Hemicorporeal morphea

Study of seven cases

Abstract

Introduction: Hemycorporal morphea is an extremely infrequent disor-der. Its limits and relationship with facial hemiatrophy and lineal mor-phea remains not truly understood. Objective: To define its characteristics to let early acknowledge of this disorder trying to avoid the severe course of the disease.Material and method: Seven cases of hemychorporal morphea (six women and one man) six years to thirty five years-old studied between 2000-2009 in our Department of Dermatology were analized. The pres-ence of sclerosis or atrophy, the age of consultation and onset of dis-ease, site of initiation, ipsylateral or contralateral involvement, presence of contractures, dystonia or myoclonus, loss or diminished vissual func-tion, dry eye, and laboratory findings, neurological examination, MR and CRL findings were reported here.Results: Five cases started on the lower or upper limb, three out of them were sinchronic with other body area. Three cases started on the face or trunk, all of them simultaneously with a limb involvement. Rapid spreading (days or weeks) was characteristic of this disorder. It followed a course spreading up or down. Sclerosis seemed to be prevalent in most, but one case only exhibited atrophy. Contralateral lesions were seen and one case was a systemic sclerosis. Ulcer arose on the affected lower limb (in one case with moderate levels of antiphospholipids an-tibodies in one out of three determinations). Dystonia contractures and myoclonus were reported by all patients. Small areas of demyelinization in MR, and olygoclonal bands in CRL were present in one patient; this case exhibited only atrophy and a doubtful frontal sclerosis. Two cases showed slower esophagic transit. Dry eye was associated in two cases also. In the laboratory investigations hyperglobulinemia and positivity for RF, ACLA, FAN were found in the seven cases, diminished.fractions of complement in two. Only one case tested positive for Scl70.Conclusions: This particular form of morphea follows a singular course and behaviour: it usually starts as a lineal morphea with a limb involve-ment, shows rapid spreading, often includes neurological involvement or others, and exhibits laboratory unspecific findings. Cutaneous atro-phy may be the only manifestation, and early treatment may arrest tem-porarely the progression of cutaneous involvement and /or neurologic symptoms in the early follow-up.