Indexed with PubMed and Science Citation Index (E) 
Users online: 16497 
     Home | Feedback | Login 
About Current Issue Archive Ahead of print Search Instructions Online Submission Subscribe What's New Contact  
  Navigate here 
   Next article
   Previous article 
   Table of Contents
 Resource links
   Similar in PUBMED
    Search Pubmed for
    Search in Google Scholar for
  Related articles
   [PDF Not available] *
   Citation Manager
   Access Statistics
   Reader Comments
   Email Alert *
   Add to My List *
* Registration required (free)  
  In this article
    Case Report

 Article Access Statistics
    PDF Downloaded0    
    Comments [Add]    

Recommend this journal

Year : 2003  |  Volume : 69  |  Issue : 7  |  Page : 37-38

Papuloerythroderma of ofuji-is it a homogeneous entity?

Department of Dermatology, Venereology & Leprology, Mahatma Gandhi National Institute of Medical Sciences, Sitapura, Jaipur.

Correspondence Address:
C-118, Shivaji Marg, Tilak Nagar, Jaipur-302 004


A case of papuloerythroderma of Ofuji, who presented with erythroderma comprising of multiple coalescing papules, deck chair sign and eosinophilia, is reported. Parior case reports of this syndrome have variably mentioned associated features like lymphadenopathy, nailfold and buttock infarcts and palmoplantar keratoderma, visceral malignancies, laboratory features like circulating Sezary cells, and biopsy findings like exocytosis, vessel wall thickeningor extravasation of red blood cells, which were not evident in this patient. This inconsistency in findings points towards heterogeneous nature of the disorder.

How to cite this article:
Gulati R. Papuloerythroderma of ofuji-is it a homogeneous entity?. Indian J Dermatol Venereol Leprol 2003;69, Suppl S1:37-8

How to cite this URL:
Gulati R. Papuloerythroderma of ofuji-is it a homogeneous entity?. Indian J Dermatol Venereol Leprol [serial online] 2003 [cited 2020 Nov 30];69, Suppl S1:37-8. Available from:

   Introduction Top

Papuloerythroderma of Ofuji is a rare intensely pruritic generalized eruption of unknown etiology characterized by peripheral eosinophilia and typical deck chair sign.[1] Largely a clinical diagnosis, the disorder has also been shown to be variably associated with lymphopenia, high serum IgE level, Langerhans cells in the dermis, and even circulating Sezary cells.[2] Biopsy usually reveals a mixed upper dermal infiltrate, mostly comprising of lymphocytes, histiocytes and eosinophils. Variable pathological changes are also evident in the epidermis and vessel walls in upper dermis. Following is description of a similar case with some variation from the usual.

   Case Report Top

A 70-year-old man presented in the Dermatology out patient department with a generalized erythematous eruption associated with intense itching. The eruption had started on the trunk ten years back, and within a span of two years had covered the entire body.
Cutaneous examination revealed erythroderma composed of coalescing papular lesions, but remarkably sparing the body folds, especially anterior transverse abdominal ones - the typical deck chair sign. Excoriations were a pointer towards itchy nature of the lesions. There was no lymphadenopathy or cutaneous infarcts, though few lesions of senile purpura were evident on the dorsa of forearms.
Laboratory investigations showed marked eosinophilia of 11%, while serum IgE level was in normal range. Skin biopsy from the anterior abdominal wall showed hyperkeratosis, acanthosis, focal spongiosis, moderate upper dermal infiltrate composed of lymphocytes and few eosinophils, and mild vessel wall dilatation. No exocytosis, vessel wall thickening or extravasation of red blood cells as seen. In the past, the patient had intermittently taken treatment, the nature of which was unknown. He was treated with oral prednisolone 40 mg daily (gradually tapered), antihistamines, topical betamethasone dipropionate and emollients. Improvement was evident in the form of partial remission of eruption at the end of one month.

   Discussion Top

The presence of papuloerythroderma, deck chair sign and eosinophilia in the above case favour the diagnosis of Ofuji's disease. But contrary to the cases reported before, this patient had no evidence of high serum IgE level, lymphopenia, exocytosis, vessel wall thickening or extravasation of red blood cells. Apart from this, associated variable clinical features like lymphadenopathy, nailfold and buttock infarcts and palmoplantar keratoderma, visceral malignancies, and laboratory features like circulating Sezary cells, as mentioned by many authors, were also absent.[3],[4],[5] This points to heterogeneity of the disease, which tends to manifest in more than one form, or may be the cases represent variants of a single disorder. This question will remain unanswered till the root cause of this rare enigmatic entity comes to light. 

   References Top

1.Ofuji S, Furukawa F, Miyachi Y et al. Papuloerythroderma. Dermatologica 1984; 169;125-130.  Back to cited text no. 1    
2.Tay YK, Tan KC, Wong WK et al. Papuloerythroderma of Ofuji: a report of three cases and review of the literature 1994; 130:773-776.  Back to cited text no. 2    
3.Staughton R, Langtry J, Rowland Payne C. Papuloerythroderma: the first European case. In:Clinical dermatology, Edited by Wilkinson DS et al., Stuttgart: Schattauer, 1987; 181-182.  Back to cited text no. 3    
4.Ofuji S. Papuloerythroderma. J Am Acad Dermatol 1990; 22:697.  Back to cited text no. 4    
5.Grob JJ, Collet-Villette AM, Horchowski N, et al. Ofuji papuloerythroderma. Report of a case with T-cell skin lymphoma and discussion of the nature of this disease. J Am Acad Dermatol 1989; 20:927-931.  Back to cited text no. 5    


Print this article  Email this article
Previous article Next article


Online since 15th March '04
Published by Wolters Kluwer - Medknow