Segmental Pigmentation Disorder: Clinical Manifestations and Epidemiological Features in 144 patients, a Retrospective Case-control Study

Authors

  • Meital Oren-Shabtai Division of Dermatology, Rabin Medical Center
  • Aryeh Metzker
  • Dan Ben Amitai
  • Eli Sprecher
  • Ilan Goldberg Department of Dermatology, Tel Aviv Sourasky Medical Center, 6 Weizman Street, Tel Aviv 64239 Israel

DOI:

https://doi.org/10.2340/actadv.v102.399

Keywords:

pigmentation, segmental, mosaicism, extracutaneous manifestations

Abstract

Segmental pigmentation disorder (SPD) is characterized by hypo- or hyper-pigmented patches segmentally distributed, present in infancy, more prominently in darker-skinned children. The aim of this study was to define the demographic and clinical characteristics of SPD in a large series of patients. This was a retrospective case-control study at 2 paediatric dermatology centres in Israel. Data were collected through a telephone questionnaire and medical records. The study group consisted of 144 individuals with SPD and 144 individuals visiting the same institutions matched for age and sex. Median age of onset of SPD was near birth; 51% of patients were Sephardic Jews, and patients were followed up for a median period of 27 years. The patches were located on the torso (43%), mostly hypopigmented (52%), and remained of the same intensity and size in 55% and 41% of cases, accordingly. No differences in extracutaneous morbidities were found between SPD and control patients. This study delineates the demographic and clinical characteristics of SPD, confirms that cutaneous findings in SPD are more prominent in darker skin, tends not to expand in size or accentuate throughout the years, nor to be associated with extracutaneous morbidities.

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References

Metzker A, Morag C, Weitz R. Segmental pigmentation disorder. Acta Derm Venereol 1983; 63: 167-169.

Orion E, Matz H, Wolf D, Wolf R. Café au lait has a hue of its own. Dermatol Online J 2003; 9: 8.

https://doi.org/10.5070/D38PQ4J4ND

Min EA. Puzzling brown area on toddler's skin. JAAPA 2007; 20: 15.

https://doi.org/10.1097/01720610-200706000-00004

Lombillo VA, Sybert VP. Mosaicism in cutaneous pigmentation. Curr Opin Pediatrics 2005; 17: 494-500.

https://doi.org/10.1097/01.mop.0000171319.84053.45

Taïeb A, Boralevi F. Hypermelanoses of the newborn and of the infant. Dermatol Clin 2007; 25: 327-336.

https://doi.org/10.1016/j.det.2007.04.011

Hogeling M, Frieden IJ. Segmental pigmentation disorder. Br J Dermatol 2010; 162: 1337-1341.

https://doi.org/10.1111/j.1365-2133.2010.09702.x

Happle R. Mosaicism in human skin. Understanding the patterns and mechanisms. Arch Dermatol 1993; 129: 1460-1470.

https://doi.org/10.1001/archderm.1993.01680320094012

Lerner AB, Dnell RS, Chanco-turner ML, McGuire JS. Vitiligo and sympathectomy. Arch Dermatol 1966; 94: 269-277.

https://doi.org/10.1001/archderm.1966.01600270019004

Cho E, Cho SH, Lee JD. Patterned pigmentation in a child: a case of segmental pigmentation disorder. J Dermatol 2011; 38:1094-1096.

https://doi.org/10.1111/j.1346-8138.2011.01225.x

Findlay GH, Moores PP. Pigment anomalies of the skin in the human chimaera, their relation to systematized naevi. Br J Dermatol 1980; 103: 489-498.

https://doi.org/10.1111/j.1365-2133.1980.tb01663.x

Loomis CA. Linear hypopigmentation and hyperpigmentation, including mosaicism. Semin Cutan Med Surg 1997; 16: 44-53.

https://doi.org/10.1016/S1085-5629(97)80035-1

Ortonne JP, Brocard E, Floret D, Perrot H, Thivolet J. Diagnostic value of café-au-lait spots. Ann Dermatol Venereol 1980; 107: 313-327.

Lee HS, Chun YS, Hann SK. Nevus depigmentosus: clinical features and histopathologic characteristics in 67 patients. J Am Acad Dermatol 1999; 40: 21-26.

https://doi.org/10.1016/S0190-9622(99)70524-4

Kim SK, Kang HY, Lee ES, Kim YC. Clinical and histopathologic characteristics of nevus depigmentosus. J Am Acad Dermatol 2006; 55: 423-428.

https://doi.org/10.1016/j.jaad.2006.04.053

Xu AE, Huang B, Li YW, Wang P, Shen H. Clinical, histopathological and ultrastructural characteristics of naevus depigmentosus. Clin Exp Dermatol 2008; 33: 400-405.

https://doi.org/10.1111/j.1365-2230.2008.02714.x

Landau M, Krafchik BR. The diagnostic value of café-au-lait macules. J Am Acad Dermatol 1999; 40: 877-890.

https://doi.org/10.1016/S0190-9622(99)70075-7

Rieger E, Kofler R, Borkenstein M, Schwingshandl J, Soyer HP, Kerl H. Melanotic macules following Blaschko's lines in McCune-Albright syndrome. Br J Dermatol 1994; 130: 215-220.

https://doi.org/10.1111/j.1365-2133.1994.tb02903.x

Völkl TM, Dörr HG. McCune-Albright syndrome: clinical picture and natural history in children and adolescents. J Pediatr Endocrinology Metab 2006; 19: 551-559.

https://doi.org/10.1515/JPEM.2006.19.S2.551

Davies JH, Barton JS, Gregory JW, Mills C. Infantile McCune-Albright syndrome. Pediatr Dermatol 2001; 18: 504-506.

https://doi.org/10.1046/j.1525-1470.2001.1862003.x

Schaffer JV, Orlow SJ, Lazova R, Bolognia JL. Speckled lentiginous nevus: within the spectrum of congenital melanocytic nevi. Arch Dermatol 2001; 137: 172-178.

Kalter DC, Griffiths WA, Atherton DJ. Linear and whorled nevoid hypermelanosis. J Am Acad Dermatol 1988; 19: 1037-1044.

https://doi.org/10.1016/S0190-9622(88)70269-8

Sarma N. Pigmentary nevi on face have unique patterns and implications: the concept of Blaschko's lines for pigmentary nevi. Indian J Dermatol 2012; 57: 30-34.

https://doi.org/10.4103/0019-5154.92673

Effendy I, Happle R. Linear arrangement of multiple congenital melanocytic nevi. J Am Acad Dermatol 1992; 27: 853-854.

https://doi.org/10.1016/0190-9622(92)70265-H

Langenbach N, Pfau A, Landthaler M, Stolz W. Naevi spili, Café-au-lait spots and melanocytic naevi aggregated alongside Blaschko's lines, with a review of segmental melanocytic lesions. Acta Derm Venereol 1998; 78: 378-380.

https://doi.org/10.1080/000155598443114

Nachbar F, Merkle T, Ruzicka T, Plewig G. Congenital junctional naevi following Blaschko's lines. Eur J Dermatol 1993; 3: 478-479.

Hanayama H, Terashi H, Hashikawa K, Tahara S. Congenital melanocytic nevi and nevus spilus have a tendency to follow the lines of Blaschko: an examination of 200 cases. J Dermatol 2007; 34: 159-163.

https://doi.org/10.1111/j.1346-8138.2007.00242.x

Rao AG. Bilateral symmetrical congenital giant Becker's nevus: a rare presentation. Indian J Dermatol 2015; 60: 522.

https://doi.org/10.4103/0019-5154.164441

Sood A, D'Souza P, Verma KK. Becker's naevus occurring at birth and in early childhood. Acta Derm Venereol 1998; 78: 311.

https://doi.org/10.1080/000155598442034

Listernick R, Mancini AJ, Charrow J. Segmental neurofibromatosis in childhood. Am J Med Genet 2003; 121A: 132-135.

https://doi.org/10.1002/ajmg.a.20183

Bolognia JL, Pawelek JM. Biology of hypopigmentation. J Am Acad Dermatol 1988; 19: 217-255.

https://doi.org/10.1016/S0190-9622(88)70168-1

Akhami RN, Schwartz RA. Nevus anemicus. Dermatology 1999; 198: 327-329.

https://doi.org/10.1159/000018169

Trauner MA, Ruben BS, Lynch PJ. Segmental tuberous sclerosis presenting as unilateral facial angiofibromas. J Am Acad Dermatol 2003; 49: S164-166.

https://doi.org/10.1067/mjd.2003.146

Happle R. Lethal genes surviving by mosaicism: a possible explanation for sporadic birth defects involving the skin. J Am Acad Dermatol 1987; 16: 899-906.

https://doi.org/10.1016/S0190-9622(87)80249-9

Bieber T, Bussmann C. Atopic dermatitis. In: Bolognia JL, Jorizzo JL, Schaffer JV, editors. Dermatology, 3rd edn. Elsevier, China; 2012: p. 203-204.

Little H, Kamat D, Sivaswamy L. Common neurocutaneous syndromes. Pediatric Annals 2015; 44: 496-504.

https://doi.org/10.3928/00904481-20151112-11

Nehal KS, PeBenito R, Orlow SJ. Analysis of 54 cases of hypopigmentation and hyperpigmentation along the lines of Blaschko. Arch Dermatol 1996; 32: 1167-1170.

https://doi.org/10.1001/archderm.1996.03890340027005

Dhar S, Kanwar AJ, Kaur S. Nevus depigmentosus in India: experience with 50 patients. Pediatr Dermatol 1993; 10: 299-300.

https://doi.org/10.1111/j.1525-1470.1993.tb00387.x

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Published

2022-05-10

How to Cite

Oren-Shabtai, M., Metzker, A., Ben Amitai, D., Sprecher, E., & Goldberg, I. (2022). Segmental Pigmentation Disorder: Clinical Manifestations and Epidemiological Features in 144 patients, a Retrospective Case-control Study. Acta Dermato-Venereologica, 102, adv00707. https://doi.org/10.2340/actadv.v102.399

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