darier 's disease is an autosomal dominant disorder of keratinization affecting the skin , mucosa , and nails that typically manifests in the first and second decade of life .
we describe an elderly man with nonmetastatic gastric adenocarcinoma presenting with an acquired variant of darier 's disease presumably of paraneoplastic origin .
a 62-year - old muslim farmer was in otherwise good health until 2 months back when he suddenly started noticing some dark - colored , mildly itchy skin eruption .
he also complained of nausea , vomiting , and loss of appetite and a single episode of passage of black , tarry stool . on examination of his skin , numerous hyperpigmented
, warty papules were seen over the face , neck , trunk , and scalp .
the lesions were characteristically distributed in seborrheic areas like head  neck area , back , and front of chest [ figure 1 ] .
there was punctate keratoderma of palms and soles with a few palmar pits as well [ figure 2 ] .
v-like notch at the free edge of the nail plate without longitudinal streaks . on the basis of these clinical features ,
there was neither a past history of similar lesions nor any family history of the same .
dirty , warty papules over the back palms showing pits and keratoderma histopathology of the skin lesions revealed suprabasal acantholytic cleft and dyskeratotic cells like corps ronds and grains [ figure 3a and b ] .
diminution of lesions following tumor resection ( a ) on back ; ( b ) on chest and palm he was referred to the gastroenterology department where after a series of investigations including endoscopic biopsy , he was diagnosed with gastric adenocarcinoma . on his next follow - up in our outpatient department ( opd ) 2 months later , we discovered that the skin lesions had remarkably diminished [ figure 4a and b ] . by that time
it was then that we retrospectively considered darier 's disease to be a paraneoplastic phenomenon .
histopathology showing ( a ) prominent hyperkeratosis and suprabasal acantholytic cleft with villi ( h and e , 100 ) ; ( b ) dyskeratotic cells ( h and e , 400 )
paraneoplastic dermatoses describes those benign skin changes in which there is a direct , often parallel course of a dermatosis with an underlying malignancy .
there are several proposed hypotheses on the pathomechanism of paraneoplastic dermatoses , which highlight the role of various tumor - derived growth factors like transforming growth factor  ( tgf- ) , fibroblast growth factor , and so on .
regarding the pathogenesis of paraneoplastic darier 's disease , it can be speculated that the tumor product interferes with keratinocyte calcium homeostasis leading to disturbances in intracellular trafficking of desmosomal proteins .
the autosomal dominant variety is due to mutations in the serca2 ( sarco / endoplasmic reticulum calcium atpase type 2 ) , which regulates calcium homoestasis in the endoplasmic reticulum .
our case involves an acquired form of darier 's disease whose diagnosis was supported both clinically and histopathologically .
although there are several variants of darier 's disease , the paraneoplastic variety is extremely rare .
there is only a single case report on its association with metastatic papillary carcinoma of thyroid .
there are some sporadic reports on the occurrence of squamous cell carcinoma of skin , nail bed , esophagus , mouth , and vagina in patients with pre - existing darier 's disease but paraneoplastic association is very rare .
according to the criteria proposed by helen ollendorff curth , a specific dermatosis occurs with a specific neoplasm , and a high percentage of association between two conditions is noted . this does not hold true in our case .
the paraneoplastic dermatoses that have been reportedly associated with gastric adenocarcinoma are acanthosis nigricans , tripe palms , and florid cutaneous papillomatoses .
we consider this a paraneoplastic process due to its concurrent onset and almost parallel course with malignant neoplasm , which are also important requisites in the curth criteria .
moreover , in this case , onset of darier 's disease was late , occurring in the seventh decade of life in contrast to its usual onset in the second decade of life . also the skin changes resolved with treatment of the underlying cancer .
this further served as an eye - opener for the clinical investigators of this case .
its paraneoplastic association with gastric adenocarcinoma is very unusual and not known to be mentioned in any dermatology literature so far and hence reported here .
darier 's disease is an unusual paraneoplastic manifestation in gastric malignancy , not reported before .
 darier 's disease is an autosomal dominant genodermatosis resulting from atp2a2 gene mutation . a 62-year - old male presented at our outpatient ( opd ) with sudden - onset numerous dirty , warty papules over the head , neck , and back since 2 months . 
 histopathology of the skin lesions revealed acantholytic dyskeratosis suggestive of darier 's disease . 
 he was referred to the gastroenterology department for some gastrointestinal ( gi ) symptoms where he was diagnosed with adenocarcinoma of the stomach and was subsequently operated . on his next visit to our department for follow - up , we found a marked diminution of the skin lesions in the absence of any specific treatment . in view of the above finding 
 , we concluded that paraneoplastic dermatosis in the form of darier 's disease occurred in this patient . 
 paraneoplastic darier 's disease with gastric adenocarcinoma is not yet described in dermatology literature and is hence reported here . 