NHDP CPC 2 Case 2 Jan 24 2005 Dr. Richard Wing Communicable Disease Director, DSHS, Region 11 Harlingen, Texas

9 ½ year old boy born in Monterrey, Mexico Lived with grandparents in China, Mexico until age 2 Living in Mission, Texas for the last 7 ½ years Mission • •McAllen China • • Monterrey

Grandfather with leg lesions for over 10 years Mother with leg lesions 5 years ago (resolved) Patient with leg lesions 3 years ago (resolved) May 2005 nodular lesions to face progressing to target lesions over entire body June 2005 biopsy done suggesting leprosy (tuberculoid)

Multiple plaques with heavy scale; slight loss of sensation within some of the lesions Nasal septum with edema & mucosal erosions Normal motor strength Slit skin smears: knees 4+/globi; back 3+; elbows 3+; ears NF

Enlarged right radial cutaneous & ulnar nerves Large plaque to palm of right hand Fissures at the base of the fingers

4th right finger swollen at proximal interphlangeal joint Painful with movement & tender to touch

X-ray: bony erosion to distal end of 1st phalanx of the right 4th finger WHO classification: multibacillary; Ridley-Jopling classification: BT/BB

Treatment: Dapsone 50 mg daily (1-2 mg/kg); Rifampin 300 mg daily (10 mg/kg) No Clofazamine given: none available to MD in south Texas & not FDA approved for use in children ( as per Dr. Pat Joyce ) Paradoxical reaction: golf ball size lymph node swelling in right axilla 2 weeks after starting therapy

After 3 weeks of therapy: development of bilateral hand edema, increased tenderness/induration of all lesions, & extensive desquamation of all lesions No new nerve findings

Diagnosis: reversal reaction Treatment: Prednisone 20 mg daily; Rifampin 300 mg changed to once monthly dosing ( DOT); Dapsone remained at 50 mg daily

Significant clinical improvement after 3 weeks of steroid therapy: flattening of lesions with fading erythema

Same day: facial lesions show resolution of the desquamation Prednisone dose is decreased to 15 mg daily

Prednisone dose subsequently decreased to 10 mg daily Within 2 weeks of decreasing Prednisone the patient experiences a flare-up of all lesions

Prednisone dose now increased to 30 mg daily with a plan for a much slower taper than before

Despite the breakthrough of the skin lesions on the lower dose of Prednisone, by this time in the therapy the swelling & pain to the right 4th finger had completely resolved

A little more than a month later the patient’s skin lesions have again faded He is tolerating the DDS/Rifampin & Prednisone without any difficulty

No breakthrough reactions to this point with the slower steroid taper The patient comes to the clinic much happier than before because his skin lesions are no longer a topic of conversation among his classmates

The grandfather comes from Mexico for an evaluation The grandfather comes from Mexico for an evaluation. History of ulcerations to hands/feet for more than 10 years, as well as nasal stuffiness for at least 4 years. Long history of shooting & skinning armadillos.

Complete loss of sensation to upper & lower extremities in a stocking/glove distribution; note the amputation of the left 3rd toe due to previous gangrene Slit skin smears: knees/elbows 1+; ears NF Receiving treatment in Mexico for “poor circulation”

In a rather strange twist to the story: the patient is well-known to leprosy control in Mexico and was supposedly receiving standard multidrug therapy However, there is another man in his town with the same name and apparently the other man was receiving this gentleman’s leprosy medications