Papular and nodular mucinosis as a sign of lupus erythematosus

We describe 3 cases of papular and nodular mucinosis (PNM), a clinically distinctive cutaneous mucinosis associated with lupus erythematosus (LE), which has received little attention in the dermatologic literature. Histopathology shows deposits of mucin in the dermis without microscopic features of LE, while immunofluorescent studies disclose linear or granular deposits of IgG, IgM and C3 at the dermoepidermal junction. In about 80% of the 14 cases described in the literature, PMN has been associated with systemic LE with prevalent joint and kidney involvement. The possible prognostic significance of this singular dermatosis is discussed.     

Lupus erythematosus and papules. 4 cases]

Papular skin eruptions are uncommon in lupus erythematosus (LE), and their occurrence may suggest several diagnoses. We report four cases of papular eruptions in LE patients. Two of these patients had purely lupoid papules on acute LE in one and on chronic LE in the other. In the remaining two cases the papules were formed by mucinous deposits that were either secondary to LE or belonged to an associated primary mucinosis. These four cases prompted us to discuss the significance of papular eruptions in lupus erythematosus. LE-specific papular manifestations have a purely lupoid histological and immunopathological substratum. In systemic LE, the frequency of these manifestations varies from one series to another, but they seem to rank fourth after vespertilio, alopecia and photosensitivity. The papules vary in size and number, and they usually complicate a severe and active systemic LE. In chronic LE, a papulo-nodular eruption may be observed, but this is even rarer. Papules occurring in the course of LE may suggest a diagnosis of mucinosis, this disease being either secondary to, or associated with LE. Secondary mucinosis is intricated with LE-specific histological abnormalities. It appears as a symptomatic alcianophilic deposit induced by cytological alterations in some dysimmune collagen diseases, such as dermatomyositis or LE. In the literature, three cases of mucinosis secondary to LE are well documented, and to these we add a fourth case in which the papules contained lupoid lesions and a variable amount of dermal mucinous deposit. The literature has also yielded sixteen cases of primary papular mucinosis associated with systemic LE (12 cases) or chronic LE (4 cases); we add to these a case of systemic LE in which the papular eruptions varied in course, density and size of the papules. As in isolated papular mucinosis, histology regularly shows a copious mucinous deposit. When present, the LE-specific skin manifestations are clearly distinct from those of mucinosis (except for the above-mentioned cases of papular lupus). The course of papular mucinosis usually runs parallel to that of LE which is characterized by its severity and its articular, haematological and renal manifestations. Analysis of the literature also provides various anatomico-clinical elements which help in differentiating between isolated papular mucinosis and primary papular mucinosis associated with LE. In the majority of cases treatment relies on corticosteroid therapy or synthetic antimalarial agents, but the results are irregular since LE seems to be more resistant to treatment than mucinosis.(ABSTRACT TRUNCATED AT 400 WORDS)     

Adult Still's disease

Adult Still's disease (ASD) is a rare disorder of unknown aetiology, characterized by an evanescent, erythematous, maculopapular rash, fever, arthralgia, and a variety of systemic features. We report a case which illustrates the typical features of ASD, and manifests the hitherto unreported complication of diffuse cutaneous mucinosis.     

Papulonodular mucinosis associated with subacute cutaneous lupus erythematosus

Mucin deposition is a common histopathologic finding in lupus erythematosus (LE) but is rarely present in sufficient quantity to produce clinically apparent skin lesions. Until now, fewer than 40 cases of papulonodular mucinosis associated with LE have been reported in the literature, and it was associated with either systemic LE or discoid LE. For the first time, to our knowledge, we describe 2 patients with subacute cutaneous LE who presented with papulonodular mucinosis as a major clinical manifestation of their disease.     

Secondary cutaneous mucinosis with systemic lupus erythematosus. A case presentation and review of the literature.

A patient had papular and nodular cutaneous deposits of mucin and cutaneous and systemic manifestations of lupus erythematosus (LE). Since many of the mucinous deposits occurred at sites that were clinically free of skin lesions of LE, we considered initially that the patient had both LE and papular mucinosis. However, after a review of the English literature and further study of the patient, it seemed more likely that the papular and nodular deposits of mucin were secondary to LE and not a previously unreported simultaneous occurrence of the two diseases in the same patient. To our knowledge, this is the third case report of a patient with papular and nodular cutaneous mucinosis secondary to LE. In addition to the case report, this article is concerned with a discussion of cutaneous mucinosis in LE and other "collagen vascular" diseases.     

Papulonodular mucinosis indicating systemic lupus erythematosus

We describe an 18‐year‐old girl with systemic lupus erythematosus (SLE) who had cutaneous papulonodular mucinosis (PNM) as the first sign of SLE. She presented with multiple flesh‐coloured papules on the face, abdomen and limbs. Histological examination of a biopsy taken from a papule showed diffuse deposition of mucin throughout the dermis, and direct immunofluorescence of lesional skin showed a dermoepidermal junction band composed of IgG, IgM and C3, consistent with PNM. Investigations showed that that the patient had leucopenia, positive antinuclear and anti‐double‐stranded DNA antibodies and lupus nephritis. PMN can be an unusual clinical presentation of SLE.     

Seltene kutane Manifestationsformen des Lupus erythematodes Eine klinische Übersicht

Lupus erythematosus (LE) is a disease with a wide spectrum of cutaneous and systemic manifestations and has been the subject of many studies over several decades. Clinical features of patients with LE show a great variation, and for this reason it is difficult to develop a unifying concept of this disease. Consequently, this has led to the identification of subsets which have been defined by constellations of clinical and photobiological features, histological changes as well as laboratory abnormalities. Besides the characteristic classical forms such as systemic LE (SLE), subacute cutaneous LE (SCLE), and discoid LE (DLE), there are uncommon variants of LE which often lead to diagnostic difficulties. Bullous LE (BLE) and urticarial vasculitis are listed as characteristic but non-specific manifestations of systemic LE. LE tumidus (LET), LE hypertrophic/verrucous (LEHV), chilblain LE, and LE profundus (LEP) are uncommon subtypes of chronic cutaneous LE. Annular erythema and papulonodular mucinosis are further uncommon cutaneous manifestations of LE. This clinical review summarizes the typical features of the uncommon forms of LE in order to improve clinical diagnostic precision and to achieve a better differentiation of the subtypes.     

Cutaneous lupus erythematosus: a review

This article will review and update information about the pathogenesis, clinical presentation, diagnosis, and treatment of cutaneous lupus erythematosus. Lupus erythematosus (LE) can present as a skin eruption, with or without systemic disease. Cutaneous LE is subdivided into chronic cutaneous LE, subacute cutaneous LE and acute LE. The prevalence of systemic lupus erythematosus (SLE) is 17-48/100,000 population worldwide. Skin disease is one of the most frequent clinical complaints of patients suffering from SLE. It has been found to occur in up to 70% of patients during the course of the disease. The most frequent mucocutaneous manifestations of SLE are malar rash (40%), alopecia (24%), and oral ulcers (19%). It has been suggested that risk factors that are more likely to signal transition of cutaneous into systemic LE are high ANA titers (> 1:320) and the presence of arthralgias. CLE patients who exhibit these symptoms should be monitored closely, since they may be at increased risk to develop SLE.     

Acral persistent papular mucinosis

Cutaneous mucinoses, also called lichen myxoedematosus, are a group of diseases characterized by mucin deposit on the skin. They may be associated to systemic diseases (scleromyxedema) or be primary ones (papular mucinosis, localized lichen myxoedematous), although they share common findings (intermediate or atypical forms) in some cases. Acral persistent papular mucinosis (APPM) is a type of papular mucinosis that is located exclusively on the back of the hands and in the distal zone of the forearms and is not associated to any systemic disease. We present the case of a 52-year-old healthy woman who had skin lesions on the back of her hands and whose histological study confirmed an APPM.     

Clinical course and prognosis of cutaneous lupus erythematosus

Classical variants of specific cutaneous LE lesions are chronic discoid LE (CDLE) and subacute cutaneous LE (SCLE). CDLE and SCLE may appear at any age; however, the most common age of onset is between 20 and 40 years, with a female predominance of 3:1 in CDLE and 3-6:1 in SCLE. Nonspecific LE skin lesions such as generalized or acrolocalized vasculitis (4-30%), livedo reticularis (22-35%), and alopecia (38-78%) are frequently seen in patients with cutaneous LE. Other typical cutaneous LE subsets such as LE profundus/panniculitis, LE tumidus, urticaria vasculitis, hypertrophic LE, and bullous LE are rather rare variants. Butterfly rash and/or macular exanthema are characteristic skin lesions of systemic lupus erythematosus (SLE) rarely found in patients with cutaneous LE.     

Hyperpigmented acral papular mucinosis, systemic lupus erythematosus and universal alopecia

A 42-year-old man presented with systemic lupus erythematosus, universal alopecia and non-pruritic hyperpigmented papular mucinosis. The latter was most evident on acral areas. In hyperpigmented areas of the face the immunofluorescence showed deposits as in LE and with alcian blue and colloidal iron an abundance of mucin was demonstrated in the dermis. A lesion on the back showed only papular mucinosis. Fifteen cases of LE and papular mucinosis reported in the literature are reviewed. Our patient differs with respect to the marked pigmentation of his lesions, their localization and the association with universal alopecia.     

The prevalence of antinuclear antibodies in patients with apparent polymorphic light eruption

Polymorphic light eruption (PLE) is a very common photosensitive disorder, the most important differential diagnosis of which is lupus erythematosus (LE). One-hundred and forty-two patients with PLE were screened for circulating antinuclear (ANA), Ro and La antibodies over a 2-year period. Results were negative in 66 patients. Sixty-two patients had low-titre ANA of various patterns, ranging from trace to 1/80 without evidence of LE although one later developed subacute cutaneous LE. Fourteen had more significant findings, six with ANA ranging from 1/160 to 1/1280 but no anti-Ro antibodies, four with ANA ranging from 1/160 to 1/1280 and also with anti-Ro antibodies and four patients with anti-Ro antibodies but low-titre ANA, one of whom later developed discoid LE. Three of these 14 patients fulfilled the American Rheumatism Association criteria for the diagnosis of systemic LE, but it was not certain in any of the patients whether the PLE-like rash represented cutaneous LE or coincidental PLE. However the overall 10% incidence of definite or possible LE in patients with suspected PLE suggests that all PLE patients should be screened for LE.     

Topical tacrolimus as a therapeutic adjunct in patients with cutaneous lupus erythematosus. A report of three cases

The topical therapy of cutaneous lupus erythematosus (LE) consists mainly of corticosteroids which may lead to significant side effects when overused. We report the efficacy of topical tacrolimus as a therapeutic adjunct in 3 patients with cutaneous LE of the face. All patients, 1 with systemic LE and a malar rash, 1 with annular subacute cutaneous LE, the other with a papular variant of subacute cutaneous LE, experienced significant improvement following application of tacrolimus ointment. Treatment with topical tacrolimus was tolerated well without major side effects in all patients. The presented cases are in line with recent reports that topical tacrolimus may be effective in facial LE.     

The cutaneous pathology of lupus erythematosus: a review

The presentation of lupus erythematosus (LE) ranges from a skin rash unaccompanied by extracutaneous stigmata to a rapidly progressive lethal multiorgan disease. The diagnosis and subclassification is traditionally based on the correlation of serological and clinical findings. The latter include a photoinduced skin rash, arthralgia, arthritis, fever, Raynaud's phenomenon, anemia, leukopenia, serositis, nephritis and central nervous sysdtem disease. The conventional classification scheme includes systemic, subacute cutaneous and discoid LE. Recent advances in our understanding of the cutaneous histopathology which correlates with the traditional forms of LE, along with certain novel LE subtypes, are the focus of this review. In addition to the main subtypes of LE, we will discuss associated vasculopathic lesions and the contribution of immunofluorescence microscopy to the diagnosis of LE and related connective tissue disease syndromes. Consideration will be given to unusual variants of LE such as anti-Ro/SSA-positive systemic lupus erythematosus (SLE), bullous SLE, lymphomatoid LE, lupus erythematosus profundus, drug induced LE, linear cutaneous LE, chiblains LE and parvovirus B19-associated LE.     

Reticular erythematous mucinosis syndrome in a patient with polyarthritis

A patient with seronegative oligoarthritis who developed the reticular erythematous mucinosis (REM) syndrome is described. This syndrome is considered to be a dermatological entity unrelated to systemic disorders. Aggravation of the rash by exposure to sunlight and a good response to anti-malarial agents suggest a relationship with rheumatological disorders, e.g. rheumatoid arthritis and systemic lupus erythematosus. Dermatologists consulted by a patient with the REM syndrome should be aware of the possibility of an associated rheumatological disease.     

Lichen myxedematosus (papular mucinosis): new concepts and perspectives for an old disease

Lichen myxedematosus (LM) is an idiopathic cutaneous mucinosis; its classification dates back to 1953, when Montgomery and Underwood distinguished 4 types of LM. In the literature, the terms LM, papular mucinosis, and scleromyxedema often have been used indiscriminately as synonyms, but most reported cases of LM or papular mucinosis without indication of the subtype appear in fact to be cases of scleromyxedema. Actually, LM includes 2 clinicopathologic subsets: a generalized papular and sclerodermoid form (the only one which should be called scleromyxedema) with systemic, even lethal, manifestations and a localized form, which does not run a disabling course. The localized form is subdivided into 4 subtypes: (1) a discrete papular form involving any site; (2) acral persistent papular mucinosis involving only the extensor surface of the hands and wrists; (3) papular mucinosis of infancy, a pediatric variant of the discrete form or the acral form of persistent papular mucinosis; and (4) nodular form. A third group of atypical or intermediate forms, not meeting the criteria for either scleromyxedema or the localized form, includes cases of (1) scleromyxedema without monoclonal gammopathy, (2) localized forms with monoclonal gammopathy and/or systemic symptoms, (3) localized forms with mixed features of the subtypes, and (4) not well-specified cases.     

Lichen Myxedematosus

Lichen myxedematosus (papular mucinosis) is a slowly progressive mucinous disorder that is thought by many to have no internal organ involvement. We report a case of lichen myxedematosus (lichenoid plaque type) that is remarkable for the sudden and rapid nature of the infiltrative process with both cutaneous and possibly systemic manifestations. The cutaneous abnormalities were successfully treated with cyclophosphamide and intralesional steroids. The fact that our patient's systemic signs and symptoms also responded to therapy supports the concept of internal involvement in lichen myxedematosus.     

Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema

Lichen myxedematosus (LM) is an idiopathic cutaneous mucinosis; its classification dates back to 1953, when Montgomery and Underwood distinguished 4 types of LM: a generalized lichenoid eruption, later called scleromyxedema, a discrete papular form, a localized or generalized lichenoid plaque form, and an urticarial plaque form. In the literature, the terms LM, papular mucinosis, and scleromyxedema have been often used indiscriminately as synonyms, but most reported cases of LM or papular mucinosis without indication of the subtype appear in fact to be cases of scleromyxedema. On the basis of personal experience, the anatomoclinical manifestations of published cases of LM, papular mucinosis, and scleromyxedema are reviewed to distinguish clearly between a generalized form with systemic, even lethal, manifestations and a localized form, which does not run a disabling course. LM includes two clinicopathologic subsets: a generalized papular and sclerodermoid form (also called scleromyxedema) and a localized papular form. Diagnosis of scleromyxedema should fulfill the following criteria: (1) generalized papular and sclerodermoid eruption; (2) mucin deposition, fibroblast proliferation, and fibrosis; (3) monoclonal gammopathy; and (4) the absence of thyroid disease. The criteria for localized LM are as follows: (1) papular or nodular/plaque eruption; (2) mucin deposition with variable fibroblast proliferation; and (3) the absence of both monoclonal gammopathy and thyroid disease. The localized form is subdivided into 5 subtypes: (1) a discrete papular form involving any site; (2) acral persistent papular mucinosis involving only the extensor surface of the hands and wrists; (3) self-healing papular mucinosis, of a juvenile and an adult type; (4) papular mucinosis of infancy, a pediatric variant of the discrete form or of acral persistent papular mucinosis; and (5) nodular form. A third group of atypical or intermediate forms, not meeting the criteria for either scleromyxedema or the localized form, includes cases of (1) scleromyxedema without monoclonal gammopathy, (2) localized forms with monoclonal gammopathy and/or systemic symptoms, (3) localized forms with mixed features of the 5 subtypes, and (4) not well-specified cases.     

Cutaneous Manifestations of Childhood Systemic Lupus Erythematosus

Abstract: We assessed the mucocutaneous signs in 57 children with classical systemic lupus erythematosus seen during a 6 year period. The female:male ratio was 4.2:1. Ages ranged from 4 to 15 years (mean 11.9 years) at the time of diagnosis. Cutaneous manifestations (77%) were the second most common finding, next to renal involvement (84%). The skin changes noted were malar rash (74%), oral ulcer (46%), vasculitis (42%), photosensitivity (40%), alopecia (32%), and discoid lupus erythematosus (LE) (19%). All 11 discoid LE patients were girls. Periungual erythema, Raynaud's phenomenon, periungual gangrene, nail involvement, and subacute LE were rare. Antinuclear antibody reaction was positive in 93% and anti-dsDNA was positive in 46%. Eight patients died, six from severe infection and two from renal failure.