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Year : 2005  |  Volume : 71  |  Issue : 3  |  Page : 217-218

Hyperpigmented patch on the trunk of a neonate


1 Department of Dermatology,Venereology and Leprosy, BLDEAs SBMP Medical College, Hospital and Research Centre, Bijapur, India
2 Department of Pathology, BLDEAs SBMP Medical College, Hospital and Research Centre, Bijapur, India

Correspondence Address:
Arun C Inamadar
Department of Dermatology, Venereology and Leprosy, BLDEAs SBMP Medical College, Hospital and Research Centre, Bijapur - 586103, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0378-6323.16252

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How to cite this article:
Palit A, Inamadar AC, Athanikar S B, Sampagavi V V, Deshmukh N S, Yelikar B R. Hyperpigmented patch on the trunk of a neonate. Indian J Dermatol Venereol Leprol 2005;71:217-8

How to cite this URL:
Palit A, Inamadar AC, Athanikar S B, Sampagavi V V, Deshmukh N S, Yelikar B R. Hyperpigmented patch on the trunk of a neonate. Indian J Dermatol Venereol Leprol [serial online] 2005 [cited 2019 Jun 25];71:217-8. Available from: http://www.ijdvl.com/text.asp?2005/71/3/217/16252


A one-day-old, full-term, normally delivered, female neonate was being routinely examined. A brownish irregular patch of 2 cm ´ 1.5 cm, with fine long hairs was observed on her right loin [Figure - 1]. On gentle stroking, the lesion became more prominent along with the appearance of goose bumps. A punch biopsy was taken from the edge of the lesion. Histopathological preparation on H/E stain showed mild hyperkeratosis of the epidermis and basal cell hyperpigmentation. In the reticular dermis, well-defined smooth muscle fiber bundles were seen, interspersed among hair follicles [Figure - 2]. Masson Trichrome stain [Figure - 3] confirmed the presence of smooth muscle fibers (stained red).


  WHAT IS THE DIAGNOSIS? Top


Answer to Quiz

Diagnosis:
Congenital smooth muscle hamartoma (CSMH) or congenital arrector pili hamartoma.


  Discussion Top


CSMH was first described by Stokes in 1923.[1] The lesions may be solitary or multiple, the former being commoner (1 in 3000 births).[2] Classically, it is present at birth or seen during the first week of life, located most commonly over the lumbo-sacral region.[2] Other sites of occurrence are buttocks, proximal extremities, and rarely the scalp.[3] Multiple lesions are rare and may occur in a linear pattern.[4],[5] Diffuse involvement may occur with the Michelin type baby syndrome.[6]

The commonest morphological pattern is a skin-colored or slightly hyperpigmented patch or plaque associated with long vellus hairs or small follicular papules all over the lesion.[2] On gentle stroking, the lesion shows vermiculation and pilo-erection (Pseudo-Darier's sign)[2],[7] or occasionally fasciculation.[2] Lesions occurring as a linear atrophic plaque[4] and a patch with perifollicular papules[5] have been reported.

CSMH is related to the arrectores pilorum muscle,[8] which in fetal life originates from a diffuse metachromatic zone of mesoderm situated near the hair germ. Over-proliferation of this region is likely to give rise to the lesion. Clinically, CSMH has to be differentiated from cafι-au-lait macule, solitary mastocytoma, congenital melanocytic nevus and connective tissue nevus.[2] Histopathologically, pilar leiomyoma is an important differential diagnosis.[8] A well-defined bundle of long, straight smooth muscle cells in the deeper dermis, which are separated from the dermal collagen by a clear space, is characteristic of CSMH. Irregular smooth muscle bundles, intermingled with collagen, which may be attached to the hair follicle, are found in pilar leiomyoma.[5],[8]

An acquired form of smooth muscle hamartoma without hyperpigmentation or excessive hair has been reported.[9] Other acquired lesions occur in association with Becker's nevus.[4] There are clinical and histopathological overlaps between Becker's nevus and CSMH.[7],[8] Some authors include both the entities in a spectrum of hamartomatous disorders,[7] involving the epidermis (hyperpigmentation), hair follicles (hypertrichosis) and the dermis (smooth muscle). Children with the diffuse form of the disease may have other congenital malformations, growth and mental retardation.[7] No treatment is necessary for solitary lesions, which may persist unchanged or there may be slight reduction of the hypertrichosis.



 
  References Top

1.Stokes JH. Nevus pilaris with hyperplasia of non-striated muscle. Arch Dermatol Syphil 1923;7:479.  Back to cited text no. 1      
2.Atherton DJ. Naevi and other developmental defects. In : Champion RH, Burton JL, Burns DA, Breathnach SM, editors. Textbook of Dermatology, 6th Ed. Oxford: Blackwell-Science; 1998. p. 519-616.  Back to cited text no. 2      
3.Knable A, Treadwell P. Pigmented plaque with hypertrichosis on the scalp of an infant. Pediatr Dermatol 1996;13:431-3.  Back to cited text no. 3  [PUBMED]    
4.Grau-Massanes M, Raimer S, Colome-Grimmer M, Yen A, Sanchez RL. Congenital smooth muscle hamartoma presenting as a linear atrophic plaque: Case report and review of the literature. Pediatr Dermatol 1996;13:222-5.  Back to cited text no. 4  [PUBMED]    
5.Jang HS, Kim MB, Oh CK, Kwon KS, Chung TA. Linear congenital smooth muscle hamartoma with follicular spotted appearance. Br J Dermatol 2000;142:138-42.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]  
6.Oku T, Iwasaki K, Fujita H. Folded skin with an underlying cutaneous smooth muscle hamartoma. Br J Dermatol 1993;129:606-8.  Back to cited text no. 6  [PUBMED]    
7.Albrecht S. Neoplasias and hyperplasias of neural and muscular origin. In : Freedberg IM, Eisen AZ, Wolff KW, Austen KF, Goldsmith LA, Katz SI, editors. Fitzpatrick's Dermatology in General Medicine, 6th Ed. New York: McGraw-Hill; 2003. p. 1032-41.  Back to cited text no. 7      
8.Ragsdale BD. Tumors of fatty, muscular and osseous tissue: fat as a tissue, an organ and a source of tumors. In : Elder D, Elenitsas R, Jaworsky C, Johnson B Jr., editors. Lever's Histopathology of skin, 8th Ed. Philadelphia: Lippincott-Raven; 1997. p. 457-502.  Back to cited text no. 8      
9.Darling TN, Kamino H, Murray JC. Acquired cutaneous smooth muscle hamartoma. J Am Acad Dermatol 1993;28:844-5.  Back to cited text no. 9  [PUBMED]    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3]

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1 Hamartoma de músculo liso congénito: importancia de un correcto diagnóstico diferencial
C. Suárez Castañón,J. Martínez Blanco,B. Corrales Canel,M. Morán Poladura,J. Melgar Pérez
Anales de Pediatría. 2012; 77(6): 425
[Pubmed] | [DOI]



 

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