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CASE REPORT
Year : 2009  |  Volume : 54  |  Issue : 1  |  Page : 68-71
Multiple smooth muscle hamartoma: Case report and review of the literature


Department of Dermatology, Tehran University of Medical Sciences, Razi Hospital, Tehran, Iran

Correspondence Address:
Noormohammadpour Pedram
11996 Vahdate Eslami Ave, Vahdate Eslami Sq, Razi Hospital, Tehran
Iran
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.48993

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   Abstract 

Smooth muscle hamartoma (SMH) is a proliferative disorder of cells originating from muscle cells. It is a benign tumoral mass that usually presents as a single congenital skin-colored and hypertrichotic plaque involving the trunk and extremities. Multiple SMHs have rarely been reported in the literature. We describe the case of a seven-month-old girl with multiple SMHs located over the back and arm areas. The diagnosis was confirmed by biopsy and immunohistochemical (IHC) staining. She had no cerebral or skeletal abnormalities and her growth and development were normal.


Keywords: Multiple, congenital, smooth muscle hamartoma, immunohistochemical, skeletal abnormalities


How to cite this article:
Haydeh G, Massoud A, Pedram N. Multiple smooth muscle hamartoma: Case report and review of the literature. Indian J Dermatol 2009;54:68-71

How to cite this URL:
Haydeh G, Massoud A, Pedram N. Multiple smooth muscle hamartoma: Case report and review of the literature. Indian J Dermatol [serial online] 2009 [cited 2019 Jun 26];54:68-71. Available from: http://www.e-ijd.org/text.asp?2009/54/1/68/48993



   Introduction Top


A variety of cutaneous smooth muscle neoplasms may arise in the skin. [1] Smooth-muscle hamartoma (SMH) is an uncommon, usually congenital cutaneous hyperplasia of the arrectores pilorum muscles. When acquired, it may be confused with Becker's nevus with a prominent smooth-muscle component. [2] Smooth-muscle hamartoma was first described by Stokes in 1923. [3] Subsequently, in 1969, Sourreil, Beylot and Delfour reported congenital SMH (CMSH). [4] The lesions present as well-circumscribed, skin-colored plaques, frequently hypertrichotic, most commonly located on the trunk, buttocks or proximal extremities. [5] The estimated prevalence is about one per 2600 live births, with slight male predominance. [6]

Smooth-muscle hamartoma has been subdivided into two types, congenital (CSMH) and acquired (ASMH). The acquired type is distinct from the congenital form. CMSH is generally single, but multiple lesions have also been rarely reported in the literature. [6],[7] A positive pseudo-Darier sign (temporary induration or piloerection after rubbing) is present in 80% of the patients. [6] Treatment of SMH is not necessary. [1],[8]


   Case Report Top


A seven-month-old girl was evaluated for multiple lesions on the left mid-back and on the surface of the arms. Clinical examination found congenital multiple skin-colored, slightly raised plaques with hypertrichosis [Figure 1],[Figure 2],[Figure 3]. Hyperpigmentation was not present and the lesions were asymptomatic. A pseudo-Darier sign was observed after rubbing over some lesions.

Histopathologically, there were masses of spindle smooth muscle cells, distantly arranged in the superficial and deep dermis [Figure 4]. Immunohistochemical (IHC) stain with smooth muscle actin confirmed a diagnosis of SMH [Figure 5],[Figure 6] and the nature of the proliferation. An MRI (magnetic resonance imaging) and CT (computerized tomography) scan showed no neurological deficit and abnormality in the central nervous system (CNS). Electromyography (EMG) and nerve conduction velocity (NCV) findings were also normal. The rest of the clinical examination was normal.


   Discussion Top


Hamartoma is derived from the Greek word hamartanein , which means "to err" or to "fail". Hamartomas are defined as lesions that are (1) most commonly present at birth, but can also be acquired (2) composed of aberrant mature or nearly mature structures. [5]

Cutaneous smooth muscle hamartomas (SMHs) are caused by benign proliferation of smooth muscle bundle within the dermis. Congenital SMH (CSMH) is an uncommon malformation of the pilar smooth muscle that frequently involves the back and the lower limbs. Unusual cutaneous locations are the upper extremities, the face, and the mammary region. [9] Scrotal and eyelid-eyebrow involvement were also recently reported. Smooth muscle hamartomas present more frequently in male patients. [8],[10]

In a review of 26 cases, 8% had prominent overlying hair, 12% were patches with peri-follicular papules, but no prominent hair, 61% were hyperpigmented, and 39% were skin colored. [11]

Rubbing of the plaque may result in a pseudo-Darier sign consisting of transient edema, erythema, and induration of the affected area, which may diminish in intensity with age. [12],[13],[14]

Most cases are congenital; a prevalence of up to 0.2% in children has been reported and has been increasingly recognized during the last two decades. [15] Most frequently, CSMHs are single; multiple lesions have been reported rarely. [7],[9],[27]

Familial cases have also been recently reported, but a great majority of the cases occur in a sporadic fashion. [16] Unusual clinical patterns have been reported in the literature, in particular multiple congenital lesions, or generalized forms with deep systemic involvement. A generalized variant with hypertrichosis and folding of the skin, called 'Michelin Tire baby' has been reported. [17],[18] Congenital smooth muscle hamartoma (CSMH) with follicular spotted appearance is a rare clinical variant of CMSH in which the patients have marked peri-follicular papules in the patches. A linear distribution and a linear atrophic presentation of CSMH have also been reported. [19],[20] Association with bilateral Becker's nevus too has been reported. [21] The clinical differential diagnosis of a single CSMH includes congenital melanocytic (pigmented) nevus, Becker's melanosis, solitary mastocytoma, pilo-leiomyoma, cafe-au-lait spots, and nevus pilosus and connective tissues nevi. [22] For multiple lesions, the main differential diagnosis is fetal alcohol syndrome. [7]

The histopathologic pattern is distinct from all other benign muscle tumors of the skin and is mimicked only by the smooth muscle hyperplasia present in Becker's nevus. Some authors consider these lesions as a part of a spectrum, while others prefer to keep them separate. [23]

The clinical lesions usually become less prominent with time and a review of the approximately 50 cases reported in the literature showed that there is no known associated systemic involvement or malignant transformation. [24]

There appears to be a rare acquired type of smooth muscle proliferation, which has been described under the title acquired SMH (ASMH). [8],[25],[26] The color of the lesions varies from pink to flesh tones to brown. They may be without hyperpigmentation or hypertrichosis. It has been reported most frequently in association with a Becker's nevus, but there are also reports of AMSH alone. [8]

Histologically, there is a marked increase of smooth muscle fibers in the dermis, especially in its deep portion. The fibers are grouped in sharply circumscribed bundles that are arranged haphazardly. They are not necessarily attached to hair follicles. There may be basal hyperpigmentation, as well as acanthosis and papillomatosis of the overlying epidermis. [16]

Histochemical stains such as Masson's trichrome and/or immunohistochemical (IHC) stains such as smooth muscle actin, muscle-specific actin, desmin and electron microscopic studies may confirm the smooth muscle nature of the proliferation. [1],[18]

Clinical diagnosis of SMH is difficult, because of the lack of specific diagnostic criteria. The disorder must be suspected in any congenital hairy lesion, especially in the lumbosacral area. [1]

Treatment of SMH is not necessary, but involves surgical excision, if desired. [1],[8],[9],[12]

 
   References Top

1.Holst VA, Junkins-Hopkins JM, Elenitsas R. Cutaneous smooth muscle neoplasms: Clinical features, histologic findings and treatment options. J Am Acad Dermatol 2002;46:477-90.  Back to cited text no. 1    
2.Garcia Muret MP, Puig L, Pujol RM, Matias-Guiu X, Alomar A. Non-congenital smooth muscle hamartoma: 3 cases. Ann Dermatol Venereol 2002;129:417-20.  Back to cited text no. 2    
3.Stokes JH. Nevus pilaris with hyperplasia of nonstriated muscle. Arch Dermatol Syph 1923;7:479-81.  Back to cited text no. 3    
4.Sourreil MMP, Beylot MC, Delfour MM. Hamertome par hyperplasie des muscles arecteurs des poils chez un nourrisson dún mois. Bull Soc Fr Dermatol Syphiligr 1969;76:602.  Back to cited text no. 4    
5.Viglizzo G, Nemelka O, Nozza P, Occella C, Rongioletti F. Congenital smooth muscle hamartoma presenting with an unusual pseudo-Darier's sign. Clin Exp Dermatol 2006;31:148-9.  Back to cited text no. 5    
6.Zvulunov A, Rotem A, Merlob P, Metzker A. Congenital smooth muscle hamartoma. Am J Dis Child 1990;144:782-4.  Back to cited text no. 6    
7.Guillot B, Huet P, Joujoux JM, Lorette G. Multiple congenital smooth muscle hamartoma. Ann Dermatol Venereol 1998;125:118-20.  Back to cited text no. 7    
8.Gualandri L, Cambiaghi S, Eramacora E, Tadini G, Gianotti R, Caputo R. Multiple familial smooth muscle hamartomas. Pediatr Dermatol 2001;18:17-20.  Back to cited text no. 8    
9.Hsiao GH, Chen JS. Acquired genital smooth muscle hamartoma. Am J Dermatopathol 1995;17:67-70.  Back to cited text no. 9    
10.Metzker A, Amir J, Rotem A, Merlob P. Congenital smooth muscle hamartoma of the skin. Pediatr Deramatol 1984;2:45-8.  Back to cited text no. 10    
11.Morales-Callaghan A, Vila JB, Cardenal EA, Bernier MA, Fraile HA, Miranda-Romero A. Acquired cutaneous smooth muscle hamartoma. J Eur Acad Dermatol Venereol 2005;19:142-3.  Back to cited text no. 11    
12.Johnson MD, Jacobs AH. Congenital smooth muscle hamartoma: A report of six cases and a review of the literature. Arch Dermatol 1989;125:820-2.  Back to cited text no. 12    
13.Slifman NR, Harrist TJ, Rhodes AR. Congenital arrector pili hamartoma: A case report and review of the spectrum of Becker's melanosis and pilar smooth-muscle hamartoma. Arch Dermatol 1985;121:1034-7.  Back to cited text no. 13    
14.Fine HL, Possick PA, Myrow RE. Myokymia vs transient rippling of the skin. Arch Dermatol 1976;112:554.  Back to cited text no. 14    
15.White LE, Levy RM, Alam M. Neoplasias and Hyperplasias of mascular and Neural Origin. In: Klaus W, Lowell A, Goldsmith S, et al , editors. Fitzpatrick's Dermatology in general medicine. Chapter 127. 2008. p. 1174.  Back to cited text no. 15    
16.Atherton DJ, Moss C. Naevi and other developmental defects. In: Burns T, Breat S, Cox N, Griffiths C, editors. Rook's textbook of dermatology. Chapter 15. 7th ed. Blackwell; 2004. p. 33-4.  Back to cited text no. 16    
17.Schnur RE, Herzberg AJ, Spinner N, Kant JA, Magnusson M, McDonald-McGinn D, et al . Variability in the Michelin tire syndrome: A child with multiple anomalies, smooth muscle hamartoma, and familial paracentric inversion of chromosome 7q. J Am Acad Dermatol 1993;28:364-70.  Back to cited text no. 17    
18.Goldman MP, Kaplan RP, Heng MC. Congenital smooth-muscle hamartoma. Int J Dermatol 1987;26:448-52.  Back to cited text no. 18    
19.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. 19    
20.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. 20    
21.Thappa DM, Sirka CS, Srikanth S. Smooth muscle hamartoma associated with bilateral Becker's nevus. Indian J Dermatol Venereol Leprol 1997;63:387-9.  Back to cited text no. 21    Medknow Journal
22.Spencer JM, Amonette RA. Tumors with smooth muscle differentiation. Dermatol Surg 1996;22:761-8.  Back to cited text no. 22    
23.Berger TG, Levin MW. Congenital smooth muscle hamartoma. J Am Acad Dermatol 1984;11:709-12.  Back to cited text no. 23    
24.Gagnι EJ, Su WP. Congenital smooth muscle hamartoma of the skin. Pediatr Dermatol 1993;10:142-5.  Back to cited text no. 24    
25.Darling TN, Kamino H, Murray JC. Acquired cutaneous smooth muscle hamartoma. J Am Acad Dermatol 1993;28:844-5.  Back to cited text no. 25    
26.ul Bari A, Rahman SB. Acquired smooth muscle hamartoma. Indian J Dermatol Venereol Leprol 2006;72:178.  Back to cited text no. 26  [PUBMED]  Medknow Journal
27.Palit A, Inamadar AC. Circumferential skin folds in a child: A case of Michelin tire baby syndrome. Indian J dermatol Venerol Leprol 2007;73:49-51.  Back to cited text no. 27    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]

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    Abstract
    Introduction
    Case Report
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