Sulzberger-Garbe exudative discoid and lichenoid chronic dermatosis ("Oid-Oid disease")--reality or fiction?]

In 1937, Sulzberger and Garbe singled out an exudative discoid and lichenoid chronic dermatosis characterized by the combination of various symptoms which by themselves are not specific from the heterogeneous eczema group. The report of a 7-year-old girl is used as a basis to describe the characteristics of the disease and to present the authors' own interpretation. Clinically, there were discoid and lichenoid lesions with severe pruritus. Blood examination revealed eosinophilia. Histopathological examination of skin lesions showed psoriasiform, spongiotic, lichenoid dermatitis. A therapeutic regimen of oral corticosteroids led to complete regression of the skin changes. We feel that there are no clinical or histological findings to differentiate Sulzberger-Garbe disease definitely from extensive nummular eczema.     

Dermatoses of the Glans Penis in Korea: A 10-Year Single Center Experience

BackgroundA variety of infectious, inflammatory, and neoplastic dermatoses can develop on the glans penis, and definitive diagnosis in such cases may be difficult owing to their non-specific symptoms and clinical appearance. Furthermore, data on dermatoses of the glans penis in Korea are limited.ObjectiveIn the present study, we aimed to determine the prevalence of dermatoses of the glans penis in Korea and provide clinical data to assist in making an accurate diagnosis.MethodsWe retrospectively reviewed the medical records, clinical photographs, and histologic slides of 65 patients with dermatoses of the glans penis that visited the Pusan National University Hospital between January 2004 and August 2013.ResultsTwenty-six types of dermatoses were identified: inflammatory dermatosis was the most common (38/65, 58.5%), followed by infectious (13/65, 20.0%), neoplastic (10/65, 15.4%), and other dermatoses (4/65, 6.2%). The most common dermatosis of the glans penis was seborrheic dermatitis, followed by lichen planus, herpes progenitalis, condyloma accuminatum, erythroplasia of Queyrat, Zoon''s balanitis, and psoriasis. In the topographic analysis, the most common type of dermatosis was dermatoses that localized to the glans penis (39/65, 60.0%), followed by dermatoses involving the extra-genitalia and glans penis (22/65, 33.9%), and the genitalia (glans penis plus other genital areas) (4/65, 6.2%).ConclusionThis study shows the usefulness of a topographic approach in the diagnosis of dermatoses of the glans penis in Korea. The findings could be used as baseline data for establishing an accurate diagnosis in Koreans.     

Distinctive exudative discoid and lichenoid chronic dermatosis (Sulzberger and Garbe) re-examined–1978

The dermatosis described over 40 years ago by Sulzberger and Garbe is still being seen and is still without known cause, aetiology or pathogenetic mechanism. Its kaleidoscopic changes in clinical appearance cause it to mimic many other dermatoses. But in typical cases it can be recognized by an assemblage of clinical and histopathological characteristics and distinguished from other known dermatoses. Modern techniques may shed light upon its causal mechanism and either establish it as an entity or place it in nosological perspective.     

Lichen aureus: A localized persistent form of pigmented purpuric dermatitis

Localized persistent pigmented macules and plaques due to a chronic form of pigmented purpuric dermatitis are described in forty-two patients. The designation 'lichen aureus' for this eruption can be justified because of the striking yellowish or bronze-like colour assumed by many of the lesions. The lower legs were the commonest sites but other body regions can be affected also. Histologically lichen aureus differs from other pigmented purpuric dermatoses in the density of the lichenoid tissue reaction and the marked accumulation of pigment-containing macrophages. Recently developed endothelial cell markers have been studied in selected cases.     

Neutrophilic Dermatoses

Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis are neutrophilic dermatoses – conditions that have an inflammatory infiltrate consisting of mature polymorphonuclear leukocytes. The neutrophils are usually located within the dermis in Sweet syndrome and pyoderma gangrenosum; however, in subcorneal pustular dermatosis, they are found in the upper layers of the epidermis. Sweet syndrome, also referred to as acute febrile neutrophilic dermatosis, is characterized by pyrexia, elevated neutrophil count, painful erythematous cutaneous lesions that have an infiltrate of mature neutrophils typically located in the upper dermis, and prompt clinical improvement following the initiation of systemic corticosteroid therapy. Classical, malignancy-associated, and drug-induced variants of Sweet syndrome exist. Pyoderma gangrenosum is characterized by painful, enlarging necrotic ulcers with bluish undermined borders surrounded by advancing zones of erythema; its clinical variants include: ulcerative or classic, pustular, bullous or atypical, vegetative, peristomal, and drug-induced. Subcorneal pustular dermatosis is an uncommon relapsing symmetric pustular eruption that involves flexural and intertriginous areas; it can be idiopathic or associated with cancer, infections, medications, and systemic diseases. Since Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis share not only the same inflammatory cell but also similar associated systemic diseases, it is not surprising that the concurrent or sequential development of these neutrophilic dermatoses has been observed in the same individual. Also, it is not unexpected that several of the effective therapeutic interventions – including systemic drugs, topical agents, and other treatment modalities – for the management of these dermatoses are the same. The treatment of choice for Sweet syndrome and idiopathic pyoderma gangrenosum is systemic corticosteroids; however, for subcorneal pustular dermatosis, dapsone is the drug of choice. Yet, tumor necrosis factor-α antagonists are becoming the preferred choice when pyoderma gangrenosum is accompanied by inflammatory bowel disease or rheumatoid arthritis. Potassium iodide and colchicine are alternative first-line therapies for Sweet syndrome and indomethacin (indometacin), clofazimine, cyclosporine (ciclosporin), and dapsone are second-line treatments. Cyclosporine is effective in the acute management of pyoderma gangrenosum; however, when tapering the drug, additional systemic agents are necessary for maintaining the clinical response. In some patients with subcorneal pustular dermatosis, systemic corticosteroids may be effective; yet, systemic retinoids (such as etretinate and acitretin) have effectively been used for treating this neutrophilic dermatosis – either as monotherapy or in combination with dapsone or as a component of phototherapy with psoralen andUVAradiation. Topical agents can have an adjuvant role in themanagement of these neutrophilic dermatoses; however, high-potency topical corticosteroids may successfully treat localized manifestations of Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis. Intralesional corticosteroid therapy for patients with Sweet syndrome and pyoderma gangrenosum, hyperbaric oxygen and plasmapheresis for patients with pyoderma grangrenosum, and phototherapy for patients with subcorneal pustular dermatosis are other modalities that have been used effectively for treating individuals with these neutrophilic dermatoses.     

From acute febrile neutrophilic dermatosis to neutrophilic disease: forty years of clinical research

In 1964, Sweet described an acute febrile neutrophilic dermatosis. It is now widely accepted that Sweet's syndrome belongs to a group of associated neutrophilic dermatoses. Although clinically dissimilar, Sweet's syndrome, pyoderma gangrenosum, subcorneal pustular dermatosis, erythema elevatum diutinum, and a few other conditions can be considered a part of this same pathologic spectrum of inflammatory disorders because of (1) the existence of transitional and overlap forms; (2) the similar histopathologic feature of an infiltrate by normal polymorphonuclear leukocytes; (3) the possible occurrence of extracutaneous neutrophilic infiltrates, defining the neutrophilic disease; and (4) the frequent association with systemic diseases. According to the localization of the neutrophilic infiltrate, we describe neutrophilic dermatoses en plaques (dermal), superficial (epidermal), and deep (dermal and hypodermal). Almost every organ of the body may be involved by a neutrophilic aseptic inflammation. The main systemic diseases associated with neutrophlic dermatoses are hematologic, gastrointestinal, and rheumatologic diseases. Although the pathophysiology of these conditions is still poorly understood, treatment with systemic anti-inflammatory agents is usually efficacious.     

Non-bullous neutrophilic dermatosis: an uncommon dermatologic manifestation in patients with lupus erythematosus

BACKGROUND: Rare cases of a non-bullous neutrophilic dermatosis occurring in patients with lupus erythematosus (LE) have been reported, often as the presenting manifestation of the disease. METHODS: We reviewed hematoxylin and eosin-stained slides and obtained clinical information from four additional patients, two of whom had no prior history of LE. RESULTS: All patients were female, aged 16-59 (mean age 37). Clinically, the skin lesions were characterized by widely distributed pruritic papules and plaques. Three patients presented with systemic symptoms, including fever, arthritis and malaise. Histopathologic examination in all cases showed a superficial perivascular and interstitial neutrophilic infiltrate with leukocytoclasis. There was no evidence of vasculitis. Mild focal vacuolar change was a subtle feature seen only in the biopsies of the two patients with a prior history of LE. CONCLUSIONS: It is important to consider LE in the differential diagnosis of non-bullous neutrophilic dermatoses.     

Pigmented purpuric eruptions

The pigmented purpuras comprise a group of dermatoses that are most commonly located on the lower extremities and share related clinical and histopathologic appearances. Included is a discussion of the following entities: progressive pigmentary disease of the skin (Schamberg's disease), purpura annularis telangiectodes (Majocchi's disease), pigmented purpuric lichenoid dermatosis (Gougerot and Blum's disease), and lichen aureus.     

Melanocytic and pseudomelanocytic nests coexist in interface dermatitis from head-neck sun-exposed skin: A report of three cases

Discrete junctional cellular aggregates (“nests”), partially staining with melanocytic markers, are described in lichenoid tissue reaction, mainly from chronically sun-exposed skin. The concomitant epidermal flattening and papillary dermal fibrosis with melanophages, may raise the differential diagnosis to that of a regressing melanoma. We describe three cases of interface dermatitis of the head/neck area with clinicopathological features of melanotic discoid lupus erythematosus. These cases showed junctional aggregates, a few composed of inflammatory cells and colloid bodies (“pseudomelanocytic nests”), while others composed of S100- but MART-1+, MITF+, and SOX-10+ cells (“true melanocytic nests”); negativity of the melanocytic component for PRAME was a clue to benignity. True junctional melanocytic nesting may be induced by lichenoid dermatoses on chronically sun-damaged skin. The presence of colloid bodies and of the double negativity for S100 (within nests) and PRAME (both within nests and single melanocytes), together with clinicopathological correlation, avoids misdiagnosis.     

Annular lichenoid dermatitis of youth – a further case in a 12‐year‐old girl

Annular lichenoid dermatitis of youth was first described by Annessi et al. in 2003. Clinical criteria are persistent erythematous macules and annular lesions with a red‐brown edge and a central hypopigmentation usually found on the flanks and groins of children and adolescents. Histologically, the disease is characterized by a lichenoid interface dermatitis with necrotic keratinocytes at the tip of the rete ridges. In our case a 12‐year old girl developed annular red‐brown macules with papules at the borders in an inframammary location. The histology of the lesion's border showed a lichenoid lymphocytic infiltrate with apoptotic keratinocytes at the tip of rete ridges. The lesions cleared with 0.03% tacrolimus ointment. Annular lichenoid dermatitis of youth is probably a new entity in the group of lichenoid dermatoses.     

Keratosis lichenoides chronica and eruptive keratoacanthoma-like lesions in a patient with multiple myeloma

We describe a 72-year-old woman with a 13-year history of a lichenoid dermatitis, who developed multiple, papular keratoacanthoma (KA)-like lesions and few crater-like nodules on the extremities over a period of 6 months before our observation. Her medical history also recorded multiple myeloma diagnosed a few years before. The long-standing dermatosis was diagnosed, clinically, as keratosis lichenoides chronica (KLC), although, histologically, a lichenoid tissue reaction pattern was not evident. On the other hand, histology from papular and nodular lesions of recent onset was consistent with a possible early phase of KA and spinocellular carcinoma, respectively. Oral acitretin induced regression of KA-like lesions and improvement of KLC but had no effects on crater-like nodules, which required surgical excision. KLC is a chronic disorder of keratinization characterized by lichenoid hyperkeratotic papules arranged in a linear pattern, erythematosquamous plaques and seborrhoea-like dermatitis. We emphasize in our case the association between KLC and multiple possible KAs, never previously reported, and speculate that these two rare conditions may represent here a 'continuum' from a pathogenetic point of view.     

Artificial penile nodules and secondary syphilis.

A patient with artificial penile nodules and ulcerative plaques on the penis is described. Clinical course, histology and serological examination revealed a diagnosis of secondary syphilis. The differential diagnosis of the ulcerative genital lesions in the presence of artificial penile nodules is discussed.     

大连地区化妆品皮肤病890例分析

目的 探讨化妆品皮肤病的临床特点及其相关化妆品类型。方法 对890例化妆品皮肤病进行分析,对可疑化妆品行斑贴试验。结果 临床表现主要为接触性皮炎占90％,依次为色素沉着占3.93％、痤疮样损害占3.37%、光敏性皮炎占2.24％、接触性荨麻疹占0.56%。可疑致病化妆品种类2019种,以护肤品为主,占92%。890例患者中斑贴试验阳性者346例,阳性率39％。结论 化妆品皮肤病的发生原因是多方面的,应加强化妆品知识宣传,建立一个完整的化妆品皮肤病监测系统。     

Dermatosen-imitierende kutane Arzneimittelreaktionen

Background

Cutaneous adverse drug reactions are frequent and present with heterogenous clinical manifestations.

Methods and results

Increasingly, case reports describe drug reactions which mimic dermatoses, although exact data on prevalence are missing. Psoriasiform, lichenoid and pityriasiform exanthems are most frequent. The differentiation of these variants from the respective authentic dermatoses is difficult and only a few clinical and histological criteria are helpful. Other dermatoses like lupus erythematosus or bullous autoimmune dermatoses (pemphigus vulgaris, bullous pemphigoid) can be induced by drugs as well. These drug-triggered variations are classified as distinct subclasses of the respective dermatosis.

Conclusions

Exact history and evaluation of the clinical course are essential for the diagnosis of drug reactions mimicking dermatoses and present an important challenge for the clinician. A clear-cut differentiation between the genuine dermatosis and its drug-driven simulator is not always possible.     

Circumcision and genital dermatoses

CONTEXT: It is well recognized that the presence of a foreskin predisposes to penile carcinoma and sexually transmitted infections. We have investigated the relationship between the presence or absence of the foreskin and penile dermatoses. OBJECTIVE: To determine whether there is an association between circumcision and penile dermatoses. DESIGN: A retrospective case control study of patients attending the department of dermatology with genital skin conditions. SUBJECTS: The study population consisted of 357 male patients referred for diagnosis and management of genital skin disease. The control population consisted of 305 male patients without genital skin disease attending the general dermatology clinics over a 4-month period. MAIN OUTCOME MEASURES: The relationship between circumcision and the presence or absence of skin disease involving the penis was investigated. The rate of circumcision in the general male dermatology population was determined. RESULTS: The most common diagnoses were psoriasis (n = 94), penile infections (n = 58), lichen sclerosus (n = 52), lichen planus (n = 39), seborrheic dermatitis (n = 29), and Zoon balanitis (n = 27). Less common diagnoses included squamous cell carcinoma (n = 4), bowenoid papulosis (n = 3), and Bowen disease (n = 3). The age-adjusted odds ratio for all penile skin diseases associated with presence of the foreskin was 3.24 (95% confidence interval, 2.26-4.64). All patients with Zoon balanitis, bowenoid papulosis, and nonspecific balanoposthitis were uncircumcised. Lichen sclerosus was diagnosed in only 1 circumcised patient. Most patients with psoriasis, lichen planus, and seborrheic eczema (72%, 69%, and 72%, respectively) were uncircumcised at presentation. The majority of men with penile infections (84%) were uncircumcised. CONCLUSIONS: Most cases of inflammatory dermatoses were diagnosed in uncircumcised men, suggesting that circumcision protects against inflammatory dermatoses. The presence of the foreskin may promote inflammation by a k?ebnerization phenomenon, or the presence of infectious agents, as yet unidentified, may induce inflammation. The data suggest that circumcision prevents or protects against common infective penile dermatoses.     

Annular lichenoid dermatitis of youth

BACKGROUND: Lichenoid dermatoses are composed of a wide spectrum of disorders with a common histopathologic interface pattern but diverse causes and pathophysiology. OBJECTIVE: We describe a series of young patients with a peculiar annular lichenoid dermatitis, the clinical appearance of which initially suggested diagnoses of morphea, mycosis fungoides, or annular erythema. RESULTS: The study involved 23 patients (median age 10 years; age range 5-22 years). Lesions consisted of persistent asymptomatic erythematous macules and round annular patches with a red-brownish border and central hypopigmentation, mostly distributed on the groin and flanks. Histology revealed a peculiar lichenoid dermatitis with massive necrosis/apoptosis of the keratinocytes limited to the tips of rete ridges, in the absence of dermal sclerosis and epidermotropism of atypical lymphocytes. The infiltrate was composed mainly of memory CD4(+) CD30(-) T cells with few B cells and macrophages. Analysis of T-cell receptor-gamma-chain gene rearrangement in skin biopsy specimens revealed polyclonality in all the 15 cases studied. Topical and systemic corticosteroids or phototherapy were effective in most patients with relapse after treatment withdrawal. CONCLUSIONS: We suggest that this is a distinctive inflammatory condition, and we propose to term it "annular lichenoid dermatitis of youth."     

Parakeratosis variegata: a possible role of environmental hazards?

We report 2 cases of parakeratosis variegata (PV) evolving from lesions beginning with characteristics of ashy dermatosis. Both patients presented with a reticulated, poikilodermatous and hyperpigmented eruption with bizarre coalescent lichenoid papules. Histology showed lichenoid epidermotropic infiltrates, more pronounced in case No. 1, consistent with early malignancy. The course was chronic: after more than 10 years, systemic symptoms were not present. In patient No. 1, a monoclonal T-cell population was detected 12 years after the onset of the disease. Both patients had close contact with fertilizers and insecticides. In patient No. 2, the lesions spontaneously regressed within 3 years after cessation of exposure. PV may be a prelymphomatous stage of mycosis fungoides or some closely related cutaneous T-cell lymphoma and does not always evolve into overt malignancy. Gene rearrangement detection techniques may be helpful in predicting the course of the disease.     

Disseminated superficial actinic porokeratosis with hyperkeratotic lesions

A patient with disseminated superficial actinic porokeratosis (DSAP) presented with unusual exudative hyperkeratotic plaques in addition to lesions more typical of the disease. The plaques were unresponsive to various systemic antibiotics, but responded dramatically to systemic corticosteroid therapy.     

Erythema elevatum et diutinum as a systemic disease

Erythema elevatum et diutinum (EED) is a rare, chronic dermatosis. It has been associated with extracutaneous findings, including arthralgias, scleritis, panuveitis, peripheral ulcerative keratitis, oral and penile ulcers, and neuropathy. Additionally, EED is connected with various systemic diseases, including HIV, IgA paraproteinemia, myelomas, neutrophilic dermatoses, and inflammatory bowel diseases. The presence of such extracutaneous manifestations in EED patients suggests that EED may be a multiorgan entity. Extracutaneous manifestations in EED may involve deposition of circulating immune complexes; thus, patients with EED should be evaluated for systemic manifestations to ensure targeted management.     

Keratosis lichenoides chronica. Report of a case associated with B-cell lymphoma and leg panniculitis

Keratosis lichenoides chronica (KLC) is a rare chronic dermatosis characterized by lichenoid hyperkeratotic papules arranged in a linear and reticular pattern, and seborrheic-dermatitis-like lesions on the face. Less frequently, palmoplantar keratoderma, nail dystrophies, mucosal as well as eye lesions are present. KLC affects adults and very few cases have been reported in childhood. Although infrequently, KLC has been associated with systemic diseases, including chronic infectious diseases, kidney disorders and lymphoma. Here we report the case of an adult KLC patient with skin, nail and mucosal involvement, and onset in the first year of life who developed a leg panniculitis and a mantle cell lymphoma. Following chemotherapy for the lymphoma, panniculitis resolved completely, and skin and mucosal KLC lesions ameliorated.