Acquired reactive perforating collagenosis with the histological features of IgG4‐related sclerosing disease in a patient with Mikulicz's disease

Abstract

Acquired reactive perforating collagenosis has been reported in association with various conditions. To the authors' knowledge there has been no report of a patient with Mikulicz's disease showing acquired reactive perforating collagenosis. A 61-year-old Japanese man presented with non-pruritic multiple umbilicated papules with central keratotic and crusted plagues on the trunk, neck, and scalp. He had been diagnosed with Mikulicz's disease. Histologically, cutaneous biopsy showed a shallow cup-shaped lesion with a central crusted ulceration containing degenerated collagen in vertical strands, parakeratotic horny material, neutrophils, basophilic granular debris and elimination of collagen bundles from the dermis through to the epidermis. These clinical and histological findings suggested reactive perforating collagenosis. In addition, lymphocytes and plasma cells had infiltrated the dermis, with a tendency to be distributed around the sweat glands, accompanied by sclerotic fibrosis. On immunohistochemistry most plasma cells were positive for IgG and IgG4 (IgG4+/IgG+ plasma cells >50%). These histological findings of the skin were similar to findings previously reported for IgG4-related sclerosing diseases in other organs. Herein is described the first case of a patient with Mikulicz's disease showing acquired reactive perforating collagenosis accompanied by the histological features of IgG4-related sclerosing disease.

Colagenosis perforante reactiva adquirida: Caso clínico

“…En un estudio inglés hecho en una población de insuficientes renales crónicos en hemodiálisis, se demostró que 11% presentaba colagenosis perforante reactiva adquirida 15 . Además se ha descrito la asociación con otras enfermedades como hipotiroidismo, linfomas, hiperparatiroidismo, neurodermatitis, SIDA, fibrosis pulmonar, enfermedades hepáticas, dermatitis atópica, infección por citomegalovirus, mucormicosis cutánea, síndrome de Treacher Collins, púrpura de Schönlein-Henoch, enfermedad de Mikulicz, reacción adversa a medicamentos, escabiosis y herpes zoster 5,16 . Sin embargo, muchas de estas enfermedades se presentaron asociadas además a diabetes o a falla renal.…”

A new category for chronic sclerosing sialadenitis as an IgG4 related syndrome

“…In addition, a malignant disease cannot be ruled out because the mass is unilateral, often leaving no choice other than excision. IgG4 related sclerosing disease occurs relatively often among cases of submandibular gland excision and suspected cases of SS (wherein the diagnostic criteria are not satisfied) 18 – 20…”

“…This disease, complicated with chronic sclerosing sialadenitis,2 , 3 autoimmune pancreatitis (AIP),4 – 9 SC,10 – 12 abdominal aortic aneurysm,13 , 14 OPT,15 interstitial lung disease,16 IRF and exhibiting high serum IgG4 values has recently attracted attention, but the findings have been insufficient 17. This report presents three cases of immunoglobulin (Ig) G4 related sclerosing disease complicated with chronic sclerosing sialadenitis.…”

IgG4-Related Skin Disease, a Mimic of Angiolymphoid Hyperplasia with Eosinophilia

“…Shiomi et al [32 ]reported the case of a patient with Mikulicz’s disease who had an acquired reactive perforating collagenosis with histological findings of IgG4-related disease. Another report described 3 cases of cutaneous plasmacytosis that showed a high ratio of IgG4-expressing plasma cell infiltration in histological sections.…”