Congenital erosive and vesicular dermatosis | |
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Specialty | Dermatology |
Congenital erosive and vesicular dermatosis is a cutaneous condition characterized by generalized erosions, vesicles, crusting and 'scalded skin-like' erythematous areas affecting up to 75% of the body surface area. [1]
Congenital erosive and vesicular dermatosis exhibits erythema, vesicles, erosions, crusts, and fissures affecting more than 75% of the skin at birth. Within ten days to three months, the lesions heal on their own, leaving behind a pathognomonic reticulate scar. [2]
Clinical characteristics linked to congenital erosive and vesicular dermatosis include cicatricial baldness (39%), nail dysplasia or hypoplasia (39%), hyperthermia with/or hypohidrosis in scarred areas (39%), ophthalmological abnormalities (36%), and neurodevelopmental abnormalities (30%). [3]
There is no established pathophysiology for congenital erosive and vesicular dermatosis, and the illness seems to be sporadic. Intrauterine infections, amniotic adhesions, and a developmental abnormality with atypical repair in preterm skin are among the etiologic possibilities. [4]
The congenital erosive and vesicular dermatosis histopathologic results vary according to the disease's stage. [4] Epidermal necrosis, subepidermal vesiculation, and an eroded epidermis with a primarily neutrophilic or mixed (containing eosinophils, histiocytes, lymphocytes, and neutrophils) dermal infiltration have all been reported in early inflammatory lesions. [3] Biopsy specimens from late lesions might either look normal in the dermis or exhibit scar development with a decrease in hair follicles and absent eccrine glands, according to histopathologic evaluation. [5]
Amelanotic melanoma is a type of skin cancer in which the cells do not make any melanin. They can be pink, red, purple or of normal skin color, and are therefore difficult to diagnose correctly. They can occur anywhere on the body, just as a typical melanoma can.
Pili torti is characterized by short and brittle hairs that appear flattened and twisted when viewed through a microscope.
Anetoderma is a benign but uncommon disorder that causes localized areas of flaccid or herniated sac-like skin due to a focal reduction of dermal elastic tissue. Anetoderma is subclassified as primary anetoderma, secondary anetoderma, iatrogenic anetoderma of prematurity, congenital anetoderma, familial anetoderma, and drug-induced anetoderma.
Palisaded neutrophilic and granulomatous dermatitis (PNGS) is usually associated with a well-defined connective tissue disease, lupus erythematosus or rheumatoid arthritis most commonly, and often presents with eroded or ulcerated symmetrically distributed umbilicated papules or nodules on the elbows.
Acrokeratoelastoidosis of Costa or Acrokeratoelastoidosis is a hereditary form of marginal keratoderma, and can be defined as a palmoplantar keratoderma. It is distinguished by tiny, firm pearly or warty papules on the sides of the hands and, occasionally, the feet. It is less common than the hereditary type of marginal keratoderma, keratoelastoidosis marginalis.
Multifocal lymphangioendotheliomatosis, also known as congenital cutaneovisceral angiomatosis with thrombocytopenia and multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT), is a skin condition that presents at birth with hundreds of red-brown plaques as large as several centimeters.
Targetoid hemosiderotic hemangioma, also known as a hobnail hemangioma is a skin condition characterized by a central brown or purplish papule that is surrounded by an ecchymotic halo. It may appear similar to melanoma. It was first described by Santa Cruz and Aronberg in 1988.
Erosive pustular dermatitis of the scalp presents with pustules, erosions, and crusts on the scalp of primarily older Caucasian females, and on biopsy, has a lymphoplasmacytic infiltrate with or without foreign body giant cells and pilosebaceous atrophy.
Melanonychia is a black or brown pigmentation of a nail, and may be present as a normal finding on many digits in Afro-Caribbeans, as a result of trauma, systemic disease, or medications, or as a postinflammatory event from such localized events as lichen planus or fixed drug eruption.
Generalized bullous fixed drug eruption (GBFDE) most commonly refers to a drug reaction in the erythema multiforme group. These are uncommon reactions to medications, with an incidence of 0.4 to 1.2 per million person-years for toxic epidermal necrolysis and 1.2 to 6.0 per million person-years for Stevens–Johnson syndrome. The primary skin lesions are large erythemas, most often irregularly distributed and of a characteristic purplish-livid color, at times with flaccid blisters.
Linear IgA bullous dermatosis is a rare immune-mediated blistering skin disease frequently associated with medication exposure, especially vancomycin, with men and women being equally affected. It was first described by Tadeusz Chorzelski in 1979 and may be divided into two types:
Neutrophilic dermatosis of the dorsal hands (NDDH) is a skin condition that presents with edematous pustular or ulcerative nodules or plaques localized to the dorsal hands.
Pressure urticaria or delayed pressure urticaria is a physical urticaria caused by pressure applied to the skin, and is characterized by the development of swelling and pain that usually occurs 3 to 12 hours after local pressure has been applied.
Acral persistent papular mucinosis (APPM) is a rare form of lichen myxedematosus. It is characterized by small papules on the backs of the hands, wrists, and extensor aspects of the distal forearms, with no further clinical or laboratory indications. Lesions tend to persist and may grow in number gradually. Because there are no symptoms, treatment is rarely required.
Generalized granuloma annulare is a skin condition of unknown cause, tending to affect women in the fifth and sixth decades, presenting as a diffuse but symmetrical, papular or annular eruption of more than ten skin lesions, and often hundreds.
Postinflammatory hypopigmentation is a cutaneous condition characterized by decreased pigment in the skin following inflammation of the skin.
Desmoplastic trichoepithelioma is a cutaneous condition characterized by a solitary, firm skin lesion on the face. Familial cases have been reported suggesting a possible genetic link.
Livedoid vasculopathy (LV) is an uncommon thrombotic dermal vasculopathy that is characterized by excruciating, recurrent ulcers on the lower limbs. Livedo racemosa, along with painful ulceration in the distal regions of the lower extremities, is the characteristic clinical appearance. It heals to form porcelain-white, atrophic scars, also known as Atrophie blanche.
Rheumatoid neutrophilic dermatitis, also known as rheumatoid neutrophilic dermatosis, is a cutaneous condition associated with rheumatoid arthritis.
Necrolytic acral erythema is a cutaneous condition that is a manifestation of hepatitis C viral infection or zinc deficiency. In the early stages, bullae, erosions, and erythematous or violaceous papules are its defining characteristics. Well-defined plaques with erythema on the outer rim, lichenification, secondary hyperpigmentation, and fine desquamation on the surface begin to appear in the late phase.