|Year : 1990 | Volume
| Issue : 4 | Page : 308-309
Generalised linear morphoea with bone cyst
PVS Prasad, R Mathai, Mary Jacob
Bone changes in morphoea are well recognised. However bone cyst in morphoea has been reported only once in the past. We describe a second case of bone syst associated with generalised linear morphoea.
Keywords: Morphoea, Bone cyst
|How to cite this article:|
Prasad P, Mathai R, Jacob M. Generalised linear morphoea with bone cyst. Indian J Dermatol Venereol Leprol 1990;56:308-9
|How to cite this URL:|
Prasad P, Mathai R, Jacob M. Generalised linear morphoea with bone cyst. Indian J Dermatol Venereol Leprol [serial online] 1990 [cited 2013 May 23];56:308-9. Available from: http://www.ijdvl.com/text.asp?1990/56/4/308/3555
Generalised linear morphoea is a rare disease in which sclerosis of the skin occurs in a widespread linear fashion. The disease affects females more than males and the commonly affected age group is between 3040 years. Bone lesions are the most frequent noncutaneous association of generalised linear morphoea. We report an association of a congenital bone cyst-in the humerus in a patient with linear morphoea since such an association has not so far been reported.
| Case Report|| |
A 21 year old male patient presented with a six year history of skin lesions on the left side of the body and progressive weakness and wasting of the same side for 5 years. The initial lesion appeared as a hyperpigmented patch on the left thigh at the age of 15 years. Five months later, this lesion began to spread proximally and distally until the entire left half of the body became involved over a period of 5 years. One year after appearance of the lesion patient noticed gradually progressive wasting of the left half of the body. He also had restriction of movements at the left shoulder, elbow and wrist as well as disfigurement due to asymmetry of the face and limbs. There were no systemic complaints, personal and family history were non contributory. On examination there was facial asymmetry due to hemiatrophy of the left side. The girths of the upper and lower limbs on the left were less than those of the right upto 4cm and 18 cm respectively. Large hyperpigmented patches were seen mostly on the left side of the body involving the scalp, face trunk and extremities. Lesions on the right were limited to the back of the trunk and thigh. A linear area of depigmentation extended from the left shoulder to the elbow. [Figure - 1]. Skin was bound down and sclerotic along the entire length of the left upper and lower limbs. Skin was also dry and ichthyotic in the affected limbs. Tongue showed left hemiatrophy. Left testis appeared smaller than the right. Muscle assessment revealed normal muscle power in all 4 limbs both in the proximal and distal groups. A diagnosis of linear morphoea with pansclerotic atrophy was made. Investigations revealed normal haemogram. LE cells were negative. VDRL was nonreactive. Rheumatoid factor was positive. Pulmonary function tests, ECG and EMG were normal. X-rays of the skull, chest and bon ,-s of the left upper and lower extremities were done. The left humerus showed a well defined osteolytic area on the greater tuberosity [Figure - 2]. Other bones were normal. Biopsy of the skin showed epidermal atrophy, diffuse dense collagenisation of the dermis with focal collections of lymphocytes and atrophic adnexal structures. Biopsy of the left testis showed seminiferous tubules with normal spermatogenesis. There was no evidence of either vasculitis or fibrosis. Biopsy from the left triceps muscle was normal.
| Comments|| |
Bone changes in morphoea have been extensively studied in the past; these being the most frequent non cutaneous findings in this condition. Congenital anomalies of the spine were noted in 12.8% of as series of 235 cases seen in the Mayo clinic over a period of 32 years . Less, frequently encountered bone anomalies in morphoea are rudimentary ribs, cervical ribs, atrophic clavicle, contracted pelvis, shortened ulna, deformity of bone of hands and feet etc. A congenital cyst at the proximal end of the femur in association with several other abnormalities like thoraco-lumbar kyphoscoliosis, spina bifida occulta and multiple congenital spider angiomas has been reported in a patient who was born with linear morphoea. Osteoporosis as the commonest secondary bone change has been reported by Jose et al in 5 out of 14 patients.
Our patient had the rare association of a bone cyst probably congenital in origin with generalised linear morphoea. In spite of the wasting of the tissues underlying the skin lesions, there was no functional or structural abnormality of the muscles as evidenced by normal muscle power, normal EMG and normal histology. The testis which was clinically small in size on the affected side was also histologically normal.
| References|| |
|1.||Rowell NR, The collagen or connective tissue disease, in: Text book of Dermatology, Fourth ed, Editors, Arthur Rook, Wilkinson DS, Ebling FJG et al: Oxford University Press, India; P 1334-1341. |
|2.||Christianson HB, Dorsey CS, and O'Leary PA et al: Localised Scleroderma, A clinical study of two hundred and thirty five cases, Arch Dermatol, 1956; 74: 629-639. |
|3.||Perez JD, Susanne MC, and Winkelmann RK: Disabling pansclerotic morphoea of children, Arch Dermatol, 1980; 116: 169-173. |
[Figure - 1], [Figure - 2]