Case of keratosis lichenoides chronica with atypical sarcoidal granulomatous inflammation

Keratosis lichenoides chronica (KLC) is a rare chronic disease characterized by violaceous, papular and nodular lesions typically arranged in a linear and reticulate pattern. The etiology of KLC is unknown, but it may be associated with internal diseases such as hypothyroidism, glomerulonephritis and lymphoproliferative disorders. Herein, we describe the case of 44-year-old male patient with characteristic lesions of KLC on the trunk and extremities, present for 12 years. The clinical diagnosis was proven by histopathological examination on several occasions. In the years following the diagnosis of KLC, he developed bilateral hilar and multiple mediastinal, cervical and inguinal lymphadenopathies and hepatosplenomegaly. In 2000, diffuse interstitial and then reticulonodular pulmonary infiltrates associated with fever, weight loss, malaise and subcutaneous nodules developed. Biopsies taken from peripheral and mediastinal lymph nodes, pulmonary parenchyma, pleural tissue, bone marrow and skin showed non-necrotizing granulomas, indicating a sarcoidal granulomatous reaction. Characteristic histopathological findings and the absence of atypical cells, clonality and a high proliferative index excluded lymphomas. Furthermore, detailed tests showed no evidence of an infectious granulomatous disease. As far as we know, this is the first reported case of KLC associated with a sarcoidal granulomatous reaction.     

慢性苔藓样角化病5例临床病理分析

报告5例慢性苔藓样角化病。男4例,女1例,年龄13～50岁。3例皮损为带状或网状分布的紫红色丘疹,2例皮损呈疣状。皮损多无自觉症状,但有1例患者皮损伴有剧烈的瘙痒。组织病理改变主要为表皮角化过度伴局灶性角化不全,局部颗粒层增厚,棘层不规则肥厚,表现为棘层肥厚和萎缩相间,基底细胞液化变性。真皮浅层炎性细胞呈带状浸润,主要为淋巴细胞和组织细胞,并可见少量嗜酸性粒细胞和浆细胞。4例患者的毛囊和真皮血管周围也有炎性细胞浸润。本病需与扁平苔藓和扁平苔藓样角化病等鉴别。     

慢性苔藓样角化病1例

报告１例罕见的慢性苔藓样角化病，患者女性７０岁。躯干、上肢发生角化性丘疹，部分皮疹排列成线状，伴口腔粘膜糜烂。组织病理变化为：表皮角化过度，棘层不规则肥厚与萎缩，基底细胞液化变笥。真此上部密集淋巴样细胞浸润。用皮质类固醇激素治疗无效，口服依曲替酯有效。讨论了此病的临床特点、组织病理、超微结构及治疗。     

Keratosis lichenoides chronica: marked response to calcipotriol ointment

Keratosis lichenoides chronica (KCL) is a rare dermatosis characterized by a distinctive seborrheic dermatitis-like facial eruption, together with violaceous, papular, and nodular lesions on the extremities and trunk, typically arranged in a linear and reticulate pattern. KLC is resistant to therapy, although spontaneous remission has been reported. We describe a 35-year-old woman with KLC who had the typical features of widespread violaceous, reticulate, and striae-like eruptions with a prominent keratotic component over a nine-year period and who responded well to treatment with calcipotriol ointment. The immunohistochemical profiles are presented in addition to typical histopathologic features.     

Bullous lichen planus developed on erythema ab igne

Lichen planus developed on erythema ab igne which was induced by repeated exposures to a stove. The clinical and histopathological features of the eruption were an admixture of typical lichen planus and erythema ab igne. Subepidermal bullae were also found on the lichen planus. Direct immunofluorescence tests revealed deposits of fibrin in the basement membrane zone and IgM in the colloid bodies. Indirect immunofluorescence studies detected no circulating antibodies against cutaneous structures in the patient's serum. This study indicates that repeated thermal exposure over a prolonged period may produce lichen planus lesions.     

Plattenepithelkarzinome bei Erythema ab igne

Erythema ab igne (EAI) is a red-brown net-like hyperpigmentation due to repetitive exposure to submaximal heat over a long period of time. This rash is typically seen on the anterior lower legs of people who sit in front of open fireplaces or coal stoves and on the trunk of patients with chronic disorders who seek pain relief by long-term use of hot-water bottles or heating pads. EAI can not only be a diagnostic sign for underlying internal diseases including malignant tumors but can also give rise to cutaneous malignancies. We report on a 75-year-old female patient who developed several thermal keratoses and squamous cell carcinomas at the site of erythema ab igne caused by chronic heat exposure over several years.     

Cutaneous reactive angiomatosis associated with erythema ab igne

We present the case of a 49‐year‐old man with an 18‐month history of an intermittently pruritic red‐brown reticulate eruption of the medial legs, with occasional blistering. There were areas of scale, crust and superficial erosions. The patient worked long overnight shifts standing in front of hot machinery wearing shorts, and spent time sitting in front of a heater at home. A clinical diagnosis of erythema ab igne (EAI) was made. This settled with heater avoidance and protection of the legs with long trousers, as well as a topical corticosteroid. Histology showed features of cutaneous reactive angiomatosis, a rare condition with various associations but only previously reported once in association with EAI.     

Squamous cell carcinoma developing in thermal keratosis

Two cases of squamous cell carcinoma (SCC), both of them developed from thermal keratosis arising in areas of erythema ab igne (EAI) are reported. The lesions were located on the legs of two elderly Japanese women who had histories of chronic exposure to heat from a “kotatsu”, a type of leg-warmer widely used in Japan. In one case, metastasis to regional lymph nodes occurred. It is emphasized that keratotic lesions arising in an area of EAI should be excised or biopsied because of the possibility of metastasizing SCC developing from them.     

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.     

Keratosis lichenoides chronica. Bath PUVA therapy

We describe the case of a 51-year-old male patient with characteristic lesions of keratosis lichenoides chronica confined to the back of his hands and feet. The lichenoid papules, linear hyperkeratotic ridges and erythematosquamous plaques appeared first in early childhood and recurred after a short episode of spontaneous remission. They didn't respond to various topical treatment modalities over the years. After a local PUVA therapy all lesions disappeared with no recurrence for over two years now. Our case report indicates a new promising indication for bath-PUVA-therapy.     

Hot pop brown spot: erythema Ab igne induced by heated popcorn

Erythema ab igne is caused by chronic heat exposure and presents with reticulated pigmentation. Although various causes of erythema ab igne have been reported, in the United States, its incidence has been declining due to the advent of central heating. When seen, it is usually in the setting of local applications of a heated source, such as a hot water bottle, to treat muscular or arthritic pains. We report a novel cause of erythema ab igne occurring in a patient with chronic arthritic pains. This patient applied popcorn kernels, heated in a microwave, to his right wrist and knee for 30 minutes at a time for over four months.     

The use of synchrotron infrared microspectroscopy in the assessment of cutaneous T‐cell lymphoma vs. pityriasis lichenoides chronica

Background: The diagnosis of cutaneous lymphomas remains a challenge for both the clinician and dermatopathologist. Objectives: To differentiate between frank malignant and premalignant lymphocytes within the skin. Methods: This study was performed on 20 patients with a mean age of 50 years. They were divided into two groups: mycosis fungoides (MF) (stage IA, IB and IIA) and pityriasis lichenoides chronica (PLC). Immunophenotyping using antibodies CD3, CD4, CD8, CD20 and CD30 was performed. Synchrotron Fourier transform infrared microspectroscopy (S‐FTIRM) was performed on cell nuclei to assess chemical differences between MF and PLC cases as a potential complementary screening tool. Dermal spectra of both MF and PLC were compared using principal components analysis (PCA) of the S‐FTIRM data. Results: All PLC spectra was clustered together. However, the MF spectra formed two clusters, one that grouped with the PLC and the other grouped separately. Moreover, protein and nucleic acids showed highly significant differences between MF (IIA and IB), MF (IA) and PLC. Conclusions: The malignant transformation within lymphocytes was identifiable through the spectroscopic analysis of protein, RNA and DNA with S‐FTIRM, making it a promising tool for classifying the progression of cutaneous T‐cell lymphoma.     

Palmar Erythema Ab Igne without Detectable Type IV Collagen at the Basement Membrane Zone

A 42-year-old baker had hyperkeratotic lesions on reticular erythema of his palms and volar digits. Attachment between the epidermis and the dermis was loose at the hyperkeratotic sites, but no blisters had formed. After he stopped work for 2 months, the skin lesions disappeared, leaving a slight scar. Immunofluorescence studies showed a normal distribution of type VII collagen and laminin, but there was almost no fluorescence for type IV collagen in the basement membrane zone. Disappearance of cytoplasmic organelles and aggregated tonofibrils was evident under electron microscopy in the basal cells which appeared shrunken under light microscopy. The structures of lamina lucida, basal lamina and anchoring fibrils were disarranged. The loose dermo-epidermal attachment is probably due in part to this disorganized basement membrane structure lacking type IV collagen, which may have been digested by heat-activated matrix metalloproteinases. In addition, his work-related chronic heat exposure might have caused hyperproliferation of keratinocytes.