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[Acute febrile neutrophilic dermatosis (Sweet syndrome). A retrospective clinical and histological analysis].

Acute febrile neutrophilic dermatosis (Sweet's syndrome) was first described in 1964. The condition presents with rapidly developing inflammatory plaques, a neutrophilic dermal infiltrate, fever and leucocytosis. In this study, clinical and histological features of 18 patients with Sweet's syndrome have been analysed. The arms (83%), face (67%) and legs (67%) were the most common sites of involvement. Morphologically, plaques, nodes and pseudo-vesicles prevailed. Elevated ESR (89%), fever (72%) and peripheral blood leucocytosis (44%) were noted in many, but not all, cases. Histology revealed a dermal infiltrate with numerous neutrophils (100%) without vasculitis. Nuclear dust (72%) and extravasation of erythrocytes (44%) were also encountered. Collagen degeneration was not observed. Differential diagnosis included erythema multiforme, erythema nodosum and adverse drug reaction. Possible causative factors observed were upper respiratory tract infection, gastrointestinal infection, and malignancies. Sweet's syndrome may be considered as an extreme manifestation within a continuous spectrum of cutaneous reactions to various stimuli or as an ill-defined entity that has some overlap with other dermatoses.

Acute Disease↗

Degos' disease: a distinctive pattern of disease, chiefly of lupus erythematosus, and not a specific disease per se.

Degos' disease, known confusingly as malignant strophic papularis, is an uncommon condition of unknown cause characterized by distinctive infarctive lesions in the skin, gastrointestinal tract, and central nervous system; the lesions at the two latter sites often result in death. We deem Degos' disease to be analogous to lupus erythematosus in the sense that each is fundamentally a systemic pathologic process involving several organs, among them the skin, but, moreover, we regard Degos' disease, in most instances, to be an actual manifestation of lupus erythematosus. Histopathologically, the findings in sections of tissue of skin lesions of Degos' disease are indistinguishable from those of one expression of cutaneous lupus erythematosus; immunopathologically, some patients with morphologic findings stereotypical of Degos' disease display signs characteristic of lupus erythematosus. For these reasons, we consider Degos' disease to be a distinctive pattern of disease, rather than a specific disease per se, just as are erythema multiforme, erythema nodosum, leukocytoclastic vasculitis, Sweet's syndrome, and pyoderma gangrenosum, to name but five of scores of them. The singular pattern that is designated Degos' disease usually is an expression of lupus erythematosus, but, episodically, of conditions like dermatomyositis and rheumatoid arthritis.

Adolescent↗

[Allergic phenomena in the course of dermatomycoses].

Fungi, well known as infectious factors so far, are important allergens too. A fungal id reaction is defined as a distant skin manifestation of an established fungal infection. The most typical setting is in association with tinea of the feet. The main id reactions are well established. A widespread lichen trichophyticus--eruption of small follicular papules grouped or diffuse. The eruption is symmetrical, usually pronounced on the trunk but in severe cases extending down to the lower limbs, even sometimes involving the face. The common cause of this type of id reaction is scalp ringworm of kerion type, caused typically by Trichophyton verrucosum. The treatment of the original ringworm lesion may play a role in initiation of the process. A pompholyx-like id affecting the interdigital spaces and palmar surfaces of the fingers, the palms and sometimes the dorsal surfaces of the hands. This eruption is associated with an acute inflammatory tinea of the feet and may develop spontaneously or as a result of inappropriate treatment. The palmar and web space skin may be covered with papules or vesicles. Sometimes blisters or pustules may occur. Other varieties of mycides include eczematous eruptions, erysipelas-like dermatitis, psoriasiform dermatitis, erythema multiforme, erythema annulare centrifugum, erythema nodosum, and urticaria. Adequate antifungal therapy is recommended as the treatment of allergic diseases caused by superficial fungi.

Candida↗

Reactions and their management.

The uneventful response to chemotherapy in leprosy is marked by clinically disturbing episodes encountered in 20-30% of patients and these phenomena are called "reactions". Generally they are classified as reversal reaction (type-1) and erythema nodosum leprosum (type-2). The cutaneous menifestations are: (1) Type-2 reactions in LL, BL types constituting erythema nodosum leprosum, erythema multiforme, erythema necroticans, subcutaneous nodules, lepromatous exacerbation. (2) Type-1 reactions in borderline and tuberculoid leprosy. The other manifestations include: Acute neuritis, lymphadenitis, arthritis, oedema of the hands and feet, ocular lesions, etc. Sequelae of reactions are: Paralytic deformities, non-paralytic deformities, extensive scarring and renal damage. A simple guideline to identify the risk-prone cases has been narrated. Prednisolone in standard dosage schedule as recommended by WHO is now being widely used in control programmes.

Arthritis↗

Acute febrile neutrophilic dermatosis--a marker of malignancy?

A retrospective survey during a 2-year period disclosed 18 patients with acute febrile neutrophilic dermatosis (Sweet's syndrome). An associated lympho- or myeloproliferative malignancy was found in 6 patients. Attacks of Sweet's syndrome preceded the diagnosis of neoplasia in 4 patients (3 months to 6 years). Some differences in symptoms and signs were found in the group of patients with associated malignancy compared with the group without, that is, male predominance, mucosal symptoms, anemia, and frequent recurrence of skin symptoms. The onset of Sweet's syndrome indicates an acute infectious disease, and the patients are frequently referred to departments of internal medicine and infectious diseases. In addition, the skin lesions may mimic those which often accompany a generalized infection (erythema multiforme, erythema nodosum, vasculitis, pustular eruptions and urticaria). Since Sweet's syndrome may precede the possibly associated malignant disease, the initial diagnosis of the syndrome is important and should be made with confidence with increasing awareness of the characteristic symptoms.

Acute Disease↗

Pruritic rash associated with cat scratch disease.

Cat scratch disease is a benign, self-limited illness characterized by regional lymphadenopathy that usually occurs in association with a history of contact with a cat. Cases of cat scratch disease with skin manifestations that included erythema nodosum; erythema multiforme; erythema marginatum; and non-specific maculopapular, petechial, and morbilliform rashes have been reported. No case of pruritic rash associated with cat scratch disease has been previously reported. In fact, one authority specifically states that the rash of cat scratch disease is nonpruritic. We report a well-documented case of cat scratch disease in which the patient's principal symptom was a pruritic rash. It is possible that this rash was the result of an immunologic reaction to the infectious agent of cat scratch disease. We conclude that cat scratch disease should be included in the differential diagnosis of pruritic rashes in children.

Cat-Scratch Disease↗

Plasma activity of interleukin-10 in drug-induced cutaneous reactions.

Plasma concentrations of interleukin-10 (IL-10) were examined in 126 patients with drug-induced cutaneous reactions: maculopapular eruptions (ME), erythema multiforme (EM), erythema multiforme coexisting with erythema nodosum (EMN), drug-induced urticaria (DU), hyperergic vasculitis (HV), Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN). Activity of the cytokine was measured using the immunoenzymatic ELISA method: a) in the acute stage of disease before treatment was administered, and b) after clearing of skin lesions, after treatment. In the acute stage of disease highly elevated mean concentrations of IL-10 in all 6 groups of patients were found (p<0.001) in comparison with the control. After clearing of clinical symptoms IL-10 concentrations were decreased highly significantly (ME, EM, DU, HV) or significantly (EMN, SJS/TEN) in comparison with the values before treatment, but remained still considerably elevated (p<0.001; p<0.01) when compared with the healthy control. Results of this study indicate that the compensatory antiinflammatory response, expressed as elevated IL-10 activity, is induced as early as in the acute stage of skin lesions and lasts longer than clinical symptoms of drug-induced cutaneous reactions.

Adolescent↗

Interleukin-2 and its soluble receptor in selected drug-induced cutaneous reactions.

Plasma concentrations of interleukin-2 (IL-2) and its soluble receptor (sIL-2R) were examined in 126 patients with drug-induced skin reactions: maculopapular eruptions (ME), erythema multiforme (EM), erythema multiforme coexisting with erythema nodosum (EMN), drug-induced urticaria (DU), hyperergic vasculitis (HV), Stevens-Johnsson syndrome and toxic epidermal necrolysis (SJS/TEN). The activity of both proteins were measured using immunoenzymatic ELISA method: a) in the acute stage of disease, before treatment was administered, and b) after clearing of skin symptoms, after treatment. In the acute stage of disease highly elevated mean concentrations of IL-2 and sIL-2R in all 6 groups of patients were found (p<0.001) in comparison with the control. After clearing of skin lesions IL-2 mean concentrations were lowered to the level not different significantly from the control (p>0.05), but slL-2R mean plasma concentrations, despite the deep decrease, were still highly significantly elevated in comparison with control values (p<0.001).

Acute Disease↗

[Light-induced postherpetic erythema exsudativum multiforme].

A 37-year-old man had erythema multiforme over a period of 20 years following recurrent herpes simplex infections. On the basis of the results of provocative UV-A tests as well as histological findings a diagnosis of photosensitive erythema multiforme was made. The erythema multiforme exsudativum like variant of polymorphous light eruption is discussed in the differential diagnosis.

Adult↗