Clinicopathological analysis of six cases of disabling pansclerotic morphea / 中华皮肤科杂志

Objective To investigate clinical and pathological features of 6 cases of disabling pansclerotic morphea (DPM).Methods Clinical and pathological manifestations of and follow-up results in 6 patients,who were clinically and histopathologically diagnosed with DPM in the Department of Pathology,Hospital for Skin Diseases,Chinese Academy of Medical Sciences and Peking Union Medical College from 2007 to 2017,were retrospectively analyzed.Results Among the 6 patients,4 were male and 2 were female.The age of onset ranged from 3 to 10 years,with an average age of 6.5 years.The average duration from the occurrence to the confirmation of the diagnosis was 6.2 years (range,2-10 years).At all the lesional sites,skin atrophy,thining and tightness occurred,and the limbs became thin.Additionally,there were muscular atrophy and visible deep thick veins on the surface of the limbs.The contracture,deformity and dysfunction of the adjacent joints occurred in 4 cases,and the lower limbs were obviously shortened in 2 cases.Peripheral blood examination showed no increase of eosinophils or hypergammaglobulinemia.Imaging examination revealed smooth cortical bone and clear trabecular bone,and no osseous abnormality was observed.Histopathological examination of contracted skin lesions of the lower limbs revealed atrophic and thinned epidermis,hyperpigmentation in the basal layer,hyperplastic,thickened,hardened and partly homogenized collagen fibers in the middle to deep dermis,subcutaneous adipose tissue region and deep tissue of the skin.Conclusions DPM usually does not affect viscera,but often involves deep tissue of the limbs.Histopathologically,DPM is mainly characterized by obviously hyperplastic and hardened collagen fibers in the dermis and subcutaneous tissue.

Squamous Cell Carcinoma Arising from Chronic Ulcerative Lesion in a Patient with Disabling Pansclerotic Morphea

Authors report herein a case of a 27-year-old male patient who had been suffering from chronic ulcerative dermatitis with scar-like changes and successive involvement of the ankles, limb folds, nape and abdomen, and no tendency to heal from age 4. At the age 14, an immunologic study showed a selective IgA deficiency with partial T-lymphocyte inactivation. Ten years later, at age 24, he showed a severe form of morphea over a generalized area and disabling joint contractures, and was diagnosed as disabling pansclerotic morphea with an IgA value that returned to a near normal level. At the age 27, an adult-fist, 7×6×4cm sized, squamous cell carcinoma with an easy bleeding tendency like an overgrowing granulation tissue vascular tumor had developed on the chronic ulcerative lesion on the posterior aspect of the right ankle for 2 months. Aggressive metastatic lesion occurred on the right popliteal area 3 months later. He died 1 month thereafter.

Disabling Pansclerotic Morphea / 대한피부과학회지

Disibling pansclerotic morphea, a severe form of generalized morphea, has a relentless clisabling course, producing marked contract,ion and joint deformity due to sclerosis of the skin and the deeper structures. We report herein an unusual case of morphea in a 25 year old male who had suffered from chronic recurrent ulceration with scar like sclerotic changes overlying limb folds, ankles, nape and abdomen since 4 years of age. The indurated lesions on the chest and abdomen were ivory colored, sclerotic guttae and plaques having a, violac ous border. At l4 years of age, the skin lesions were diagnostically ambiguous, wherea.s the immunologic results revealed a selective IgA deficiency with partial T lymphacyte inactivation. About 10 years later, the follow up visit showed this severe form of morphea over generalized area and disabling contractures of knees, elbows and alkles, but IgA value returned nearly to the nomal level.