SNF in 1977.,, It is a rare condition, 10 to 20 times less frequent than NF. Tinschert et al. proposed that SNF is caused by somatic mutation of NF1. There is female preponderance and the right side of the body is commonly affected. Bilateral involvement has been described in only 6% of cases. The sites of predilection for SNF are thorax and abdomen (55%), arms (20%), lower limb, and face (10%). As per Roth’s classification of SNF, our case falls under the bilateral segmental category, who had the presence of neurofibromas only over the lower abdomen and back without extracutaneous and other cutaneous features of NF. Duman and Elmas have described for the first time dermoscopic description of neurofibroma as a peripheral pigment network, a peripheral halo of brown pigmentation, fingerprint-like structures, blood vessels, and pink-red structureless areas. In our case, dermoscopy of neurofibroma revealed peripheral pigment network, a peripheral halo of brown pigmentation and fingerprint-like structure. Nevus spilus (speckled lentiginous nevus, SLN) most frequently presents in the first year of life with no sex predilection. According to Happle, it is classically described as macular or papular SLN. It is mostly an isolated lesion. In a few cases, larger skin segments may be involved in the checkerboard pattern or have a zosteriform arrangement. Matsudo et al. proposed the term zosteriform SLN in 1973., It has a predilection for the trunk and lower limbs and rarely oral mucosae can be involved. Our case had macular SLN with the involvement of right lower half of face with ipsilateral involvement of shoulder, upper limb, upper chest, and back. The differential diagnoses for SLN include CALMs, congenital melanocytic nevus (CMN), Becker’s nevus, and agminated lentigines. CALM and CMN lack intralesional speckled macules and papules seen in SLN clinically. Becker’s nevus develops hypertrichosis and lacks intralesional speckled macules and papules. Agminated lentigines have sharp borders and present over normal-appearing skin and not in the light brown background like SLN. Manganoni et al. described pale or light brown areas with the reticular globular pattern as dermoscopy of NS. Kaminska-Winciorek described various characteristics as a reticular, globular, homogenous, or mixed pattern with solitary spitzoid central structure on dermoscopy. Our patient had hyperpigmented, brownish homogenous, and reticular macules on the dermoscopy of NS.
SLN has been reported in association with bilateral nevus of Ito, blue nevus, centrofacial lentiginosis, and nevus sebaceous. It has also been observed in phakomatosis pigmento-vascularis, phacomatosis pigmento-keratotica, and SLN syndrome. Paik et al. reported for the first time the association of bilateral SNF with speckled lentiginous nevus in a 41-year-old male, in the year 1996. Whether this finding is coincidental or there is an association needs further validation, as no theories have been postulated for this association. Recently, Arif et al. reported the coexistence of speckled lentiginous nevus with neurofibromatosis 1. To the contrary of previously published case reports, our case had coexistence of zosteriform speckled lentiginous nevus with bilateral SNF. To the best of our knowledge, this is the first instance of coexistence of zosteriform speckled lentiginous nevus with bilateral segmental neurofibromatosis, diagnosed on the basis of clinico-dermoscopic-histopathological findings in a 25-year-old female.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]