Hansen's disease with McCune–Albright syndrome
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 and ]. Cutaneous examination revealed a large CALM over the back, extending across the midline and multiple anesthetic patches over scapula and lower back []. There was no evidence of goiter, acromegaloid features, and thickened nerves, and the rest of the examination was normal.\nHormonal 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 99mTc-MDP scan of the bone were not considered necessary to establish the diagnosis in this classical case. He was diagnosed as a case of Hansen's disease with MAS. 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. He is under regular follow-up for the skin and bone condition.