Unilateral linear pansclerotic morphea affecting face and limbs.

Disabling pansclerotic morphea is a rare atrophying and sclerosing disorder of the subcutaneous tissue, muscle and bone. It is characterized by atrophy of the skin, subcutaneous fat, muscle and bone involving half of the face. In some patients the atrophic lesions extend to involve the ipsilateral or contralateral upper and lower limbs with radiological evidence of hemiatrophy. The patients may present with arthralgia, convulsions or cramps. We report a case of a woman with deformity of face, and left upper and lower limbs that had started as an indurated plaque on the left half of forehead at the age of 5 years and had gradually enlarged, followed by the development of atrophic changes in left eye. The case is being reported in view of its rare occurrence.

Manifestations articulaires, musculaires et cutanees des etats reactionnels au niveau de la main du lepreux

The reactional states seen in lepromatous form (Erythema nodosum, exacerbation) and in interpolar forms (tuberculoid exacerbation and tuberculoid reversal reaction) result in cutaneous, nervous, lymphatic, visceral and polvarhritic acute manifestations. The successif relapses result in articular, cutaneous and muscular, sequelae of the hand but a paralysis of medial and ulnar nerves, often associated with leprosy, make them different from rheumatoid arthritis. They can be summarized thus: 1. Fexed claws hand associated with stiffness, erosions and sometimes subluxations of interphalangeal joints, mainly proximal and metacarpophalangeal joints. 2. Thickening and retractions of the skin of the dorsal face of the hand resulting in a trouble of the flexion of the fingers. 3. Contracture of the intrinsic muscles of the fingers and swan neck deformity. The treatment of the articular acute manifestations prevents the formation of sequelae. The authors study also the possibility of a curative treatment of fixed claws hand, skin retractions and swan neck deformities of the fingers, in hansenian patients