Pathology of Lupus Pernio (Cutaneous Sarcoidosis )

Dr Sampurna Roy MD            

It was first described in 1889 by Ernest Besnier.

Lupus pernio presents as a violaceous lesion or shiny nodules.

The lesions are usually located over the head and neck region and is predominantly localized to the nose, cheeks, and ears.

Lupus pernio can present either as an isolated skin lesion or it can be an early manifestation of systemic sarcoidosis. It is often a predictor of systemic sarcoidosis.

Patients with lupus pernio have a higher risk for pulmonary disease.Pulmonary parenchymal involvement was present more often in patients with lupus pernio or sarcoidosis involving scar tissue compared to patients with erythema nodosum or other skin lesions of sarcoidosis.

Skin sarcoidosis occurs in about 25% of patients with systemic disease and may also arise in isolation.

A wide range of clinical presentations of cutaneous sarcoidosis is recognised.

The diagnosis rests on the presence of non-caseating granulomas on skin biopsy and the exclusion of other granulomatous skin disease.

The treatment and overall prognosis of cutaneous sarcoidosis is primarily dependent on the degree of systemic involvement.

In patients with aggressive disease limited to the skin immunosuppressive therapy may be indicated.  

Related posts:  

Erythema Nodosum; 

Microscopic Images of Cutaneous Sarcoidal Granulomas:

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Dermatopathology Quiz Case No 63: 

Further reading:

The histologic spectrum of cutaneous sarcoidosis: a study of twenty-eight cases.

Comparison of cutaneous sarcoidosis with systemic sarcoidosis: a retrospective analysis

Verrucous cutaneous sarcoidosis: case report and review of this unusual variant of cutaneous sarcoidosis.

Adaptive Immune Responses in Primary Cutaneous Sarcoidosis

Diagnosis of cutaneous sarcoidosis; clinical and the prognostic significance of skin lesions

Granulomatous cutaneous sarcoidosis: diagnosis, relationship to systemic disease, prognosis and treatment.

Sarcoidosis can present with necrotizing granulomas histologically: two cases of ulcerated sarcoidosis and review of the literature.

Skin manifestations of sarcoidosis.

Clinical spectrum and histological analysis of 32 cases of specific cutaneous sarcoidosis.