Panniculitides of particular interest to the rheumatologist

Abstract The panniculitides remain as one of the most challenging areas for clinicians, as they comprise a heterogeneous group of inflammatory diseases involving the subcutaneous fat with potentially-shared clinical and histopathological features. Clinically, most panniculitides present as red edematous nodules or plaques. Therefore, in addition to a detailed clinical history, a large scalpel biopsy of a recent-stage lesion with adequate representation of the subcutaneous tissue is essential to specific diagnosis and appropriate clinical management. Herein we review the panniculitides of particular interest to the rheumatologist.

Bilateral Facial Lipoatrophy-As Sole Manifestation of Lupus Panniculitis.

Lupus erythematosus can have varied manifestation in skin ranging from classical lesion of DLE (Discoid Lupus Erythematosus) and photosensitivity to Necrotic lesions of vasculitis or atrophic lesion. Panniculitis as histological finding in biopsy taken from any lesion of SLE (Systemic Lupus Erythematosus) is expected as associated finding. We report one case with lipoatrophy as sole manifestation of lupus panniculitis in which patient presented to us with bilateral facial lipoatrophy. On further investigation patient was ANA (Antinuclear Antibody) positive and histopathology was also suggestive of lupus panniculitis. LE (Lupus Erythematous) presenting solely as lupus panniculitis is rare clinical manifestation and poses a considerable diagnostic challenge more so when presentation is lipoatropy.

Calcifying Lupus panniculitis in a patient without manifestations of systemic lupus Erythematosus / Paniculitis Lúpica calcificante en un paciente sin manifestaciones de lupus eritematoso sistémico

Lupus panniculitis or lupus profundus is a variant of lupus Erythematosus cutaneous that primarily affects subcutaneous tissue. Clinically, it is characterized by one or several firm subcutaneous nodules and/or plaques with or without overlying epidermal changes. It is reported to occur with a frequency of 2-3% in patients with Systemic Lupus Erythematosus (SLE). Between 10 and 50 percent of patients with lupus panniculitis will have or eventually develop Systemic Lupus Erythematosus. In nearly all cases there are deep, erythematosus plaques and nodules, and some of them ulcers, which usually involve the proximal extremities, trunk, breasts, buttocks, and face. These lesions may be tender and painful and frequently heal with atrophy and scaring, turning as a chronic condition and subsequently heal with disfigurement. We describe a patient who suffers from lupus panniculitis with no association to SLE symptoms and complicated by several progressive and disabling cutaneous lesions.

La paniculitis lúpica o también llamada lupus profundus es una variante del lupus eritematoso cutáneo que afecta el tejido celular subcutáneo. Se caracteriza clínicamente por uno o varios nódulos subcutáneos que son firmes y/o placa con o sin cambios epidérmicos. Se ha informado su frecuencia en 2% a 3% de casos de Lupus eritematoso sistémico. Entre el 10 al 50% de los casos de paniculitis lúpica va a desarrollar lupus eritematoso sistémico. En casi todos los casos hay placas eritematosas y/o nódulos que en algunos casos se ulceran y que usualmente están localizados en las áreas proximales de las extremidades, tronco, mamas, nalgas y la cara. Estas lesiones pueden ser clínicamente dolorosas y sensibles a la presión y frecuentemente cicatrizan con desfiguración del área circundante. Describimos un paciente que padece de paniculitis lúpica sin asociación de lupus eritematoso sistémico y que se complicó con varias lesiones cutáneas progresivas y discapacitantes.

A Case of Lupus Panniculitis-Misdiagnosed as Alopecia Areata

We present a 24-year-old woman with systemic lupus erythematosus presented with multiple patches of non-scarring alopecia closely resembling alopecia areata. Scalp biopsy showed a predominantly subcutaneous and dermal lymphocytic infiltrate that surrounded the deep follicular segments and hair bulb. This pattern is capable of producing a temporary hair loss, clinically misdiagnosed as alopecia areata. The clinical history, presence of erythema on bald patches and scalp tenderness as well as the biopsy findings were important clues in diagnosis of lupus erythematosus. We report here a case of lupus panniculitis that presented as multiple bald patches misdiagnosed as alopecia areata.

Paniculitis lúpica (Lupus profundo) / Lupus panniculitis (Lupus profundus)

Se comunica un caso de lupus profundo en una mujer de 45 años, localizado en el tronco que inicialmente fue diagnosticado ecográfica y clínicamente como un lipoma. Efectuamos una actualización de esta infrecuente paniculitis.

A case of lupus profundus located in the trunk of a 40 years old woman, which was initially clinically and by ultrasound diagnosed as lipoma is reported. A clinical and histopathological review of this infrequent panniculitis is made.

A Case of Linear Lupus Panniculitis in Child / 대한피부과학회지

Lupus panniculitis is an uncommon clinical variant of cutaneous lupus erythematosus. The cutaneous lesions of lupus panniculitis are characterized by subcutaneous nodules or plaques. The overlying skin can often appear unchanged, but may also be associated with discoid lupus erythematosus and appear erythematous, atrophic, telangiectatic or poikilodermatous. The mean age of incidence is between 30~40 years, and children are rarely affected. Furthermore, a linear distributions of the lesions has rarely been reported. We herein describe a case of 8-year-old girl with linear lupus panniculitis on the face, and propose that the possibility of this disease be considered in cases where linear subcutaneous nodular lesions occur in children or young adults.

Lupus Panniculitis: Clinico-histopathological Analysis of a Case Series / 대한류마티스학회지

OBJECTIVE: Lupus erythematosus panniculitis (LEP) is a rare subtype of chronic cutaneous LE. Until now, a few studies about the clinico-histopathological data of LEP have been conducted in Korea. The aim of this study was to investigate clinical, serological, and histopathologic features of LEP. METHODS: The clinical survey was retrospectively performed through the available medical records in 15 cases with LEP. The histopathologic, serologic and clinical findings were included in this analysis. RESULTS: LEP occurred primarily in females (F:M=4:1). The mean age at diagnosis was 37.5 years. 20% (3/15) of the patients was associated with SLE. DLE was accompanied with LEP in 26.6% (4/15). The predilectional sites were as follows in order: upper limbs (33.3%), lower limbs (20%), trunk (26.6%), buttock (13.3%) and face (6.6%). The fluorescent antinuclear antibody (FANA) was positive in 46% (7/15) of all cases. The main histopathologic characteristics of LEP were lymphocytic lobular panniculitis (100%), hyaline degeneration (86.6%), lymphoid aggregates (60%), mucin deposition (60%), lymphocytic vasculitis (40%). The treatment included anti-malarial agents, systemic corticosteroid, dapsone, intralesional triamcinolone injection. Although significant remission was proven in most cases (86.6%) of LEP, they represented a chronic relapsing course (83.3%). CONCLUSION: LEP is a chronic inflammatory disease of subcutaneous adipose tissue that can develop during the course of SLE. Evaluation of patients suspected of having LE panniculitis should include a complete history taking and physical examination as well as serologic studies, determination of peripheral blood counts, and tests of renal function.

A Case of Lupus Panniculitis Involving the Breast / 대한피부과학회지

Lupus panniculitis is a chronic inflammatory disease of subcutaneous fat that occurs in 2~3% of patients with systemic lupus erythematosus. Lupus panniculitis typically presents with firm, indurated subcutaneous nodules or plaques commonly developed in proximal extremities, trunk, head and neck. Breast involvement with lupus panniculitis (lupus mastitis) has been infrequently reported. We experienced a case of lupus mastitis developed in a 44-year-old female confirmed by histologic features and laboratory findings. Antimalarial therapy resulted in the complete resolution of the lesions.