Hepatic impetigo herpetiformis Hebra

Impetigo herpetiformis (syn. Hebra's impetigo herpetiformis) is a disease with a severe course and possible fatal outcome. It usually develops in the second half of pregnancy, but occasionally similar changes occur in men.

The disease was first described in 1872 by Hebra. Herpetiform impetigo is very rare. It mainly affects pregnant women, but sometimes non-pregnant women, men and children also suffer from this disease.

[ 1 ], [ 2 ], [ 3 ], [ 4 ], [ 5 ], [ 6 ], [ 7 ]

Causes of Impetigo Herpetiformis Hebra

There is no single point of view on the origin of the disease. Patients with herpetiform dermatitis often have dysfunction of the endocrine glands (parathyroid and genital). This is evidenced by the fact that the disease mostly occurs during pregnancy or in the puerperal period.

There is an infectious theory, since the contents of the pustules are sterile in some cases, while in others they contain staphylococci or streptococci.

Histopathology of Hebra's impetigo herpetiformis

In the uppermost parts of the Malpighian network there is a pustule, which is formed as a result of the penetration of neutrophils here, which causes the destruction of the protoplasm and nuclei of epithelial cells. The remaining cell walls form a spongy network with neutrophils in it - the so-called spongiform pustule of Kagoi, which is a hypertrophic form of Munro's microabscess. The infiltrate in both the dermis and the epidermis contains many eosinophils.

The pathomorphology of Hebra's impetigo herpetiformis is characterized by the presence of spongiform pustules of Kogoy in the upper parts of the epidermis. Large pustules are sometimes located one under another. The contents of the pustules are neutrophilic granulocytes, sometimes with an admixture of mononuclear elements or eosinophilic granulocytes. In the dermis, there is pronounced edema, vasodilation and inflammatory perivascular infiltrates of a lymphohistiocytic nature or with an admixture of neutrophilic and eosinophilic granulocytes, tissue basophils.

Histogenesis of impetigo herpetiformis Hebra

In its course and prognosis, herpetiform impetigo is very close, if not identical to the primary pustular form of generalized pustular psoriasis. At the same time, some authors consider it a pustular variant of common psoriasis or an independent nosological entity. Great importance in the development of the disease is given to hormonal disorders (sex hormones, parathyroid hormones), hypocalcemia, hemolytic streptococcus, and immune disorders. The role of maternal and fetal incompatibility according to the HLA system is indicated. According to the immunomorphological characteristics, herpetiform impetigo is close to bullous pemphigoid: deposits of IgG, IgA, and the C3 component of complement are found in the basement membrane of the epidermis, circulating antibodies against the basement membrane of the epidermis.

Symptoms of Impetigo Herpetiformis Hebra

Small grouped pustular eruptions are localized mainly on the trunk and in skin folds on a hyperemic edematous background. Due to the spread along the periphery, the lesions can occupy large areas of the skin. When the pustules dry up, peeling occurs in the form of a collar facing the center of the element. After the process is resolved, hyperpigmentation of the skin often remains at the site of the elements. The rash is accompanied by a pronounced deterioration in the general condition of the patient (loss of appetite, headache, fever with chills, arthralgia, etc.).

On the inflammatory edematous background, herpetiform miliary or small pustules appear, which partially merge with each other. The elements, spreading centrifugally, form ring-shaped lesions. In this case, figures of the iris type can form, i.e. rings in rings. Pustules contain greenish-yellow pus, after the outpouring of which the lesions are covered with dirty-brown crusts. There is no itching. The process does not leave persistent traces, as well as pronounced pigmentation. The pathological process is often localized in the groin area, in the navel area, on the inner thighs, in the armpits, in the mammary glands, and then can gradually capture the entire or almost the entire surface of the skin. The mucous membranes of the oral cavity are often involved, sometimes - the mucous membrane of the larynx, esophagus, conjunctiva. The disease is accompanied by severe general symptoms: prolonged or intermittent septic fever, headache, chills, vomiting, diarrhea, joint pain, etc. The disease often recurs. No eosinophilia is observed in the blood, but an increase in ESR is observed.

Differential diagnosis

The disease must be distinguished from herpes gestationis, pustular psoriasis, subcorneal dermatitis, and acrodermatitis of Gallopeau.

Treatment of herpetiform impetigo Hebra

Glucocorticosteroids in combination with antibiotics, calcium preparations, vitamin D are recommended. Aniline dyes and steroid ointments are used locally. Patients often require resuscitation.