Multiple cutaneous leiomyomas of pilar origin have long been recognized to have an autosomal dominant inheritance. While the skin tumors are relatively uncommon and benign, women of affected families often develop uterine fibroids with associated infertility, pain and bleeding. In addition, a subset of these families harbors a predisposition to papillary renal cell carcinoma. Germline mutations in a recently identified classical tumor suppressor gene encoding fumarate hydratase are observed in these individuals. Appropriate screening measures for associated disorders are mandatory.

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Nephrogenic fibrosing dermopathy is a recently recognized skin disorder similar in appearance to scleromyxedema but without the systemic involvement. We describe a 14-year-old girl with new-onset systemic lupus erythematosus and acute lupus nephritis who developed on the lower extremities confluent hyperpigmented, woody, indurated plaques that contained groups of coalescing erythematous papules. Nephrogenic fibrosing dermopathy was diagnosed histologically. Possible etiologies are discussed.

A 60‐year‐old male with a history of pulmonary fibrosis and lung transplant presented to clinic with a three week history of cutaneous eruption on the extremities associated with nausea and fatigue. His medications included prednisone and azathioprine. Exam revealed the patient was afebrile with multiple perforating nodules and subcutaneous nodules in a sporotrichoid pattern over the extremities. Tissue was sent for histopathological evaluation, and culture for bacteria, acid‐fast bacteria, and deep fungal organisms was performed. Histopathology revealed necrotizing panniculitis, but no organisms were found, and tissue cultures were negative. Due to choreoathetoid movements of the right extremities and persistence of cutaneous nodules, the patient was hospitalized and an extensive search for infectious etiology of his condition undertaken. Empiric antibacterial and antifungal therapy was given, but he progressed to multiorgan failure and expired. Autopsy revealed rare amoebic trophozoite forms in the skin in addition to cysts in the lung and brain. Cause of death was determined to be disseminated Acanthamoebiasis. Review of initial skin biopsy showed rare trophozoites. Differing from Entamoeba, Acanthamoeba is a rare but important emerging pathogen. Histopathological diagnosis of Acanthamoeba rests on high index of suspicion in cases of suspected infectious etiology.