Pemphigoid nodularis

Pemphigoid nodularis
Synonyms	Nodular pemphigoid
Pronounce	N/A
Specialty	Dermatology
Symptoms	Nodules, blisters, itching
Complications	Infection, scarring
Onset	Typically in adulthood
Duration	Chronic
Types	N/A
Causes	Autoimmune disorder
Risks	Age, genetic predisposition
Diagnosis	Skin biopsy, immunofluorescence
Differential diagnosis	Pemphigus vulgaris, dermatitis herpetiformis
Prevention	N/A
Treatment	Corticosteroids, immunosuppressants
Medication	Prednisone, azathioprine
Prognosis	Variable, can be managed with treatment
Frequency	Rare
Deaths	N/A

Pemphigoid nodularis is a rare variant of Bullous pemphigoid, a chronic autoimmune skin disease. It is characterized by the presence of nodular lesions, in addition to the typical blistering lesions seen in bullous pemphigoid.

Clinical Features

Patients with pemphigoid nodularis present with pruritic, nodular lesions that may be accompanied by vesicles or bullae. The nodules are typically firm and may be located anywhere on the body, but are most commonly found on the extremities. The vesicles or bullae are usually tense and filled with clear fluid.

Pathogenesis

The pathogenesis of pemphigoid nodularis is not fully understood. It is thought to be an autoimmune disease, with autoantibodies targeting the basement membrane zone of the skin. This leads to inflammation and the formation of blisters.

Diagnosis

Diagnosis of pemphigoid nodularis is based on clinical features, histopathological examination, and immunofluorescence studies. Histopathology typically shows a subepidermal blister with a dense eosinophilic infiltrate. Direct immunofluorescence shows linear deposition of IgG and C3 along the basement membrane zone.

Treatment

Treatment of pemphigoid nodularis involves the use of systemic corticosteroids and immunosuppressive agents. Topical corticosteroids may also be used for localized disease.

Prognosis

The prognosis of pemphigoid nodularis is generally good with treatment. However, the disease tends to be chronic and may require long-term management.

See Also

• Bullous pemphigoid
• Autoimmune diseases
• Dermatology

References

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