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Cousins

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Some diseases can be thought of as "cousins";  they are related but different in at least a few distinct ways.  Learning these diseases together can be useful because examining there similarities and differences side by side helps put each disease into a conceptual context in your mind.

 

Still’s disease and Erythema Marginatum

•both evanescent (hours)
•both reticular/annular erythema
•both histologically similar:  superficial perivascular infiltrate of neutrophils, and dust, but no fibrin or RBC’s (i.e. no LCV)
•both immune complex diseases  (both with symptoms of arthritis)
•memory tool:  both associated with disease activity – Still’s appears in the evening with the high fevers;  e. marginatum is associated with active carditis

 

Erythema Marginatum:

•associated with active carditis
•one of 5 major Jones criteria for Rheumatic Fever:  subcutaneous nodules, Sydenhams chorea, carditis, polyarthritis

 

Still’s disease:

•rash appears in evening with high temperature
•dermal edema with dilated vessels

 

 

Cylindroma and Eccrine Spiradenoma

•both composed of lobules surrounded by hyaline sheath (though more striking in cylindroma)
•both composed of two types of tumor cells
•small basophilic cells – arranged along periphery of the tumor lobules
•larger, paler cells – in the center of the islands
•both belong to the least mature group of appendage tumors
•eccrine spiradenoma – direction of differentiation is toward both ductal and secretory segments of eccrine sweat glands
•cylindroma – a very immature sweat gland epithelioma, partially eccrine and partially apocrine in differentiation

 

cylindroma:

•jigsaw puzzle
•scalp or face

 

eccrine spiradenoma:

•blue balls
•no characteristic distribution
•painful tumor ddx

 

 

 

Epidermolysis Bullosa Acquisita and Bullous SLE

•has the following in common with EBA:
•antibodies recognize EBA antigen (type VII collagen)
•the split occurs in the sublamina densa zone
•indirect immunofluorescence  may be positive in intact or separated skin
•presence of HLA-DR2 is markedly increased in both conditions

 

•bullous LE distinctive features include:
•a diagnosis of SLE by criteria of the ARA
•a widespread, non-scarring vesicobullous eruption
•(skin fragility, scarring, and milia are not features)
•often begins in second or third decade (EBA in fourth or fifth)
•histology characteristically like that of dermatitis herpetiformis (rare in EBA)
•remitting and often resolves in less than a year (EBA often lasts many years)
•most patients respond dramatically to dapsone (EBA patients do not predictably respond to any treatment)

 

 

Eruptive vellus hair cysts vs. Steatocystoma multiplex

•clinically similar
•both can be autosomal dominant
•steatocystoma cysts may contain one or more lanugo hairs
•hybrid cysts with features of both are sometimes seen

 

 

 

 

Generalized Eruptive Histiocytoma

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Xanthoma Disseminatum

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Eruptive Xanthomas

•non-lipidized histiocytes

 

•xanthoma cells*

 

•xanthoma cells*

 

 

•Touton giant cells

 

•Touton giant cells

 

 

•normal serum lipids

 

•increased triglycerides

* xanthoma cells = lipidized histiocytes