Erythrophagocytosis in Dermatitis Ulcerosa (Pyoderma Gangrenosum)

Abstract

There are various names to be found in the medical literature for the same cutaneous disorder with its distinctive morphologic features well recognized as an clinical entity and at present mostly called Pyoderma gangrenosum (Brunsting, Goeakerman a. O’Leary6) or Dermatitis ulcerosa (Kresbach”)-

In this report the term Dermatitis ulcerosa is suggested as the most appropriate since it does not prejudice the etiopatogenecity of the disease which is not known and there is very low indication that the pyogenic infection is the main etiological factor.

Two female patients with classical clinical picture of Dermatitis ulcerosa and typical course of the disease were studied to broaden the concept of this rather vague disorder.

One of the patients had associated colitis ulcerosa and visceral candidiasis (candida albicans). To our experience this condition could not be considered as necessarily related to dermatitis ulcerosa and even less caused by it.

In the other patient no such or any other associated disorder were found.

The very extensive laboratory investigations could not reveal any conclusive finding which could be regarded as specific for dermatitis ulcerosa. Also, the histopathology demonstrated only the usually structures suggestive of the diagnosis.

Antibiotic treatment remained ineffective. However, steroids brought about complete involution of the lesions.

Also, in our patients the initial lesion was sterile showing in the native preparation besides cellular elements (macrophages and other) free lying elastic fibres from the destroyed tissue (illustrated by fig. 4).

It is interesting to note that the lesions in dermatitis ulcerosa — contrary to the normal wound healing — begun to heal (epithelise) in the rule in the central part of the lesion progressing to the periphery though sometimes the borders of the lesion are still in active extension.

The extension of the lesion or recurrences after complete healing \vas always going from the border to further periphery and never back to the area of the previously ulcerated skin this circumstance may indicate that an immunological mechanism might be involved (skin specific hypersensitivity).

The most peculiar findings in the report relate to the phenomenon of erythrophagocytosis which could be seen in the native preparations and Giemsa strained smears in material taken from the active lesions particularly from the peripheral borders in extension.

The macrophages seen in this material may contain numerous erythrocytes which appear to be intact and have been phagocytised whole rather than after rupture. Sometimes there could be observed in the native preparation erythrophagocytosis at different stages of the active process of phagocytosis as adherence of erythrocytes (occasionally becoming elongated) to the phagocyte, then becoming surrounded and drown into the macrophage (illustrated by fig. 5—10).

In peripheral blood smears erythrophagocytosis could not be observed in the reported patients.

This phenomenon which to our knowledge has not previously been recorded in dermatitis ulcerosa (Pyoderma gangrenosum and other denotation for the same disorder) is suggested to be a process of tissue mediated immunological mechanism.

In discussing the presumptive etiopathogenetic factors of dermatitis ulcerosa an immunological mechanism seems to be indicated although no sufficient proved evidence as reflected by altered immunoglobulin classes or respective antibodies is at present available.