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4.
Am J Dermatopathol ; 40(5): 337-341, 2018 May.
Article in English | MEDLINE | ID: mdl-28953013

ABSTRACT

Light chain deposition disease (LCDD) is a rare systemic disorder with deposition of mostly monoclonal amorphous nonamyloid light chains in multiple organs. Renal involvement with rapidly progressing renal failure presents the dominant manifestation of LCDD. Approximately 20%-30% of patients show symptomatic cardiac or liver involvement. Cutaneous manifestations are extremely rare with only a few published cases. We report 2 additional cases of cutaneous LCDD without detectable systemic disease.


Subject(s)
Immunoglobulin kappa-Chains , Paraproteinemias/pathology , Adult , Female , Humans , Male
5.
J Cutan Pathol ; 37(9): 1002-9, 2010 Sep.
Article in English | MEDLINE | ID: mdl-20175822

ABSTRACT

Microcystic adnexal carcinoma (MAC) is a rare, usually solitary, slowly growing, yet aggressive neoplasm with a tendency for local recurrences. Herein, we present two patients who had been histopathologically diagnosed as suffering from MAC on both cheeks since childhood, an unlikely scenario. Both from a clinical and from a histopathological point of view, our two cases showed some similarities with those previously described in patients with Nicolau-Balus syndrome, Rombo syndrome, and so-called eccrine-pilar hamartoma. Common to all these latter disorders are the round aggregations of elastic tissue in the papillary dermis, a histopathological feature which was also found in our patients. However, to our knowledge, the presence of a MAC-like ductal proliferation embedded in sclerotic stroma and extending to the deep dermis has not been previously described. Dermatologists and dermatopathologists should be aware of this disorder to avoid overdiagnosis of and inappropriate treatment for MAC.


Subject(s)
Adenoma/diagnosis , Facial Dermatoses/pathology , Hair Follicle/pathology , Neoplasms, Adnexal and Skin Appendage/diagnosis , Skin/pathology , Sweat Glands/pathology , Adult , Atrophy/pathology , Biopsy , Cell Proliferation , Diagnosis, Differential , Elastic Tissue/pathology , Female , Hamartoma/diagnosis , Humans , Male , Neoplasms, Multiple Primary/pathology , Sweat Gland Neoplasms/diagnosis
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