pyoderma gangrenosusm ( pg ) is a chronic , rare , inflammatory cutaneous disorder and is manifested by the development of painful ulcers , bulla , pustules and rarely vegetating lesions and histologically by predominantly neutrophilic infiltration .
we report a case of severe pg in a girl since her 4 months of age . the difficulty in diagnosis and methodical approach in such cases is highlighted .
a 6-month - old hindu girl presented with progressive , extensive , and painful ulcers since her 4 months of age .
ulcers developed from erythematous papules and vesicles , had purple red undermined margin , raw or crusted surface , merged with other ulcers in the neighborhood and formed large ( even > 8 cm ) lesions with annular , polycyclic or crescent shape .
they were distributed over scalp , face , ear , trunk ( less over anterior trunk ) , buttocks , thigh , legs , dorsum of hands and feet without any mucosal involvement [ figure 1 ] .
( a ) face , ( b ) arm , ( c ) dorsum of hand , ( d ) leg , ( e ) thigh , ( f ) back , ( g ) encircled site where intradermal normal saline was injected , ( h ) same site after 48 h showing pustulation ( positive pathergy test ) the child was playful with normal physical and intellectual growth .
there was mild fever , moderate pallor without any cyanosis , clubbing , organomegaly , lymphadenopathy , or joint abnormality .
pustule developed following intradermal injection of normal saline on normal skin ( positive pathergy test ) [ figure 1 ( g , h ) ] .
there was low hemoglobin ( 8.9 gm / dl ) , leucocytosis ( 22,600/cmm ) , neutrophilia ( 70% ) , and elevated sedimentation rate ( 69 mmhg ) .
chest x - ray , mantoux test , dna - pcr for tuberculosis , immunoglobulin level , ra factor , ana ( hep-2 cell ) , vdrl , c - anca , hiv- elisa , pus culture , and colonoscopy of rectal mucosa revealed no abnormality .
histopathology of the skin biopsies with h and e stain from the margin of the ulcer from two different sites showed epidermal ulceration and dense dermoepidermal collection of inflammatory exudates consisting predominantly of neutrophils with occasional multinucleated giant cells [ figure 2 ] .
one of the biopsies also showed foci of granulomatous reaction [ figure 2 , inset ] .
z - n stain ( for afb ) and pas stain ( for fungus ) were negative .
photomicrograph showing ulceration of epidermis and collection of inflammatory exudates along with occasional giant cells ( h and e , 100 ) .
inset showing ill formed granuloma with giant cells ( h and e , 400 ) it was diagnosed as a case of pyoderma gangrenosum .
the patient responded satisfactorily to oral prednisolone ( 2 mg / kg / day ) .
however , there were signs of relapse with dose less than 1 mg / kg / day .
at that time , the patient became irregular in follow - up and finally lost to follow up .
in most of the cases , pg is associated with some systemic diseases like inflammatory bowel diseases ( ibd ) , rheumatoid arthritis , myeloproliferative disease , etc .
extensive distribution of the disease was unusual for an infant with pg who generally have ulcers on head and buttocks .
onset at 4 months of age was extremely rare and possibly not reported so far .
due to much atypicality , diagnosis was difficult and many possibilities were considered like pg , tuberculous , atypical mycobacterial infection , fungal , malignant and vasculitic ulcers , wegener 's granulomatosis , sweet 's syndrome , and epidermolysis bullosa .
violaceous undermined margin with erythematous halo , pain , characteristic histological appearance and positive pathergy test were helpful for diagnosis .
multiple site colonoscopic biopsies from even normal rectal mucosa could have increased the diagnostic yield for any subclinical evidence of ibd . the case
 a female child developed multiple , progressive , therapy - resistant , painful large ulcers , vesicles , and pustules since her 4 months of age . 
 the ulcers were large , some even measured more than 8 cm ; most had violaceous undermined margin with surrounding erythematous halo , raw and crusted surface and were distributed extensively over scalp , face , ear , trunk , buttocks , thigh , legs , dorsum of hands , and feet without any mucosal involvement . 
 after detail clinical examination and investigation , it was diagnosed as a case of pyoderma gangrenosum . 
 extensive search did not reveal any systemic abnormality or detect any infective etiology . 
 the case highlights the problems of diagnosis of multiple ulcers at very early age . 