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Distinct Variants

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Some people are "lumpers" and others are "splitters" and depending on who named a disease, sometimes the line may be drawn between two diseases that are actually just different versions of the same condition.  Even if you disagree with some of these, it is useful to think about it and helpful in remembering different diseases and their similarities and differences by lumping them in this way:

 

still uncertain whether eosinophilic fasciitis is a distinct entity or if it represents a deep form of scleroderma;  differs from scleroderma….
absence of Raynauds, (-) ANA
visceral involvement (i.e. esophageal dysmotility or pulmonary fibrosis) is rare
and lesions usually confined to the extremities (sparing fingers and toes)

 

Hailey-Hailey was once considered to be the vesicular variant of Dariers (but Dariers is chromosome 12q vs. H-H is 3q)

 

some view Jessners benign lymphocytic infiltrate as a chronic variant of DLE

 

Ackerman says tumid LE, Jessners, and REM are the same disease (small amounts of mucin present in some Jessners)

 

actinic granuloma  appears like necrobiosis lipoidica but appears on the head and neck, hands, or forearms, commonly in middle-aged, non- diabetic women (a variant of the NLD-GA spectrum) (some say that it is simply GA on sun-damaged skin)

 

atrophoderma is simply a variant of morphea (?) with more atrophy than sclerosis

 

livedo vasculitis and atrophie blanche (see Ackermans Quandaries)

 

granuloma faciale and EED appear to be closely related and perhaps different manifestations of the same disease  (EED far more vascular involvement, less epidermal sparing, and fewer eosinophils than granuloma faciale)

 

eosinophilic pustular folliculitis may represent a more persistent form of erythema toxicum neonatorum

 

Mal de Meleda is essentially Papillon-Lefevre syndrome (i.e. palmoplantar keratoderma with transgradiens) but minus the dental abnormalities

 

Focal acral hyperkeratosis is the same as Acrokeratoelastoidosis of Costa but lacks elastorrhexis on biopsy

 

REM a variant of tumid LE (?):

both respond to antimalarial therapy (i.e. Plaquenil)
both with similar histology:  superficial and deep, perivascular and periadnexal lymphocytic infiltrate with interstitial mucin deposition

 

REM (reticular erythematous mucinosis):

mainly localized on central chest and upper back
young to middle aged women
can be induced by sun exposure, but provocative phototesting often negative

 

tumid LE:

sun exposed areas (especially face, V area of neck, upper back)
provocative phototesting positive in 70% of patients

 

 

 

 

 

I have trouble understanding the difference, or the need for distinction, between  (i.e. Im a lumper):

palmoplantar pustulosis and acrodermatitis continua of Hallapeau
large plaque parapsoriasis and patch stage MF
perioral dermatitis,  FACE,  granulomatous rosacea,  and lupus miliaria disseminata faceii