fibrous dysplasia of bone is characterized by the replacement of medullary bone with fibro - osseous tissue .
this results in the distortion and overgrowth of bone , leading to characteristic deformities . based on the bony involvement , fibrous dysplasia
is divided into three types : monostotic , polyostotic , and mccune  albright syndrome ( mas ) .
mas is characterized by a triad of monostotic / polyostotic fibrous dysplasia , caf-au - lait macules ( calms ) , and endocrine hyperfunction . the disease is congenital but not hereditary and is due to an activating mutation in g protein - coupled receptor .
it manifests usually in childhood , with recurrent fractures , distorted bones , precocious puberty , acromegaly , or hyperthyroidism .
untreated disease leads to permanent disfigurement , and the disease is classified as paucibacillary or multibacillary depending on the proliferation of the bacilli .
the risk factors for the disease are living in endemic areas with poor hygiene conditions and defects in cell - mediated immunity .
we recently encountered a patient with mas who presented with cutaneous features of hansen 's disease .
we report the same for the unusual association and to highlight the relevant review of the literature .
a 30-year - old man presented with hypoesthetic , depigmented patches over the skin of the chest and back of 1 year duration .
he gave history of recurrent fractures since the age of 4 years with shortening of right leg .
the parents gave history of hyperpigmented macules over the trunk since birth and denied similar features in family members .
the patient attained puberty at the age of 14 years and denies history suggestive of acral enlargement , thyrotoxicosis , cushing 's syndrome , and swellings in any part of the body .
examination revealed ( height 152 cm ) asymmetry of the face with expansion of right maxilla and lower jaw and short right leg ( 82 vs 86 cm ) [ figures 1 and 2 ] .
cutaneous examination revealed a large calm over the back , extending across the midline and multiple anesthetic patches over scapula and lower back [ figure 3 ] .
there was no evidence of goiter , acromegaloid features , and thickened nerves , and the rest of the examination was normal .
facial photograph showing craniofacial dysplasia legs showing short right leg ( arrow ) with expansile lesion of tibia ( arrow head ) skin over the back showing a large calm crossing midline ( long arrows ) with depigmented hypoesthetic patches ( short arrow ) hormonal profile revealed normal thyroid adrenal and gonadal axes evaluation .
skeletal survey revealed expansile osteolytic lesion over right maxilla , right hip , and shoulder .
skin biopsy specimens were obtained from ear lobes , legs , and depigmented lesions over the back , avoiding areas of calm .
specimens revealed the presence of acid - fast bacilli along with noncaseating granulomas consistent with the diagnosis of tuberculoid leprosy .
the diagnosis of fibrous dysplasia is made by the presence of bone pains , recurrent fractures , deformity of bone , and radiological evidence of fibro - osseous tissue showing expansile lytic lesion .
biopsy and tc - mdp scan of the bone were not considered necessary to establish the diagnosis in this classical case .
other laboratory workup showed elevated alkaline phosphatase ( 388 u / l ) with normal calcium , phosphorus , parathyroid hormone , and 25-hydroxy vitamin d levels .
he was treated with triple drug regimen for leprosy and also given oral calcium carbonate 1.5 g daily along with the first dose of zoledronic acid 4 mg as an intravenous infusion .
mas is characterized by the triad of polyostotic fibrous dysplasia , calm , and endocrinopathy .
the diagnosis of fibrous dysplasia is based on classical radiological findings supported by histopathology and bone scan .
hyperfunctioning of endocrine glands in mas presents with precocious puberty , hyperthyroidism , acromegaly , or cushing 's syndrome .
our patient had only two features of the triad and did not have any evidence of endocrine hyperfunction syndromes .
the calm is unusual in our patient in that it crossed the midline and was seen on both sides of the body .
the calms in mas do not cross the midline and follow the developmental lines of blashko , thus differentiating from those in neurofibromatosis .
our patient had an exceptionally large calm that crossed the midline interspersed with anesthetic depigmented macular lesions of hansen 's disease .
hansen 's disease is caused by mycobacterium leprae , and the disease spreads via respiratory droplets between close personal contacts over a prolonged duration .
immunocompromised situations are the predominant risk factors for the disease , and defects in cell - mediated immunity are implicated in persons with genetic predisposition .
the mode of acquisition of hansen 's disease in our patient is possibly due to frequent stay in the hospitals due to multiple fractures and contact with a patient .
screening of the persons involved in personal care of the patient did not reveal features of hansen 's disease .
the association of hansen 's disease with mas appears coincidental as there is no evidence that mas predisposes to the infectious diseases or leads to immunosuppressed state .
extensive literature search did not reveal any previous reports of association between hansen 's disease and mas .
they are given as intravenous or oral preparation and act by suppressing the osteoclast activation .
treatment of mas involves bisphosphonate therapy to reduce fracture rate apart from treating any associated endocrinopathy .
to conclude , we present an interesting case of mas without associated endocrinopathy who had coexisting hansen 's disease . this unusual combination was not reported earlier in world literature .
 mccune  albright syndrome ( mas ) comprises a triad of fibrous dysplasia of bone , caf - au - lait macule , and endocrinopathy . 
 the disease is due to activating mutation of g protein - coupled receptor leading to hyperfunction of glands . 
 hansen 's disease is caused by infection with mycobacterium leprae and is seen with underlying immunosuppressed conditions in genetically predisposed individuals . 
 we recently encountered a patient with hansen 's disease along with underlying mas and report the same in this report . 
INTRODUCTION
CASE REPORT
DISCUSSION
CONCLUSION