Papulonodular Mucinosis Associated With Subacute Lupus.

Papulonodular mucinosis is a rare but well-documented finding associated with systemic and cutaneous forms of lupus erythematosus (LE). It occurs exceptionally in association with subacute cutaneous lupus erythematosus (SCLE). Its etiology and pathogenesis remain to be elucidated. Herein, we report a case of papulonodular mucinosis associated with SCLE in a middle-aged woman. On physical examination, she presented with multiple flesh-coloured asymptomatic papules and nodules on the trunk and upper extremities. A biopsy specimen taken from a nodule showed mucin within the dermis with hypodermis and perivascular lymphocytic inflammation. Considering that the proportion of patients with cutaneous lupus mucinosis who progress to systemic lupus is uncertain, we suggest following these patients closely for evidence of multisystem disease.

Papulonodular mucinosis in the primary onset of juvenile systemic lupus erythematosus: An unusual association / Mucinosis papulonodular en el inicio de lupus eritematoso sistêmico juvenil: asociación inusual

ABSTRACT Cutaneous mucinosis is a group of conditions characterized by the abnormal deposition of mucin in the skin. They can be primary, which in turn can be inflammatory-degenerative, and hamartomatous-neoplastic; or secondary. Papulonodular mucinosis is part of the group of dermal inflammatory-degenerative primary mucinosis. Its association with autoimmune connective tissue diseases has been described, especially with systemic lupus erythematosus, but it is considered an unusual manifestation of this disease. The clinical case is presented of an 11 year-old girl who, at the onset of systemic lupus erythematosus, presented with skin lesions for which the histopathological diagnosis corresponded to mucinosis.

RESUMEN Las mucinosis cutáneas son un grupo de condiciones caracterizadas por el depósito anormal de mucina en la piel. Pueden ser primarias, que a su vez pueden ser inflamatorias-degenerativas (dérmicas o foliculares) y hamartomatosas-neoplásicas; o secundarias. La mucinosis papulonodular forma parte de las mucinosis primarias inflamatorias-degenerativas dérmicas. Se ha descrito su asociación con enfermedades autoinmunes del tejido conectivo, especialmente con el lupus eritematoso sistémico, pero se considera una manifestación inusual de esta enfermedad. Se presenta el caso clínico de una niña de 11 años, quien al inicio del lupus eritematoso sistémico presentaba lesiones en la piel cuyo diagnóstico histopatológico correspondió a mucinosis.

Papulonodular mucinosis, Guillain-Barré syndrome and nephrotic syndrome in a patient with systemic lupus erythematosus: a case report.

BACKGROUND: Awareness of the spectrum of clinical manifestations of systemic lupus erythematosus (SLE), especially uncommon changes, is essential for diagnosis and effective management of patients. CASE PRESENTATION: A 26-year-old Chinese man with SLE initially manifested cutaneous papulonodular mucinosis and developed acute Guillain-Barré syndrome and class V lupus nephritis 2 years later. His cutaneous nodules had not been idententified for 2 years and were resected by surgical procedures twice until SLE was diagnosed. The kidney biopsy revealed class V lupus nephritis. The patient responded well to a short course of intravenous immunoglobulins and his muscle strength almost completely recovered. So far, he has undergone five cycles of cyclophosphamide combined with hydroxychloroquine and tapering prednisone, resulting in partial remission of lupus nephritis and disappearance of hypocomplementemia. CONCLUSION: We reported a rare case of male patient with SLE with manifestation of class V lupus nephritis, Guillain-Barré syndrome and papulonodular mucinosis.

Papular Mucinosis Associated with Systemic Lupus Erythematosus.

Papulonodular mucinosis (PNM) is a rare variant of lupus erythematosus (LE) eruptions, and PNM is characterized histologically by diffuse dermal mucin without any typical epidermal inflammatory changes. We herein describe a case of papular mucinosis that was characterized by several erythematous papules on the lower back of a 32-year-old man with systemic LE. It is interesting that he didn't display any other skin manifestations of LE such as malar rash, discoid rash and photosensitivity during the previous 2 years. He achieved remission of his PNM without recurrence after 5 months treatment with topical steroids, in addition to receiving systemic antimalarials and steroids.

Papular Mucinosis Associated with Systemic Lupus Erythematosus

Papulonodular mucinosis (PNM) is a rare variant of lupus erythematosus (LE) eruptions, and PNM is characterized histologically by diffuse dermal mucin without any typical epidermal inflammatory changes. We herein describe a case of papular mucinosis that was characterized by several erythematous papules on the lower back of a 32-year-old man with systemic LE. It is interesting that he didn't display any other skin manifestations of LE such as malar rash, discoid rash and photosensitivity during the previous 2 years. He achieved remission of his PNM without recurrence after 5 months treatment with topical steroids, in addition to receiving systemic antimalarials and steroids.