Linear IgA dermatosis in association with angioimmunoblastic T-cell lymphoma infiltrating the skin: A case report with literature review

Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive form of peripheral T-cell lymphoma, characterized by systemic symptoms, diffuse lymphadenopathy, hepatosplenomegaly and immunodysregulation. Half of AITL is associated with cutaneous symptoms, but only few cases with bullous eruption have been described. Association with a linear IgA dermatosis is extremely rare. Linear IgA dermatosis can be idiopathic, or linked with drug intake or neoplastic disorders. Some cases of linear IgA dermatosis presenting as toxic epidermal necrolysis (TEN) have been described, most of them being drug induced. We here present the case of a 72-year-old man recently diagnosed with AITL who developed a bullous eruption, presenting as TEN. Histopathology showed deep cutaneous involvement of the lymphoma with a sub-epidermal blistering and direct immunofluorescence revealed a heavy IgA linear deposit on the dermal-epidermal junction. A diagnosis of linear IgA dermatosis associated with cutaneous involvement of an angioimmunoblastic T-cell lymphoma was made. Chemotherapy and corticosteroids allowed cutaneous improvement but the patient died of his lymphoma shortly after.     

Relapsing linear acantholytic dermatosis in a four-year-old boy

Linear acantholytic dermatoses are a spectrum of cutaneous disorders that form a subset of linear dermatoses with distinct clinical features and histopathologically show acantholysis. The lesions may be zosteriform or follow the lines of Blaschko. This report describes a four-year-old boy who, on a follow up of two years, exhibited a relapsing acantholytic dermatosis along the lines of Blaschko. Histopathology of a representative lesion revealed epidermal acantholysis with multiple acantholytic keratinocytes with in the prickle cell layer and an absence of corp ronds and grains, consistent with features of Hailey-Hailey disease. This, to our knowledge, is the third case of relapsing linear acantholytic dermatosis reported.     

Erythematous plaque variant of transient acantholytic dermatosis

Transient acantholytic dermatosis (TAD) is a primary cutaneous disorder of unknown cause that was first described by Grover in 1970. The common eruptions of TAD are self-limited pruritic papules and papulovesicules distributed primarily on the trunk and extremities. We report a case of an erythematous plaque variant of this skin disorder.     

Focal acantholytic dyskeratosis occurring in pityriasis rubra pilaris

Focal acantholytic dyskeratosis (FAD) is a distinctive histologic pattern characterized by suprabasilar clefts surrounding dermal papillae (villi), acantholytic and dyskeratotic cells at all levels of the epidermis, hyperkeratosis, and parakeratosis. The features of FAD are typically seen in Darier's disease, warty dyskeratoma, and transient acantholytic dermatosis; they are also present in a variety of cutaneous neoplastic and nonneoplastic lesions. FAD, however, has not been previously described in lesions of inflammatory dermatoses. We report a case of FAD occurring in lesions of pityriasis rubra pilaris (PRP). To the best of our knowledge, this is the first reported case of this kind. We also review the pertinent literature.     

Transitory acantholytic dermatosis (Grover) in an HIV infected patient

We report on a patient with HIV infection and persistent generalized lymphadenopathy (stage III of HIV infection), who developed transitory acantholytic dermatosis (Grover's disease). Both the clinical picture and the histological features were typical for Grover's disease; the skin eruptions subsided within 6 months.     

暂时性棘层松解性皮病1例

报道1例暂时性棘层松解性皮病，以丘疹为主要临床表现，病理见局限性基底细胞层上裂缝，少量棘层松解细胞。     

Transient Acantholytic Dermatosis with Involvement of Oral Mucosa

Histologic, immunologic and electron microscopic studies were performed in a patient with transient acantholytic dermatosis which involved oral mucosa. Hitologic and electron microscopic findings were almost identical in both cutaneous and mucous membrane lesions, and these were similar to pemphigus vulgaris; suprabasilar separation with acantholytic cells. Desmosome-desmosome complexes were separated without disruption of cell membranes and cytoplasmic structures were well-preserved without dyskeratosis. Lamina lucida, however, was often separated in mucous membrane lesions, in contrast to the normal lamina lucida in cutaneous lesions of such cases or in pemphigus vulgaris. Direct and indirect immunofluorescence studies for pemphigus were repeatedly negative. This study shows that transient acantholytic dermatosis may involve mucous membrane and may resemble pemphigus vulgaris histologically and ultrastructurally except for the widened lamina lucida of the mucous membrane.     

Acantholytic dermatosis localized to genitalia and crural areas of male patients: A report of three cases

A series of 3 cases of acantholytic dermatosis localized to the male genitalia and perineal area with distinct clinical and histological features is presented. These patients did not have a family history of similar disorders and had no evidence of rash elsewhere. Clinically, the eruption was characterized by multiple discrete papules or macerated patches involving the genitalia and the perineum. Histologically, the acantholytic dermatosis had features that resembled both Hailey-Hailey disease and Darier's disease. Similar clinical and histological findings have been reported in females. To the best of our knowledge, this is the first report of these lesions described confined to the genital and perineal areas in male patients. We agree with other authors that this condition may represent a distinct acantholytic eruption in the perineal area that should be distinguished from other generalized blistering acantholytic dyskeratotic disorders dial may also involve the genitalia.     

Acantholytic dyskeratosis of the vulva presenting with clinical features of vulval lichen sclerosus—A possible rare collision entity

We present a challenging case of chronic, erosive, scarring dermatosis of the vulva with clinical features of long standing lichen sclerosus (LS), namely pallor and loss of vulval architecture, but with histopathology consistently showing features of an acantholytic process. The history and clinical features of this case do not resemble other acantholytic conditions such as pemphigus vulgaris, Hailey-Hailey disease, Darier disease, or the entities described as acantholytic dermatoses affecting the vulva. As far as we are aware, the combination of the clinical features and histopathologic findings in our case do not fit with any previously described condition and we propose that this is a rare entity of a collision of LS and an erosive acantholytic process occurring together.     

Transient intra-epidermal bullous dermatosis

Transient acantholytic dermatosis is an acquired, mildly pruritic, papulovesicular disease affecting the trunk and extremities. In this report a case of the bullous variant of this disease is described, with a brief review of the literature.     

Pyoderma gangrenosum associated with transient acantholytic dermatosis (pemphigus erythematosus-like) and paraproteinemia

A 69-year-old man with recurrent eruptions of pyoderma gangrenosum for 4 years is described. The patient also suffered from paraproteinemia (increased IgA with M-component) and transient acantholytic dermatosis resembling pemphigus erythematosus. He had no intestinal symptoms.     

Schnitzler's syndrome (urticaria and macroglobulinemia) associated with pseudoxanthoma elasticum.

Schnitzler's syndrome, first described in 1974, is defined by chronic non-pruritic urticaria, osteocondentation, and a monoclonal IgM dysproteinemia, but without criteria of lymphoproliferative disease. We report a patient with chronic urticaria and macroglobulinemia. In addition, he had double monoconal dysproteinemia IgM kappa (31.3 g/l) and IgA lambda, osteocondensation, and some cutaneous lesions of pseudoxanthoma elasticum. Only 20 cases of Schnitzler's syndrome have been reported hitherto. This is the first case associated with pseudoxanthoma elasticum, which was localized and discovered at the same time as Schnitzler's syndrome. We discuss the possible role of monoclonal immunoglobulin in the occurrence of localized elastorrexhis.     

Relapsing linear acantholytic dermatosis

A 5-year-old girl with a unilateral linear systematized skin lesion showing the clinical, histological and ultrastructural features of Hailey-Hailey disease is described. We suggest that this relapsing linear acantholytic dermatosis represents a new distinct entity within the spectrum of unilateral linear skin diseases.     

Transient acantholytic dermatosis in immunocompromised febrile patients with cancer

A focal acantholytic dermatosis resembling transient acantholytic dermatosis (TAD) clinically and histologically occurred in four immunocompromised and persistently febrile patients with various malignant neoplasms. No pathologic organism was isolated in cultures from blood or from the actual skin lesions. The rash resolved over several days, coinciding with defervescence. As do authors of other reports of TAD, we believe that the etiology of TAD is related to heat, persistent fever, and/or sweating.     

Persistent acantholytic dermatosis related to actinic damage

Ten male patients aged 38-63 years are presented, in whom a chronic, itchy, papular eruption developed on the upper trunk. Histology showed the characteristic features of Darier's disease. All the patients had clinical evidence of considerable solar damage and skin malignancies were frequently present. The condition is thought to represent a distinct entity which has been called persistent acantholytic dermatosis. The relationship between this condition, transient acantholytic dermatosis and actinic keratoses with Darier-like histology is discussed.     

Leukemia cutis in association With Grover's disease

Grover's disease (GD), or transient acantholytic dermatosis, is a persistent recurrent dermatosis that usually occurs in men older than 50 years. Rare cases of GD and hematologic malignancy in the same cutaneous biopsy specimen have been reported. We report a case of GD in association with leukemia cutis. A 72-year-old man with a history of myelodysplastic syndrome presented with numerous pruritic papules on the torso, which were clinically diagnosed as GD. A skin biopsy revealed foci of suprabasal acantholysis and dyskeratosis consistent with GD and dense aggregates of mononuclear atypical cells in the superficial dermis consistent with leukemia cutis. Direct immunofluorescence was negative. This case illustrates the need to consider a diagnostic skin biopsy in any patient who presents with classic clinical findings of GD if there is any indication that the patient may be at higher risk for a hematologic malignancy.     

复发性线状棘层松解性皮病一例

复发性线状棘层松解性皮病是一种比较少见的疾病。患儿,女,6岁。右侧臀部、股后红色斑块反复5年余,加重2个月。皮肤科检查：右侧臀、股后部、腘窝见条索状分布的暗红色斑块,上覆白色鳞屑,局部结血痂,散见色素沉着斑,皮损沿Blaschko线分布。组织病理学检查示：表皮角化过度、角化不全,棘层松解呈砖墙倒塌样。直接免疫荧光(DIF)阴性。     

Immunoblastic lymphadenopathy with purpura and cryoglobulinemia.

Immunoblastic lymphadenopathy is a recently described lymphoproliferative disorder, presumably of B-cell origin. It is characterized by regional or generalized lymphadenopathy, usually associated with hypergammaglobulinemia or dysproteinemia. Other findings may be hepatosplenomegaly, dermatitis, fever, malaise, weight loss, and various altered immunologic reactions. Histologically, the involved lymph nodes show immunoblast, plasmacytoid, and plasma cell proliferation. This may be extranodal as well. The case reported here is one of the few followed up prospectively. The patient's purpuric eruption was an apparent manifestation of a type II mixed cryoglobulinemia. Differing from what has usually been reported, we noted hypogammaglobulinemia and findings in part of altered cell-mediated immunity. Despite leukopenia and anemia there were no infectious episodes. Although a satisfactory treatment regimen has not been established, there was beneficial response to prednisone and short courses of melphalan.     

Sweat-related dermatoses: old concept and new scenario

This review focuses on dermatoses that are caused by the presence of sweat--a previously underemphasized subject. A working classification based on 'internal sweating' (extravasated sweat in the dermis and epidermis) and 'external sweating' (sweat on skin surface) is proposed. Clinical observations suggest that transient acantholytic dermatosis is the consequence of extravasated sweat; we speculate that it is an example of sweat-related, protease-induced epidermal acantholysis. Neutrophilic eccrine hidradenitis may represent a sweat-related drug reaction. We emphasize the well-recognized phenomenon of 'sweat retention syndrome' in a new scenario: hospitalized febrile patients, increasing use of chemotherapy, new life style. The concept of 'sweat-gland-mediated cutaneous inflammation' is proposed.