Photogallery. Rare dermatoses. Part II

Abstract

In continuation of orphan dermatosis, I would also like to say about diseases such as lichen planus ― which is an autoimmune disease in which the expression of an antigen that has not yet been identified by keratinocytes of the basal layer leads to the activation and migration of T-lymphocytes into the skin with the formation of an immune response and an inflammatory reaction.

Bourneville–Pringle syndrome (tuberous sclerosis complex) is a rare genetic disease that is part of the group of phakomatoses, characterized by a polysystemic lesion involving the skin, central nervous system, organs of vision, etc. in the pathological process.

Bullous scleroderma is a rare type of localized scleroderma with a predominant skin lesion in the form of foci of induration and sclerosis, subepidermal blisters with serous contents.

Trichoadenoma is a rare skin tumor belonging to the group of benign follicular tumors. Consists of numerous, relatively large, round or oval funnel-shaped structures separated by an underdeveloped fibrous stroma.

Pseudolymphoma is a reactive polyclonal T- or B-cell lymphoproliferative process that can manifest as both localized and disseminated skin lesions.

Giant flaming nevus of the skin of the face is usually a benign disease, but in some cases malignancy is possible.

T-cell anaplastic lymphoma associated with sarcoidosis is characterized by lymphadenopathy, lesions, circulating abnormal lymphocytes, and hypercalcemia leading to the sarcoidosis-lymphoma syndrome.

Devergy's disease is caused by a violation of keratinization and the appearance of follicular hyperkeratotic papules, palmoplantar hyperkeratosis and erythematous scaly plaques with an orange tint.

Favre–Shay ocher yellow dermatitis occurs in patients with varicose ulcers on the inner surface of the lower third of the legs, which it either precedes or accompanies.

Sweet's febrile neutrophilic dermatosis manifests itself in three clinical cases: classical (the disease is preceded by an infectious disease), malignant (occurs as a paraneoplastic syndrome), medicinal (most often occurs in patients treated with G-CSF).

Lipodermatosclerosis is manifested by induration and hyperpigmentation of the skin, affecting one or both legs, in a characteristic "upside down champagne bottle" appearance.

Keywords

lichen planus / Bourneville-Pringle syndrome / bullous scleroderma / trichoadenoma / pseudolymphoma / giant flaming nevus of the facial skin / T-cell anaplastic lymphoma in combination with sarcoidosis / Devergy's disease / Favre-Chay ocher-yellow dermatitis / febrile Sweet's neutrophilic dermatosis / lipodermatosclerosis

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