Superficial disseminate porokeratosis in a patient with myelodysplastic syndrome

Abstract

NJ 08053[TAB]A 70‐year‐old man was admitted with a diagnosis of myelodysplastic syndrome (MDS) and fever of unknown origin. He had been diagnosed with MDS 2 months prior to admission, and was treated initially with the anabolic androgen, Danazol. He had also been on prednisone, 60 mg a day for a few months for the treatment of hemolytic anemia. Two weeks later, he noted the development of a mildly pruritic rash over his trunk and extremities. He denied any previous history of a similar rash as well as any family history of similar lesions. It was thought that he was having an allergic reaction to the Danazol and, therefore, Danazol was discontinued. The rash seemed to improve but flared again a few days prior to admission. Upon admission, his laboratory data revealed hemoglobin 9.3 gm/DL, hematocrit 27.3%, white blood cell count 3300/mm3 and platelet count 41,000/mm3. A bone marrow biopsy showed a hypercellular marrow. Chromosome analysis of the bone marrow revealed a complex karyotype including the loss of chromosomes 5 and 7, the presence of an isochromosome for the entire long arm of chromosome 17, and the presence of an additional chromosome 21; these are characteristic chromosome aberrations in myelodysplastic syndromes. Physical examination demonstrated discrete, small, thread‐like, annular papules over the trunk and extremities ( Fig. 1). A skin biopsy was performed and was compatible with porokeratosis showing a typical cornoid lamella ( Fig. 2). He was not given any treatment for his skin lesions and treatment for his MDS consisted solely of red cell concentrates. 1 Multiple annular papules with a thread‐like border as seen in porokeratosis 2 A typical cornoid lamella characteristic of porokeratosis

Atypical Porokeratosis Developing Following Bone Marrow Transplantation in a Patient with Myelodysplastic Syndrome

“…The latency period between organ transplantation and the appearance of porokeratosis ranges from 4 months to 14 years 2 . Clinically, immunosuppression-associated porokeratosis is more often characterized by multiple rather than single lesions 3 .…”

“…Although the exact type of porokeratosis has not always been specified, 50% of reported cases have been identified as DSP 3 .…”

Isolated primary cardiac amyloidosis associated with porokeratosis of Mibelli

“…However, accumulating data strongly supports the concept of an underlying severe immunoregulatory disorder [14,15], or even immunosuppression with or without myelodysplasia [16,17], almost for all types of porokeratosis.…”

Eruptive Disseminated Porokeratosis in a Patient With Type 2 Diabetes Mellitus

“…In the case of immunosuppression, expression of a mutant clone of epidermal cells, which may induce porokeratosis, may increase because immune system cannot control or delete that clone. 7,10 In addition to immunosuppressive effect, DM may directly affect keratinocytes as well. It is known that excessive glycation and/or glycoxidation of proteins in keratinocytes in DM may lead to an abnormal proliferation and differentiation of keratinocytes like in patients with DM-induced ichthyosis.…”