Introduction: Lupus panniculitis (LP) is an unusual type of cutaneous lupus erythematosus (CLE) wherein the cutaneous inflam- matory reaction involves primarily the deeper dermis and subcutaneous fat. It is characterized by the appearance of recurrent, mostly asymptomatic, firm, nodules or plaques, involving the face, upper limbs, and buttocks. Case report: In our case, a 30-year-old female presented with a non-tender, non-movable nodule on the left breast, 6 weeks prior to her admission. She had fever, chills, and joint pains. The patient later developed hyperpigmented plaques on the infra- clavicular area, and left flank extending to the abdomen. Urinalysis showed proteinuria, and RBC cast. She also had leukopenia, and anemia on CBC. Chest computerized tomography (CT) scan revealed a heterogeneously enhancing soft tissue mass in the base of the neck at the right infraclavicular region with malignant features. ANA titer was normal, while skin biopsy on two sites and direct immunofluorescence studies were compatible with lupus panniculitis. She was managed as a case of systemic lupus erythematosus (SLE) using a combination of hydroxychloroquine, and oral corticosteroids, which afforded temporary relief of symptoms. The patient however was lost to follow-up and opted for alternative medicine, and subsequently succumbed to the complications of SLE. Conclusion This case highlights the importance of a carefully made assessment after an accurate clinicopathological cor- relation was done. This case also emphasizes that although LP if associated with SLE may signify a milder condition, judicious monitoring and follow-up must still be undertaken since management is based on the disease activity.
Panniculitis, Lupus Erythematosus

Monogenic autoimmune diseases (AD) present as lupus-like clinical manifestations with recurrent fever or various vasculopathies. Recurrent fever with an elevation of acute phase reactants and various skin lesions are similar in monogenic AD and autoinflammatory disease. The molecular pathogenesis of adult systemic erythematosus can be understood through monogenic AD based on gene defects: complement, apoptosis, interferonopathy via nucleic acid sensing, tolerance, rasopathies, and others. Skin vasculopathy with chilblains and livedo reticularis, interstitial lung disease, and panniculitis are common occurrences in type I interferonopathy. Some syndromes have been reported to present with autoimmune inflammation and the general clinical findings, including cerebral calcification. Various clinical manifestations in monogenic AD present in accordance with the gene loss- or gain-of-function mutations involved. The monogenic AD for the early onset of more severe lupus-like symptoms or vasculopathy needs to be considered. Furthermore, clinical trials were conducted via targeted therapy for related molecular pathways, because conventional treatments were not effective in managing monogenic AD.
Acute-Phase Proteins ; Adult ; Apoptosis ; Autoimmune Diseases* ; Chilblains ; Complement System Proteins ; Fever ; Humans ; Inflammation ; Interferons ; Livedo Reticularis ; Lung Diseases, Interstitial ; Lupus Erythematosus, Systemic ; Panniculitis ; Skin

A 9-year-old girl presented with a 2-year history of doughnut-shaped hair loss on the frontal scalp, resembling the symmetric distribution of Blaschko's lines. Physical examination showed an alopecic patch with mild scalp induration. Histopathology revealed lymphoplasmacytic infiltration of the perifollicular dermis and subcutaneous lobules with abundant mucin deposition, consistent with a diagnosis of lupus erythematosus panniculitis. Three cases of linear lupus erythematosus panniculitis of the scalp presenting as alopecia along Blaschko's lines have been documented in Korean dermatologic literature. However, there have been no reports of doughnut-shaped alopecia until now. Herein, we present an interesting case of a pediatric patient with a unique feature of doughnut-shaped alopecia along Blaschko's lines.
Alopecia* ; Child ; Dermis ; Diagnosis ; Female ; Hair ; Humans ; Mucins ; Panniculitis, Lupus Erythematosus* ; Physical Examination ; Scalp*

The clinical manifestations and immunohistologic findings of drug-induced lupus erythematosus (DILE) are similar to those of idiopathic lupus. However, DILE is different from idiopathic lupus because it is induced after continuous drug exposure and resolves after discontinuation of the causative drug. DILE can be divided into systemic lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus (CCLE). Lupus erythematosus profundus is a subtype of CCLE, and drug-induced CCLE is very rarely reported in the literature. Herein, we report a rare case of adalimumab-induced lupus erythematosus profundus developed in a rheumatoid arthritis patient. The patient is a 43-year-old Korean woman who had multiple tender nodules and plaques on her face, trunk, and both extremities after using adalimumab for rheumatoid arthritis. She was diagnosed with adalimumab-induced lupus erythematosus profundus, and her condition improved after discontinuation of adalimumab.
Adult ; Arthritis, Rheumatoid* ; Extremities ; Female ; Humans ; Lupus Erythematosus, Cutaneous ; Lupus Erythematosus, Systemic ; Panniculitis, Lupus Erythematosus* ; Adalimumab

The clinical manifestations and immunohistologic findings of drug-induced lupus erythematosus (DILE) are similar to those of idiopathic lupus. However, DILE is different from idiopathic lupus because it is induced after continuous drug exposure and resolves after discontinuation of the causative drug. DILE can be divided into systemic lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus (CCLE). Lupus erythematosus profundus is a subtype of CCLE, and drug-induced CCLE is very rarely reported in the literature. Herein, we report a rare case of adalimumab-induced lupus erythematosus profundus developed in a rheumatoid arthritis patient. The patient is a 43-year-old Korean woman who had multiple tender nodules and plaques on her face, trunk, and both extremities after using adalimumab for rheumatoid arthritis. She was diagnosed with adalimumab-induced lupus erythematosus profundus, and her condition improved after discontinuation of adalimumab.
Adult ; Arthritis, Rheumatoid* ; Extremities ; Female ; Humans ; Lupus Erythematosus, Cutaneous ; Lupus Erythematosus, Systemic ; Panniculitis, Lupus Erythematosus* ; Adalimumab

Lupus profundus is a rare subtype of chronic cutaneous lupus erythematosus, which shows a tender subcutaneous nodule or plaque. The face, arm, buttock, trunk and thighs are frequently involved. Lupus profundus can be associated with or without systemic lupus erythematosus. But due to its rarity, it is difficult to diagnosis and there are not many reports regarding its characteristics, including its distribution. In this case, a 13-year-old boy has visited with several violaceous non-tender nodules on Lt. upper thigh with segmental distribution, which follows the Blaschko's line. Through skin biopsy and its immunoflourescent study, we diagnosed lupus profundus. We here report a case of lupus profundus with segmental distribution following the lines of Blaschko's in a child.
Arm ; Biopsy ; Buttocks ; Child ; Humans ; Leg ; Lupus Erythematosus, Cutaneous ; Lupus Erythematosus, Systemic ; Panniculitis, Lupus Erythematosus ; Skin ; Thigh

Adult ; Female ; Humans ; Lupus Erythematosus, Discoid ; diagnosis ; Lymphoma, T-Cell ; diagnosis ; Panniculitis ; diagnosis

Kikuchi's disease (KD), histiocytic necrotizing lymphadenitis, is a rare self-limited lymphadenopathy, which usually affects young women. KD has been reported to precede, coexist with or follow the diagnosis of other entities, such as systemic lupus erythematosus (SLE), adult-onset Still's disease, Hashimoto's disease, and viral infections. In a few cases, KD is associated with cutaneous lupus erythematosus (CLE), without systemic involvement. Herein, we report the first Korean case of KD associated with lupus erythematous profundus in a 9-year-old boy.
Child ; Female ; Hashimoto Disease ; Histiocytic Necrotizing Lymphadenitis ; Humans ; Lupus Erythematosus, Cutaneous ; Lupus Erythematosus, Systemic ; Lymphatic Diseases ; Panniculitis, Lupus Erythematosus ; Still's Disease, Adult-Onset

OBJECTIVETo explore the clinical and histopathological manifestations, therapy and prognosis of lupus erythematosus panniculitis (LEP).

METHODWe retrospectively reviewed the clinical data and histopathological features of 22 cases of LEP diagnosed at Peking Union Medical College Hospital from January 2008 to February 2010.

RESULTSThe lesions appeared as atrophy, erythema, subcutaneous nodules, infiltrated erythema, and ulceration over the scalp, face, upper limbs, and buttock. Histopathological features were lobular panniculitis in the subcutaneous fat; sometimes septal panniculitis could be seen. Hydroxychloroquine sulfate and corticosteroid were effective treatment; most patients responded well to the treatment, while a few experienced recurrence when the treatment tapped or discontinued.

CONCLUSIONSLEP is a rare cutaneous variant of lupus erythematosus. The diagnosis of LEP is mainly based on clinical findings and pathological features. Hydroxychloroquine and low- and middle-dose corticosteroid are effective for the treatment of LEP.

Adult ; Aged ; Female ; Humans ; Male ; Middle Aged ; Panniculitis, Lupus Erythematosus ; drug therapy ; pathology ; Prognosis ; Retrospective Studies ; Young Adult

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.
Alopecia ; Alopecia Areata ; Biopsy ; Erythema ; Female ; Hair ; Humans ; Lupus Erythematosus, Systemic ; Panniculitis, Lupus Erythematosus ; Scalp ; Young Adult