S2C17P11-Hay-Well Syndrome

S2C17P11-1: In the Hay-Well syndrome, a genetic defect produces histologic changes similar to those of necrolytic migratory erythema. The epidermis is atrophic. A granular layer is represented. The dermis is thin and atrophic.

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S2C17P11-2: In this area, the changes are similar to those of necrolytic migratory erythema, but a granular layer is represented. The dermis shows widening of spaces among collagen bundles with an increased prominence of vessels. The changes in the collagenous component is interpreted as atrophy rather than edema.

S2C17P11-3: Another field from a lesion in the Hay-Well’s syndrome shows similar changes. The cytologic features in the superficial unit of the epidermis have a koilocytotic quality.

S2C17P11-4: This one year old infant had a vesiculo-bullous eruption; the clinical impression included acrodermatitis enteropathica. This biopsy specimen is from acral skin. The epidermis shows acanthosis and elongation of rete ridges. There are focal, small, reticulated defects in the epidermis, mostly affecting the basal unit. There are mild perivascular infiltrates of lymphoid cells (the defect in the dermis is an artefact [a tear] possibly related to the condition of the knife which was used to produce the section).

S2C17P11-5: The patterns in part are psoriasiform. Focally, lymphoid cells have migrated into the basal unit of the epidermis; they are associated with small, lytic defects among the keratinocytes. To the right above the red precipitate, some of the defects contain necrotic keratinocytes.

S2C17P11-6: The patterns are focal; they could be easily dismissed as spongiosis. On the other hand, there are necrotic keratinocytes in some of the larger lytic defects. A single, rounded, dyskeratotic cell in present just above the granular layer; a green arrow points to it. Other green arrows identify necrotic keratinocytes in some of the lytic defects in the basal unit of the epidermis.

 

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