Parakeratotic horns in a patient with myeloma

Abstract

We describe a patient who in association with myeloma developed generalized keratotic horns. Light microscopy revealed parakeratotic plugs resembling those seen in Kyrle's disease. Electron microscopy showed distortion of keratinosomes and laminated bodies indicating a profound disturbance in keratinization. A strong C3 and weaker IgG and IgA immunofluorescence were seen in the areas of epidermal plugs and horns but no circulating epidermal antibodies were detected. All symptoms disappeared when therapy for myeloma was started favoring that they were of paraneoplastic origin.

Rare presentation of Kyrle′s disease in siblings

“…Primary perforating disorders include Kyrle's disease (hyperkeratosis follicularis et parafollicularis in cutem penetrans), elastosis perforans serpiginosa, perforating folliculitis and reactive perforating collagenosis 13. They need to be distinguished from the secondary perforating dermatosis due to metabolic conditions, such as uraemia, especially in patients on dialysis, diabetes, hepatic failure or as a paraneoplastic syndrome in multiple myeloma 4–9. An infective aetiology10 and exposure to chemical agents11 have also been reported to be causative factors for Kyrle's disease.…”

Eruptive ulcerative follicular spicules heralding progression of smoldering multiple myeloma

“…To the best of our knowledge, a total of 23 patients—including ours—(18 males, 5 females) have been described to date (Table ) . The eruption preceded the diagnosis of MM in 12/23 patients, while in seven patients—including ours—it heralded the progression or relapse of the plasma cell disorder . The temporal relationship between the dermatosis and the hematological disorder was not specified in four reports .…”

“…Clinically, it presents with a myriad of white‐yellowish, asymptomatic, spicules of horny appearance, mostly on the nose, ears, scalp and neck, occasionally on the trunk and extremities. Horny‐like and/or purpuric palmoplantar lesions and nail changes have also been previously described but were not studied histopathologically.…”

Multiple minute digitate hyperkeratoses associated with paraproteinaemia

“…Of those associated with multiple myeloma (the third category), the spicules can be histologically separated into two subtypes: one with follicular Ig 3–6 deposition and the second with hyperkeratosis alone 7–9 (with or without follicular association).…”

“…The second subtype is characterized by hyperkeratosis and no Ig deposition, in association with multiple myeloma. Response to systemic chemotherapy has been reported to clear these keratotic spicules 7 . The histology consists both of columns of parakeratosis and orthokeratosis with either no association with hair follicles 8 .…”