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Fox – Fordyce disease

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports Open full size image Obliteration of the follicular infundibulum with keratin in the apocrine gland–bearing skin is the cause of this rare, paroxysmally intensely itchy condition. Apocrine sweat retention and rupture of the gland duct under periods of apocrine sudomotor…

Folliculitis decalvans

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports Open full size image Folliculitis decalvans (FD) is a rare, neutrophilic, inflammatory scalp condition presenting as single or multiple areas of alopecia, characterized by pustule formation, erythema, and increased scale with ‘tufted’ hair follicles evident at the edge of the…

Folliculitis

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports Open full size image Folliculitis is defined as inflammation of the pilosebaceous unit, which can occur superficially, presenting as tender, red papules and pustules, or within the dermal portion of the follicle manifesting as abscesses and furuncles. The etiology of…

Follicular mucinosis

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports Open full size image Follicular mucinosis is characterized histologically by mucinous degeneration of the follicular outer root sheaths and sebaceous glands with an inflammatory infiltrate composed of lymphocytes, histiocytes, and eosinophils. Clinically it presents as erythematous, scaly, and infiltrated plaques…

Flushing

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports Open full size image Flushing is defined as visible reddening of the skin over the face, neck, upper portion of the chest, and upper limbs, in association with the sensation of warmth. Management Strategy The initial step in the management…

Fabry disease

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports Open full size image Fabry disease (or Anderson–Fabry disease, OMIM 301500) is a progressive, X-linked lysosomal storage disorder caused by the deficiency or absence of the enzyme α-galactosidase A (GLA). This results in the accumulation of globotriaosylceramide (Gb3) and related…

Extramammary Paget disease

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports Open full size image Extramammary Paget disease (EMPD) is a rare malignancy, first described by Crocker in 1889, who reported lesions on the scrotum and penis that were histologically similar to mammary Paget disease. EMPD most commonly occurs on apocrine…

Erythropoietic protoporphyria

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports Open full size image In this metabolic disorder, a genetically determined ferrochelatase enzyme deficiency in bone marrow erythroid cells causes excessive protoporphyrin levels in erythrocytes, plasma, liver, bile, and feces. Protoporphyrin is a photoactive heme synthesis intermediary. In the skin,…

Erythromelalgia

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports Open full size image Erythromelalgia ( erythros – redness; melos – extremity; algos – pain) is an uncommon neurovascular disorder characterized by redness, increased skin temperature, and pain that usually occurs in the extremities. Pain is aggravated by warming and…

Erythrokeratodermas

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports Courtesy of Dr Margarita Larralde, Buenos Aires, Argentina. Open full size image Erythrokeratodermas (EK) are a clinically and genetically heterogeneous group of rare inherited disorders of cornification characterized by two distinct morphologic features: localized hyperkeratosis and erythema. The best characterized…

Erythroderma

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports Open full size image Erythroderma (ED) is a skin reaction pattern presenting as an extreme state of anatomical and physiological dysfunction characterized by extensive erythema and scaling involving more than 90% of the body surface area. Several skin disorders may…

Erythrasma

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports Open full size image In its most typical form, erythrasma is characterized by well-defined, reddish-brown, flexural plaques that show fine scaling and no tendency to central clearing. It may also present with maceration of the toe webs. The responsible organism,…

Erythema nodosum

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports Open full size image Erythema nodosum (EN) is an inflammatory septal panniculitis that presents as tender, erythematous nodules and plaques distributed symmetrically on the extensor surfaces of the lower extremities. EN is thought to be a delayed-type hypersensitivity reaction and…

Erythema multiforme

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports Open full size image Erythema multiforme (EM) is a distinct cutaneous reaction pattern to a variety of stimuli, predominantly herpes simplex virus (HSV) infection, occurring mostly in young adults. It usually runs a self-limiting course but has a tendency to…

Erythema elevatum diutinum

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports Open full size image Erythema elevatum diutinum (EED) is a rare neutrophilic dermatosis consisting of violaceous, brown or red papules, plaques, nodules, and occasionally vesicobullous lesions over the extensor surfaces of the joints and buttocks, genitalia, trunk, and face. Early…

Erythema dyschromicum perstans

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports Open full size image Erythema dyschromicum perstans (EDP) is an acquired, generalized dermal hypermelanosis of unknown etiology. Clinically it presents as asymptomatic, ashen-gray-blue macules of varying sizes, most commonly on the trunk and proximal extremities. Variable components include erythema and…

Erythema annulare centrifugum

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports Courtesy of Joslyn Kirby. Open full size image Background Erythema annulare centrifugum (EAC) is a gyrate erythema characterized by minimally pruritic, polycyclic, erythematous patches or plaques that may expand up to 2–3 mm/day and clear centrally. There are two forms:…

Erosive pustular dermatosis

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports Open full size image Erosive pustular dermatosis (EPD) is a rare condition characterized by pustular, erosive, and crusted lesions in areas of alopecia that tend to be atrophic, actinically damaged, or subject to local iatrogenic or external trauma. The condition…

Epidermolysis bullosa acquisita

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports Courtesy Iris Aronson Open full size image Epidermolysis bullosa acquisita (EBA) is an uncommon, chronic, autoimmune blistering disease affecting the skin and mucous membranes. The disorder affects patients of any age, and the skin lesions occur commonly at trauma-prone skin…

Epidermolysis bullosa

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports Open full size image Epidermolysis bullosa (EB) comprises a group of rare, genetically inherited mechanobullous skin disorders. Underlying mutations impair the structural and functional integrity of the epidermis and dermoepidermal basement membrane zone (BMZ) of skin and mucous membranes. The…