lipoatrophy , also termed lipodystrophy , is a rare condition presenting as a loss of subcutaneous fat . total lipodystrophy can be congenital or acquired and is usually associated with other clinical features such as hyperpigmentation , acanthosis nigricans , diabetes mellitus , glomerulonephritis , and hepatopathy . for further information about inherited lipodystrophy syndromes visit " online mendelian inheritance in men " at http://www.ncbi.nlm.nih.gov/omim . an association with muscle fibre degeneration   and dermatomyositis   has already been described in the medical literature . a 20 year old male patient presented with a slowly progressive atrophy of his right buttocks region . personal history revealed a ck elevation of unknown origin , family history was non - contributory . physical examination demonstrated a focal atrophy of his proximal right lower extremity , distinctly pronounced in the gluteal region ( figure 1a ) . muscle strength was normal in the whole system ; deep - tendon reflexes were unremarkable . routine laboratory examinations revealed no abnormalities , the creatine - kinase level was normal ( 88 u / l ; norm < 110 u / l ) . muscle magnetic resonance imaging revealed a severe subcutaneous and intramuscular lipoatrophy in the clinically affected region ( figure 1b ) . electromyography showed slight myopathic changes in the right gluteus medius and maximus muscle ( s ) , but a normal pattern in the clinically unaffected musculature ( left vastus medialis , right tibialis anterior ) ; there was no evidence for a l5/s1 radiculopathy . a muscle biopsy from the electromyographically affected right gluteus maximus and the unaffected left vastus lateralis muscle was performed . routine histological stainings included h&e , modified gomori - trichrome , acid - phosphatase , nadh , cytochrom - c - oxidase , succinate - dehydrogenase , myoadenylate - desaminase , oil - red - o , perjodacid - schiff , and atpases . immuno - histochemical stainings included cd4 , cd8 , mhc1 , mhc2 , c5b9 , cd68 , dystrophin , dysferlin , sarcoglycanes , lamin a / c , and theletonin . h&e staining demonstrated focal endomyseal inflammatory infiltrations in both muscles , pronounced in the right gluteus maximus ( figure 2 a - c ) and to a lesser extent in the left vastus lateralis ( figure 2 d - f ) . immuno - histochemical analysis proved pathological mhc1 expression and a predominance of cd4 over cd8 positive lymphocytes . all other stainings were unremarkable ; especially there was no evidence for lamin a / c deficiency . in summary , the patient showed a localized unilateral lipoatrophy in association with a bilateral myositic process . therapeutically , oral prednisolone therapy ( initially 80 mg / day ) was initiated , but without obvious clinical effect after 6 month of treating . one month after corticoid - therapy , a second biopsy from right gluteus maximus muscle was performed for therapy evaluation and to exclude artificial induced inflammatory changes by needle emg in the first biopsy : in summary , the same histological picture as in the first biopsy was seen , i.e. inflammatory infiltrations in comparable extend . a : posterior view of patient demonstrates focal loss of subcutaneous fat overlying the right gluteus muscle . b : muscle mri ( t2 weighted image ) demonstrating severe localized lipoatrophy . skeletal muscle biopsy . a - c : right gluteus maximus ( a : hematoxylin - eosin 1:20 ; b : cd4 immunostaining 1:20 ; c : cd8 immunostaining 1:20 ) : massive endomyseal inflammatory infiltration demonstrated in he staining . immunohistochemical analysis showed cd4 positive lymphocytes in the region of the infiltration , but also cd8 positive cells endomyseal . d - f : left vastus latealis ( d : he 1:20 , e : cd4 1:20 ; f : cd8 1:20 ) : he staining revealed a more slightly inflammatory infiltration compared with the findings in the electromyographically affected right gluteus maximus ( a ) , but also a predominance of cd4 positive cells . to date , histologically confirmed focal or multifocal myositis has been reported in three cases of localized lipoatrophy : in 1982 , palliyath and garcia described a 54 year old man with a multifocal interstitial myositis associated with localized lipoatrophy isolated in the region of the right buttock . the clinical picture and the histological changes were not progressive over a period of 30 years , so the authors concluded a benign course of this hitherto not described condition . betteley reported a localized atrophy of the subcutaneous fat of the left buttock in a 12 year old boy . in this patient , ipsilateral gluteal muscles were weak , histological examination revealed perivascular mono - nuclear cell infiltration of the skin . finally , in 1994 , creange and colleagues   described a 24 years old man with severe muscle cramps , fasciculations and atrophy localized to the right lower limb . computed tomography revealed localized a right quadriceps femoris muscle biopsy showed a pronounced infiltration of the interstitial tissue by mononuclear cells , predominating in the walls of small vessels , and around them . the patient described by palliyath and colleague   was treated with prednisone 60 mg per day for three weeks without benefit , like our patient . creange et al . did not treat their patient with steroids . whereas the patient of palliyath and colleagues demonstrated mild weakness of the right shoulder girdle musculature , right quadriceps , and gastrocnemius , considering the rarity of both disorders ( focal myositis and localized lipoatrophy ) , creange et al in our opinion it is difficult to speculate on a causative pathophysiological mechanism of this association , but it seems to be possible that this association represents an unrecognized condition with a benign disease course but absent response to steroid treatment . due to a psychological strain of the patients ,
lipodystrophies represent a heterogeneous group of diseases characterized by altered body fat repartition and often metabolic alterations . here 
 we illustrate a 20 year old male with myositis in association with localized lipodystrophy . 
 immunohistochemical stainings revealed a regular pattern of dystrophin , dysferlin , sarcoglycans , and theletonin . 
 furtermore , there was no evidence of lamin a / c deficiency . 
 a nearly identical clinical and histological picture has been described in three patients up to now . 
 although it is difficult to speculate on a causative pathophysiological mechanism at this time , it is possible that this association represents an unrecognized condition .