Manifestações cutâneas de doenças sistêmicas

O que são manifestações cutâneas?

A pele pode ser afetada por uma ampla variedade de condições subjacentes e é sempre importante considerar essa possibilidade quando um paciente apresenta qualquer lesão cutânea que não tenha um diagnóstico claro, como dermatite ou psoríase. Este artigo fornece uma visão geral de algumas das condições mais comumente encontradas na prática, que podem se manifestar por alterações na pele.

Skin manifestations of hiperlipidemia include flat yellow deposits around the eye (xanthelasmata). Elsewhere on the body they present as yellowish papules or nodules called xanthomata. Sudden eruptions can appear in large numbers over the buttocks, trunk and limbs, or a few larger lesions can develop on the elbows, knees, hands and over the Achilles tendon.

Doença inflamatória intestinal

Manifestações cutâneas encontradas na doença inflamatória intestinal (Doença de Crohn, colite ulcerativa) can include pioderma gangrenoso e eritema nodoso.² Oral manifestations include aphthous stomatitis, mucosal nodularity (cobblestoning) and pyostomatitis vegetans (erythematous thickened mucosa).³

Síndrome carcinoide

Cutaneous metastases in síndrome carcinoide can present as deep nodules, hyperkeratosis and pigmentation changes similar to those seen in pellagra.⁴

Recurrent skin infections are common with diabetes mellitus, either due to fungi (eg, candidíase genital) or bacteria (eg, foliculite). Blisters and granuloma anular may be found on the feet.⁵

Máculas marrons às vezes se desenvolvem na canela; quando estas formam bolhas, a condição é chamada de bullosis diabeticorum.⁶ Necrobiose lipoídica diabeticorum can also occur on the shins. The lesions have the appearance of plaques with dark red or purple edges, atrophic centres and surface telangiectasias.

As manifestações cutâneas caracteristicamente incluem:

• Icterícia.

• Eritema das palmas.

• Púrpura.

• Nevo aranha.

• Coceira generalizada.

Líquen plano is known to be linked to infecção por hepatite C.⁷

A variety of skin conditions can occur in vírus da imunodeficiência humana (HIV)/síndrome da imunodeficiência adquirida (AIDS).

Dermatite

The appearance can resemble psoríase, artrite reativa ou dermatite seborreica and has been termed 'psoriasiform dermatitis of AIDS'.

Infecções

Bacillary angiomatosis is a treatable infection caused by a rickettsia-like organism similar to Rochalimaea quintana - the agent of febre das trincheiras. Viral infections such as chronic herpes zoster can also occur.⁹

Malignidade

Sarcoma de Kaposi is an AIDS-defining illness characterised by initial bruise-like macules developing into brown-red or purple firm-to-hard nodules. In AIDS these are widespread, especially on the face and trunk.¹⁰

Sarcoidose can present with eritema nodoso, ou com placas, pápulas ou nódulos. Estes últimos são comumente vistos como lesões lisas, de cor marrom escuro/violáceo, dispostas em um padrão anular. A infiltração de uma cicatriz antiga é característica, tornando-se de cor roxa brilhante. O lúpus pérnio é uma forma de desenvolvimento lento que se espalha em grandes áreas de placa no queixo e no nariz.

Mastocitose is a condition in which there is proliferation of mast cells. In the skin it can cause single or multiple, dark red nodules or plaques that develop into a blister or wheal when rubbed. This is at its most common in babies. It can also cause urticaria pigmentosa, either as skin wheals in infancy often following a bath, or as extensive areas of dark brown macules that swell and go red when stroked in adults.

Amiloidose appears in middle age with bruising, petechiae and purpura related to deposition of amyloid in the dermal blood supply. This is most frequently seen in the anogenital, periorbital and peri-umbilical regions, at the side of the neck and in the axillae. Atrophic waxy lesions with areas of purpura inside them may sometimes be seen, and the tips of the fingers may exhibit softening and loosening of the skin. A case of advanced primary amyloidosis presenting as a non-healing leg ulcer has also been reported.¹⁴

A amiloidose cutânea está associada a vários distúrbios autoimunes/imunológicos, e associações com sarcoidose e nefropatia por IgA foram relatadas.¹⁵

Cutaneous changes in acromegalia can include skin puffiness, oily skin with large pores, hypertrichosis, pigmented skin tags, acanthosis nigricans and psoriasis.¹⁶ Skin creases in the head area are deeper than normal (cutis verticis gyrata, or 'Klingon head').¹⁷

Em hipopituitarismo the skin is often dry, scaly and puffy and the nails become brittle. The hair is coarse and sparse, especially in the axillae. Fine wrinkles around the eyes and mouth are typical.

Em hipotireoidismo the skin in myxoedema is cool to the touch, doughy, dry and puffy and there may be hair loss. Peri-orbital oedema may be accompanied by a yellowish colour to the skin.

Em hipertireoidismo the skin is warm and moist and flushing of the face and palms is sometimes seem. Pretibial myxoedema is is an autoimmune manifestation of Graves' disease, but also occasionally occurs in tireoidite de Hashimoto.

Em Síndrome de Cushing abdominal striae may be a prominent feature, caused by skin atrophy. The skin may bruise easily and skin infections and acne may be frequent problems. Skin darkening may occur in the palmar creases, on areas subject to pressure and in the axillae.¹⁸

A insuficiência adrenal primária (doença de Addison) causa hiperpigmentação, particularmente evidente na mucosa bucal, lábios, pregas palmares, novas cicatrizes e em áreas sujeitas a pressão, como cotovelos, nós dos dedos e joelhos. As alterações não estão presentes na insuficiência adrenal secundária.

There are a number of different forms of porfiria - eg, porphyria cutanea tarda, erythropoietic porphyria.^{22 23} All are characterised by photosensitivity, with fragility and blistering of the skin when exposed to sunlight or ultraviolet rays.

Rheumatoid nodules - subcutaneous lumps seen near an affected joint - occur in about 25% of patients with artrite reumatoide. Other phenomena include thinning of the skin, translucency of the skin on the back of the hands, brittle nails which split lengthwise and reddened palms (palmar erythema). Dermatitis in which neutrophils are prominent on biopsy (neutrophilic dermatosis) may present as erythematous areas and interstitial granulomatous dermatitis is a rare condition in which rheumatoid papules may appear on the trunk. Cutaneous vasculite may present as purpuric areas on the skin.

Keratoderma blennorrhagicum is sometimes seen in artrite reativa. É caracterizado por lesões hiperqueratóticas nas palmas das mãos ou nas solas dos pés. Vesículas claras em uma base eritematosa se desenvolvem, progredindo para máculas, pápulas e nódulos. As lesões podem ser impossíveis de distinguir da psoríase pustulosa.²⁵

Em síndrome mielodisplásica various cutaneous manifestations can occur, including leukaemia cutis, photosensitivity, prurigo nodularis and purpura. Cutaneous conditions are thought to indicate that the patient belongs to a high-risk group, associated with bone marrow transformation and hypergammaglobulinaemia.²⁶