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Background: There is a growing literature regarding sclerotic and panniculitic cutaneous conditions seen in patients with end-stage renal disease (eg, calciphylaxis and soft tissue calcification). Nephrogenic fibrosing dermopathy (NFD) is a recent designation to describe cutaneous findings in patients with end-stage renal disease who developed sclerotic plaques with scleromyxedema-like histologic features. Soft tissue calcification is rare in patients with NFD and systemic involvement has not been reported., Observations: We describe a patient with end-stage renal disease who developed diffuse indurated woody plaques consistent with NFD in association with soft tissue calcification with catastrophic sequelae. A deep excisional biopsy specimen from the patient revealed thickened collagen bundles in the reticular dermis, plump bipolar spindle cells, and increased mucin. Focally, there were zones of calcium deposition in dermal collagen without vessel calcification. Autopsy of the patient revealed extensive fibrosis and calcification of the diaphragm, psoas muscle, renal tubules, and rete testes. The patient died 11 months after developing NFD., Conclusion: A subset of patients with NFD may have significant systemic involvement.

Background and Objective: Atrophoderma of Pasini and Pierini (APP) is an uncommon cutaneous disorder, with no known effective treatment, manifested by hyperpigmented patches that appear to be depressed compared with surrounding skin. This study investigated the effectiveness of the Q-switched alexandrite laser on a patient with extensive APP, and evaluated histopathologic and ultrastructural changes., Study Design/materials and Methods: A man with stable APP underwent Q-switched alexandrite laser treatment to a patch on the trunk. Biopsies were obtained from treated and untreated sites of involvement. Light and transmission electron microscopic evaluation was performed to investigate melanosome number, size, and volume, as well as melanin granule number and size., Results: After three treatment sessions, the treated area showed marked clinical improvement. Electron microscopy showed a 19% reduction in melanin granule number and size and a 65% reduction in melanosome number, size, and volume in larger melanosomes in treated compared with untreated sites., Conclusion: Treatment of APP with the Q-switched alexandrite laser results in clinical improvement. Electron microscopic evaluation suggests that the mechanism may be a reduction in the number, size, and volume of larger melanosomes as well as a decrement in melanin granule number and size., (Copyright 2000 Wiley-Liss, Inc.)

A 77-year-old man with chronic conjunctivitis, acanthosis nigricans with pachydermatoglyphy, and pemphigus-like mucocutaneous lesions was found to have a well-differentiated bronchogenic squamous cell carcinoma. Histopathologic and immunofluorescence studies confirmed the diagnosis of paraneoplastic pemphigus. Skin lesions resolved with oral prednisone and azathioprine therapy, but the conjunctivitis and mucous membrane erosions persisted. The conjunctiva later became scarred with foreshortening of the fornices and development of symblepharon. External beam irradiation arrested the growth of the tumor but did not have any effect on the diseased conjunctiva and other mucous membranes. This case demonstrates that cicatrizing conjunctivitis with bullous mucocutaneous lesions may be a clinical sign associated with an occult neoplasm.

Two unrelated young women with papular elastorrhexis have recently been seen at the University of Iowa Hospitals and Clinics. This entity, recently described in a single patient, is a distinct variant of a connective tissue nevus. Although the histologic features of papular elastorrhexis may mimic those of other connective tissue nevi, clinical characteristics allow differentiation. The clinical and histologic characteristics of papular elastorrhexis, as well as its differential diagnosis, are discussed.

The acute clinical syndrome of toxic epidermal necrolysis (TEN) is currently thought to be a distinct clinical – pathological entity typically resulting from drug hypersensitivity. We describe an adult woman who experienced a fulminate pattern of apoptotic epidermal cell injury following tanning bed exposure while taking naproxen that resulted in a clinical presentation having combined features of drug-induced TEN and an infrequently recognized form of bullous cutaneous lupus erythematosus (LE). This case calls attention to the fact that TEN-like injury can occasionally be seen in settings other than drug hypersensitivity (e.g., LE, acute graft versus host disease) and illustrates the need for a unifying concept in this area. We therefore propose the term 'Acute Syndrome of Apoptotic Pan-Epidermolysis (ASAP)' to designate a clinical syndrome that is characterized by life-threatening acute and massive cleavage of the epidermis resulting from hyperacute apoptotic injury of the epidermis. We also review vesiculobullous skin disorders that can be encountered in LE patients and suggest a new classification scheme for such lesions. [ABSTRACT FROM AUTHOR]