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Year : 2013  |  Volume : 58  |  Issue : 4  |  Page : 329
A case of senear-usher syndrome


1 Department of Dermatology, Dr. D. Y. Patil Medical College and Hospital, Pimpri, Pune, India
2 Department of Pathology, Dr. D. Y. Patil Medical College and Hospital, Pimpri, Pune, India

Date of Web Publication25-Jun-2013

Correspondence Address:
Sonal A Chavan
Department of Dermatology, Dr. D. Y. Patil Medical College and Hospital, Pimpri, Pune
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.114009

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How to cite this article:
Chavan SA, Sharma YK, Deo K, Buch AC. A case of senear-usher syndrome. Indian J Dermatol 2013;58:329

How to cite this URL:
Chavan SA, Sharma YK, Deo K, Buch AC. A case of senear-usher syndrome. Indian J Dermatol [serial online] 2013 [cited 2019 Aug 21];58:329. Available from: http://www.e-ijd.org/text.asp?2013/58/4/329/114009


Sir,

A 32-year-old female, presented with hyperpigmented papules of three months duration that commenced as erythematous papules over cheeks, ears, scalp, upper back, "V" of the chest and extensors of forearms and dorsae of hands. Lesions were associated with burning on sun exposure. Painful oral erosions interfered with food intake. Arthralgia of interphalangeal joints, knees and ankles with morning stiffness accompanied the skin changes.

Cutaneous examination revealed crusted scaly plaques with surrounding erythema over malar area [Figure 1]a, ears, chest, back and sun exposed areas of forearms and arms. Oral cavity showed erosions over upper gingiva [Figure 1]b. Hands showed proximal nail fold erythema [Figure 2]a.
Figure 1

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Figure 2

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Value of serum ANA was 2.87 IU, ESR was 40 mm, Hb was 9.3 gm% and RA factor was positive. Chest X-ray, ECG, urinalysis and anti-ds DNA were normal. Skin biopsy of lesional skin from right forearm showed hyperkeratosis, subcorneal acantholysis [Figure 3]a, basal cell vacuolation, dermoepidermal separation and superficial perivascular lymphocytic infiltrate [Figure 3]b. Direct IF of perilesional skin from right forearm revealed focal deposits of IgG [Figure 4]a in the intercellular space of the epidermis and granular deposits of IgG and IgM at dermoepidermal junction [Figure 4]b.
Figure 3

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Figure 4

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Dexamethasone-cyclophosphamide pulse therapy was administered and since five pulses, the patient has been in remission [Figure 2]b.

Senear-Usher syndrome is a disorder which results from combination of lupus erythematosus and pemphigus foliaceus. [1] Mucous membrane involvement is uncommon. [2]

The relationship of this syndrome with lupus erythematosus and its place in pemphigus spectrum has long been debated. Some authors think it to be an abortive or localized form, or an early stage of pemphigus foliaceus, others argue that it may progress to pemphigus vulgaris and pemphigus foliaceus. [1] Jablonskaetal. [3] in their direct immunofluorescence (IF) study of fifty four patients found staining of the intercellular epidermal spaces. DEJ band was seen in exposed and unexposed skin in 81% and 23% of their patients respectively. ANA were found in 31% of patients. Detection of IF band in skin specimens from a significant majority of patients with pemphigus erythematosus, presence of ANA in some, and occasional coexistence of SLE suggest some relation of the disease with lupus erythematosus. Bean and Lynch [3] reported a case with widespread lesions which showed direct and indirect IF findings of superficial pemphigus along with IgG and complement at DEJ. Our case not only had features suggestive of pemphigus foliaceus (subcorneal acantholysis and focal deposits of IgG in the intercellular space of superficial epidermis) but also of lupus erythematosus (vacuolar degeneration of basal keratinocytes and granular deposits of IgG and IgM at DEJ).

 
   References Top

1.Bean SF, Lynch FW. Senear-Usher syndrome (pemphigus erythematosus). Immunofluorescent studies in a patient. Arch Dermatol 1970;101:642-5.  Back to cited text no. 1
    
2.Maize JC, Green D, Provost TT. Pemphigus foliaceus: a case with serologic features of Senear-Usher syndrome and other autoimmune abnormalities. J Am Acad Dermatol 1982;7:736-41.  Back to cited text no. 2
    
3.Jabloñska S, Chorzelski T, Blaszczyk M, Maciejewski W. Pathogenesis of pemphigus erythematosus. Arch Dermatol Res 1977;258:135-40.  Back to cited text no. 3
    


    Figures

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



 

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