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CASE REPORT
Year : 2013  |  Volume : 2  |  Issue : 1  |  Page : 49-51

Zosteriform papular mucinosis: A rare entity


1 Department of Dermatology Venereology and Leprology, GSL Medical College and General Hospital, Rajahmundry, India
2 Consultant Pathologist, Chaitanya Medical Centre, Visakhapatnam, India
3 Consultant Dermatologist, Surya Skin Care and Research Centre, Visakhapatnam, India

Date of Web Publication13-Mar-2013

Correspondence Address:
G Raghurama Rao
Department of DVL, GSL Medical College and General Hospital, Rajahmundry, Andhra Pradesh - 533 296
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2277-8632.108514

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  Abstract 

Papular mucinosis is a relatively uncommon condition. We report a 50-year-old female patient who presented with asymptomatic skin colored lesions in a segmental pattern involving the T3 and T4 segments of the left side. Lesional biopsy (H and E) showed a sparse superficial perivascular lymphocytic infiltrate with abundant mucin throughout the reticular dermis. Special staining with Alcian blue confirmed mucinosis.

Keywords: Alcian blue, papular mucinosis, segmental


How to cite this article:
Sowjanya C L, Phaneendra Prasad A K, Sandeep K, Rao G R, Koteswarao N R, Sridevi M. Zosteriform papular mucinosis: A rare entity. J NTR Univ Health Sci 2013;2:49-51

How to cite this URL:
Sowjanya C L, Phaneendra Prasad A K, Sandeep K, Rao G R, Koteswarao N R, Sridevi M. Zosteriform papular mucinosis: A rare entity. J NTR Univ Health Sci [serial online] 2013 [cited 2020 Apr 7];2:49-51. Available from: http://www.jdrntruhs.org/text.asp?2013/2/1/49/108514


  Introduction Top


Papular mucinosis (PM) or lichen myxedematosis (LM) is a chronic idiopathic disorder characterized by lichenoid papules, nodules, and plaques caused by dermal mucin deposition and a variable degree of fibrosis in the absence of thyroid disease. LM was first described by Dubreuilh in 1906 and Reitmann in 1908 and later distinguished from scleroderma and generalized myxedema by Montgomery and Underwood in 1953. [1] Recently, Franco Rongioletti broadly classified PM into generalized papular and sclerodermoid type and localized forms. [2] Segmental PM is a rare subtype and only one case was reported in the literature.


  Case Report Top


A 50-year-old healthy female presented with multiple asymptomatic skin colored lesions on left scapular area of 5 months duration. The lesions initially started at the midline on the left side and gradually spread along the back up to the left breast region. There were no constitutional symptoms. The patient's general condition was good. All vitals were within normal limits. On examination, multiple skin colored, nontender waxy papules, and plaques were seen in a segmental pattern involving T3 and T4 segments [Figure 1]a and b and few discrete lesions were seen on the left breast. There were no lesions elsewhere on the body. No regional lymphadenopthy. Systemic examination was normal. Differential diagnosis considered were cutaneous metastasis, xanthomas, segmental steatocystoma multiplex, and keloidal Herpes Zoster. Relevant hematological and biochemical investigations were within normal limits. Human immunodeficiency virus (HIV) serology and hepatitis C serology were negative. Skiagram chest and ultrasound abdomen were normal. Lesional biopsy was done and on gross examination, the specimen showed strings of cheesy material. On hematoxylin and eosin staining, specimen showed a sparse superficial perivascular lymphocytic infiltrate with abundant mucin throughout the reticular dermis. The reticular dermis shows an increased number of fibrocytes and increased amount spaces between collagen bundles [Figure 2]. Alcian blue stain confirmed mucin deposits in the reticular dermis [Figure 3]. A final diagnosis of segmental or zosteriform PM was made based on clinical and histopathological findings. She was treated with cryotherapy and topical pimecrolimus ointment. After 2months of therapy, regression of lesions was noticed.
Figure 1: (a and b) Segmental waxy papules and plaques on the left side, not crossing the midline

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Figure 2: Superfi cial perivascular lymphocytic infi ltrate with mucin deposits throughout the reticular dermis with increased spaces among the collagen bundles (H and E, ×10)

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Figure 3: Alcian blue staining of the same section shows blue stained mucin in the reticular dermis (×10)

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  Discussion Top


LM or PM is a relatively uncommon condition, seen less frequently when compared with generalized and sclerodermoid form. Localized LM or PM is subdivided into 5 types: A discrete form, acral persistent PM, self healing PM, PM of infancy, and a pure nodular form. [2] The criteria to diagnose localized LM are (1) a papular eruption or nodules and plaques, (2) mucin deposition with a variable fibroblast proliferation, and (3) the absence of both monoclonal gammopathy and thyroid disease. Clinically the patients exhibit small, firm, waxy papules or nodules and plaques confined to few sites only, usually upper and lower limbs and trunk. The lesions progress slowly and rarely resolve spontaneously. Some cases may resolve spontaneously without sequelae in a period ranging from few weeks to months. Localized forms are seen in all age groups and a female preponderance is seen. Localized LM may also be observed in association with HIV infection, exposure to toxic oil or L-tryptophan, and hepatitis C virus infection. [3]

The pathogenesis of LM is unknown and is usually limited to the skin and has a good prognosis. Microscopic features are less characteristic when compared with scleromyxedema. Features include mucin accumulation in the upper and mid reticular dermis, with variable fibroblast proliferation and fibrosis is not marked and may even be absent. [4]

Localized LM does not require therapy and a wait-and-see approach is recommended. Topical steroids may be of benefit. A single case of resolution with pimecrolimus has been reported. One patient with associated HIV infection had complete remission after treatment with oral isotretinoin. However, spontaneous resolution may occur. [3]

Cases of segmental PM are very rare and only one case of cutaneous mucin deposition, which initially developed as papules and nodules in a segmental distribution pattern and gradually changed to brownish sclerotic macules has been reported. [5] In the present case, PM lesions initially developed on the left side of back and within 5 months, groups of papules and plaques developed in a zosteriform pattern extending onto the left breast. A rare case of acral persistent PM associated with multiple myeloma was reported. [6] We propose this PM with segmental or zosteriform distribution as a novel subgroup.

 
  References Top

1.Cokonis Georgakis CD, Falasca G, Georgakis A, Heymann WR. Sclermyxedema. Clin Dermatol 2006;24:493-7.  Back to cited text no. 1
    
2.Rongioletti F, Rebora A. Updated classification of papular mucinosis, lichen myxedematosus and scleromyxedema. J Am Acad Dermatol 2001;44:273-81.  Back to cited text no. 2
    
3.Rongioletti F. Lichen Myxedematosus (Papular Mucinosis): New Concepts and Perspectives for an Old Disease. Semin Cutan Med Surg 2006;25:100-4.  Back to cited text no. 3
    
4.Rongioletti F, Rebora A. Cutaneous mucinosis: Microscopic criteria for diagnosis. Am J Dermatopathol 2001;23:257-67.   Back to cited text no. 4
    
5.Hayashi Y, Araki E, Yagi Y, Yoshiki M, Utani A. Segmentally-arranged cutaneous mucinosis ranging from papulonodules to sclerotic pigmented macules. Eur J Dermatol 2010;20:136-7.  Back to cited text no. 5
    
6.Ahmad M, Siraj F, Ahmad M, Ahmad S. Acral persistent papular mucinosis associated with multiple myeloma- unknown presentation. JMS 2012;15:74-5.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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