Linear and whorled nevoid hypermelanosis: A case report with dermoscopic findings

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Indian Journal of

Dermatology, Venereology & Leprology

An IADVL Publication

January-February

2016

Issue 1

Volume 82

www.ijdvl.com

Indian Journal of Dermatology , V

enereology & Leprology •

V olume 82 • Issue 1 • January - February 2015 • Pages 1-00**

In the Issue…

 Targeted phototherapy

 Paradoxical reactions induced by tumor necrosis factor-alpha antagonists: A literature review based on 46 cases

 Melanocyte-keratinocyte transplantation procedure: A few insights

 Development and validation of a Hindi language health-related quality of life questionnaire for melasma in Indian patients

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91 Indian Journal of Dermatology, Venereology, and Leprology | January-February 2016 | Vol 82 | Issue 1

Letters to the Editor fifty cases. Pediatr Dermatol 1999;16:345-8.

6. Handa S, Sahoo B. Childhood lichen planus: A study of 87 cases. Int J Dermatol 2002;41:423-7.

7. Gupta LK, Garg A, Khare AK, Mittal A. Lichen planus presenting as erythroderma. Indian J Dermatol Venereol Leprol 2012;78:409.

8. Rose AE, Patel U, Chu J, Patel R, Meehan S, Latkowski JA. Erythrodermic lichen planus. Dermatol Online J 2011;17:26. 9. Campos-Domínguez M, Silvente C, de la Cueva P,

González-Carrascosa M, Lecona M, Suárez R, et al. Erythrodermic lichen planus pemphigoides. Actas Dermosifiliogr 2006;97:583-6.

10. Rym BM, Mourad M, Bechir Z, Dalenda E, Faika C, Iadh AM, et al. Erythroderma in adults: A report of 80 cases. Int J Dermatol 2005;44:731-5.

How to cite this article: Gupta C, Sinha S, Sahoo B, Garg VK.

Erythroderma secondary to lichen planus in a child. Indian J Dermatol Venereol Leprol 2016;82:89-91.

Received: December, 2014. Accepted: August, 2015.

This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

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DOI:

10.4103/0378-6323.171654

PMID: *****

Financial support and sponsorship Nil.

Confl icts of interest

There are no conflicts of interest.

Chitra Gupta, Surabhi Sinha

1

_{, ,}

Bijaylaxmi Sahoo

2

_{, Vijay Kumar Garg}

2 Central Government Health Scheme, North Zone,

1_{Departments of dermatology, venereology and leprology}

Dr. Ram Manohar Lohia Hospital, Post Graduate Institute of Medical Education and Research, 2_{Maulana Azad}

Medical College, Lok Nayak Hospital, New Delhi, India Address for correspondence:

Address for correspondence: Dr. Surabhi Sinha, C - 403, Sabka Ghar, C.G.H.S., Plot No. 23, Sector 6, Dwarka, New Delhi - 110 075, India. E-mail: surabhi2310@gmail.com

REFERENCES REFERENCES

1. Sehgal VN, Srivastava G. Erythroderma/generalized exfoliative dermatitis in pediatric practice: An overview. Int J Dermatol 2006;45:831-9.

2. Boyd AS, Neldner KH. Lichen planus. J Am Acad Dermatol 1991;25:593-619.

3. Hurwitz S, editor. Papulosquamous and related disorders in clinical paediatric dermatology. In: A Textbook of Skin Disorders of Childhood and Adolescence. 2nd_{ed. Philadelphia:}

WB Saunders; 1981. p. 83-105.

4. Kanwar AJ, Handa S, Ghosh S, Kaur S. Lichen planus in childhood: A report of 17 patients. Pediatr Dermatol 1991;8:288-91.

5. Sharma R, Maheshwari V. Childhood lichen planus: A report of

Linear and whorled nevoid

hypermelanosis: A case report

with dermoscopic fi ndings

Sir,

Linear and whorled nevoid hypermelanosis is a rare pigmentary disorder presenting with brownish macules in a streaky configuration along the lines of Blaschko; the macules lack atrophy and are not preceded by any inflammatory or palpable lesions.[1]_{The most common}

sites involved are the trunk and extremities; the palms, soles, face and mucosae are usually spared.[1-3]_{It is}

histopathologically characterized by an increase in basal layer pigmentation without melanocyte proliferation; pigment incontinence is generally absent.[1,4]_The

lesions may be present at birth or may develop in the first few weeks of life and continue to progress for a year or two before stabilization.[2]_{Hyperpigmentation}

tends to persists throughout life, although the

macules may become less prominent with age in some patients.[1,2]_{Linear and whorled nevoid hypermelanosis}

does not show a sex predilection and typically occurs sporadically but rare familial instances have also been described.[1]_{It is considered to result from the}

expression of genetic mosaicism or chimerism and several cytogenetic alterations have been reported.[3]

The condition may be associated with extracutaneous abnormalities including central nervous system diseases, cardiac defects, psychomotor delay, deafness, brachydactyly and hydrocephalus.[1]_{The true incidence}

of such associated systemic features is unknown but some studies have reported a rate between 16% and 31%.[2]_{We report a case of linear and whorled nevoid}

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Indian Journal of Dermatology, Venereology, and Leprology | January-February 2016 | Vol 82 | Issue 1 92

Letters to the Editor

progressed to involve both upper and lower limbs by the time she turned one. There was no history of previous vesiculo-bullous or inflammatory lesions and similar lesions were not present in any family member. There were no abnormalities of the mucosae or skin appendages. The girl had normal developmental milestones and systemic examination was within normal limits On dermoscopy, the whorled patches on the trunk showed numerous brownish rings and curved lines and a few brownish streak-like lines [Figure 2a], while the linear patches on the legs showed short brownish streak-like lines arranged in a parallel manner and a few curved brownish lines [Figure 2b]. Another dermoscopic finding present in both linear and whorled lesions consisted of focally distributed hypopigmented dots corresponding to perifollicular areas [Figures 2a and 2b].

A skin biopsy from a lesion on her left thigh confirmed the clinical diagnosis of linear and whorled nevoid hypermelanosis by revealing increased basal layer pigmentation without melanocytosis; there was no pigment incontinence in the dermis [Figure 3]. Genetic studies to evaluate the presence of chimerism or mosaicism were not done as the girl’s parents refused. The patient was reassured and explained about the benign nature of the disorder; no treatment was given. Dermoscopic features of linear and whorled nevoid hypermelanosis have been described in two previous instances and the findings were found to be discordant.[3,4]

Naveen and Reshme reported a “net like” pattern of pigmentation noted in both linear and whorled lesions while Ertam et al. described a “parallel” pattern which consisted of linear or circular arrangements of parallel brown streaks with an overall alignment along the lines of Blaschko.[3,4]_{In particular, on the epigastric region,}

due to the whorled-like configuration of the lesions, the authors found partially curved, circular and/or linear parallel pigmented streaks in a parallel pattern while on the popliteal region, the same curved and/or linear streak-like pigmentations were vertical to skin profiles, but parallel to each other.[4]_{The dermoscopic features}

described in our case are reminiscent of the “parallel” pattern. Moreover, the predominance of linear streaks on the linear patches on the legs, and rings/curved lines on the whorled lesions of the back may be justified by the well-known regional variations in morphology of Blaschko’s lines.[4]_{However, our patient showed one}

additional previously unreported dermoscopic feature, namely, dotted perifollicular hypopigmentation. Interestingly, these dots did not involve all the follicular

Figure 1: Brownish macules arranged in a whorled and linear pattern along the lines of Blaschko on the trunk (a) and limbs (b)

b a

Figure 3: Increased basal layer pigmentation without melanocytosis; there is no pigment incontinence in the dermis (H and E, ×200)

Figure 2: (a) Dermoscopic view shows numerous brownish rings (white star) and curved lines (white arrowhead) and a few brownish streak-like lines (white arrow) on the whorled patches of the trunk and (b) short brownish streak-like lines arranged in a parallel way (white arrow) and a few curved brownish lines (white arrowhead) on the linear patches of the legs. Focally distributed hypopigmented dots (black arrow), corresponding to perifollicular areas, are also present in both whorled (a) and linear (b) lesions (×10)

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93 Indian Journal of Dermatology, Venereology, and Leprology | January-February 2016 | Vol 82 | Issue 1

Letters to the Editor

ostia in the lesional areas which is in keeping with the mosaic nature of the disorder.[3]

In conclusion, our case emphasizes that linear and whorled nevoid hypermelanosis may be dermoscopically heterogeneous, displaying some individual variations. It also confirms that dermoscopy might be useful in differentiating this disorder from its prime differential diagnosis, namely, incontinentia pigmenti, which is characterized by blue-gray dots histologically corresponding to pigmentary incontinence.[3]

Financial support and sponsorship Nil.

Confl icts of interest

There are no conflicts of interest.

Enzo Errichetti, Enrico Pegolo

1

_{, Giuseppe Stinco}

Department of Experimental and Clinical Medicine, Institute of

Dermatology, University of Udine, 1_{Department of Medical and}

Biological Sciences, Institution of Anatomic Pathology, Santa Maria della Misericordia University Hospital, Udine, Italy Address for correspondence:

Address for correspondence: Dr. Giuseppe Stinco, Institute of Dermatology, Santa Maria della Misericordia University Hospital, Piazzale Santa Maria della Misericordia, 15-33100 Udine, Italy. E-mail: giuseppe.stinco@uniud.it

How to cite this article: Errichetti E, Pegolo E, Stinco G. Linear and

whorled nevoid hypermelanosis: A case report with dermoscopic fi ndings. Indian J Dermatol Venereol Leprol 2016;82:91-3.

Received: January, 2015. Accepted: July, 2015.

This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

Access this article online Quick Response Code: Website:

www.ijdvl.com DOI: 10.4103/0378-6323.171007 PMID: ***** REFERENCES REFERENCES

1. Metta AK, Ramachandra S, Sadath N, Manupati S. Linear and whorled nevoid hypermelanosis in three successive generations. Indian J Dermatol Venereol Leprol 2011;77:403. 2. Di Lernia V. Linear and whorled hypermelanosis. Pediatr

Dermatol 2007;24:205-10.

3. Naveen KN, Reshme P. Linear and whorled nevoid hypermelanosis with dermatoscopic features. Dermatol Online J 2014;20:13.

4. Ertam I, Turk BG, Urkmez A, Kazandi A, Ozdemir F. Linear and whorled nevoid hypermelanosis: Dermatoscopic features. J Am Acad Dermatol 2009;60:328-31.

Multifocal spindle cell

hemangioma: Report of two cases

Sir,

Spindle cell hemangioma, formerly known as spindle cell hemangioendothelioma was first described in 1986 and was initially considered to be a low-grade angiosarcoma. In 1996, its benign behavior was recognised and the name spindle cell hemangioma was proposed.[1]_{It is a}

rare vascular proliferation, with less than 200 reported cases. The tumor originates from blood vessels and recently, lymphatic origin has been suggested.[2]_The

standard treatment is wide local excision but the the tumor has a high recurrence rate. We present two cases of multifocal spindle cell hemangiomas. One case was successfully treated with sclerotherapy suggesting that this can be an alternative treatment which is less invasive and has better long-term results.

The first case was a 10-year-old girl with a 4-year history of multiple violaceous and reddish cutaneous

and subcutaneous nodules on the right ankle and foot [Figure 1]. Magnetic resonance imaging performed to determine the extent of the lesions showed multiple hypervascular deep nodules in the left foot and ankle with irregular bone resorption. She was treated with sclerotherapy. The nodules were punctured under ultrasound guidance. Under angiographic monitoring, injection of 2% etoxisclerol foam was performed [Figure 2] which lead to significant reduction in the volume of the lesions. There were no adverse effects and patient was asymptomatic at 2 years of follow up.

The second case was an 18-year-old woman with multiple subcutaneous nodules located on the left upper extremity. Magnetic resonance imaging showed multiple hyperintense nodules on T2 in the left upper extremity and neck. Patient had undergone surgical removal of 12 nodules 4 years back, all of which had recurred in 18 months and new lesions continued to appear.