Subacute Spongiotic Dermatitis Research Articles

Long term efficacy, safety, and tolerability of tildrakizumab in epileptic patient with psoriasis and eczema.

Psoriasis is a chronic inflammatory disease which mostly affects skin. Tildrakizumab is a specific anti-interleukin -23p19 monoclonal antibody approved for the treatment of plaque psoriasis in adults. Herein, we report about a patient who came to our attention for a moderate-to-severe plaque psoriasis, involving primarily upper limbs, elbow, abdomen and knees (PASI 18 - DLQI 22). His medical history was relevant for epilepsy controlled pharmacologically. In addition, an eczematous and edematous appearance of the tibial area was detected; the histologic findings did not contradict the diagnostic hypothesis of subacute spongiotic dermatitis. The patient was treated with Tildrakizumab. After 12 weeks the clinical lesions improved significantly, and the eczematous component disappeared in the tibial area bilaterally. The clinical improvement was maintained even after one year of therapy. Tildrakizumab showed excellent results in the control of psoriasis, with an excellent safety profile. The promising results of clinical trials have been confirmed in a real-life setting. There are no reports about its safety in epileptic patients. In our case, neurological adverse events did not verify and tildrakizumab managed to control both the psoriatic and eczematous components.

SEVERE PAPULAR ECZEMA SKIN REACTION AFTER INTRAVENOUS IMMUNOGLOBULIN INFUSION IN PATIENT WITH CHRONIC LYMPHOCYTIC LEUKEMIA: A CASE REPORT

Hypogammaglobulinemia is a common finding in Chronic Lymphocytic Leukemia (CLL). Their etiology is multifactorial, due to disease-related immune defects and chemo-immunotherapy treatment as Rituximab. It is present in up to 85 % of patients at some point in their disease course. It is therefore important to monitor patients for the development of an antibody deficiency due to being susceptible to infections. Strategies to prevent infections in patients with secondary antibody deficiencies include replacement Immunoglobulin (Ig). However, nearly 6% develop skin reaction Ig associated, a rare adverse event reported only in case reports. A 64 years-old man was diagnosed with CLL in 2011. Despite treatment with Chlorambucil, in July 2013, the patient had therapeutic failure, so he switched to a 2nd-line regimen, with intravenous chemotherapy with cyclophosphamide, vincristine and prednisone for 2 cycles. In September 2013, due to disease progression, he was switched to a 3rd-line regimen with fludarabine and cyclophosphamide. In 2014, he presented an increase in the size of the cervical lymph nodes, so he used 8 cycles of Rituximab. After Rituximab-therapy, hypogammaglobulinemia was detected due to recurrent infections. Then, the patient received Ig monthly with pre-medication. Fifteen days after this Ig infusion, he developed an extensive pruritic, eczematous rash and lichenified skin lesions on the upper limbs. In the following months, 48 hours after the Ig infusion, he presented excoriated eczematous plaques located in the inguinal region and posterior thighs, with follicular pustules (delayed hypersensitivity reaction, type IV). Histological analysis of a skin biopsy specimen revealed subacute spongiotic dermatitis with intraepidermal microvesiculation. The lesions progressively regressed after stopping Ig infusions, oral prednisone, dapsone and topical application of tacrolimus and corticosteroids. Although the Rituximab used in the treatment of patients with CLL is capable of depleting the population of malignant lymphocytes, they lack specificity and further contribute to the significant immune dysfunction that predisposes these patients to a serious risk of infection. As such, the use of Ig therapy is increasing in the treatment of CLL. However, the severity of its rare adverse effects is often poorly recognized. Dermatologic adverse effects of Ig are rare and include urticaria, papules, eczema, pompholyx, lichenoid dermatitis, and desquamation. The underlying mechanism resulting in skin reactions following treatment with Ig is not clear. Most of these reactions develop within 2 weeks after immunoglobulin administration. The rash is managed with topical/systemic steroids and antihistamine treatment. These adverse events can also lead to cessation of therapy. This case report highlights the fact that cutaneous adverse effects of IgIV are more common than previously believed. Although the precise mechanism of this cutaneous eruption remains to be elucidated, its occurrence within days of Ig infusion, its characteristic distribution at onset, and its clinical course should be recognized by hematologists and dermatologists.

An unusual presentation of pemphigus foliaceus following COVID‐19 vaccination

An unusual presentation of pemphigus foliaceus following COVID‐19 vaccination

CIRCULATING SEZARY CELLS IN SUBACUTE SPONGIOTIC DERMATITIS

Sezary syndrome comprises a triad of fiery red erythroderma, generalized lymphadenopathy, and circulating atypical cells with cerebriform nuclei (Sezary cells) exceeding an absolute value of 1000/cumm or exceeding 10% of circulating cells. Sezary cell is not pathognomonic of Sezary syndrome and can be seen in many benign dermatoses such as chronic eczema, psoriasis, lupus erythematosus, parapsoriasis, atopic dermatitis, and vasculitis. We report circulating Sezary cells in a patient of subacute spongiotic dermatitis, one of the rare causes for Sezary cells in peripheral blood.

FOLLICULAR MUCINOSIS IN A PATIENT WITH PARAPSORIASIS

We present the case of a young patient, without comorbidities, who developed an erythematous-squamous, disseminated, discrete itchy rash, associated with scarring alopecia at the level of the scalp and the external third of the eyebrows, in 3-year evolution, in which two skin biopsies showed: subacute spongiotic dermatitis and psoriasiform dermatitis. As new lesions continued to appear despite treatment with phototherapy, systemic, local corticosteroids, it was decided to perform another skin biopsy: loose areas, granular appearance that color blue with Alcian Blue staining, thus supporting the diagnosis of follicular mucinosis. The treatment consisted of Acitretin 30 mg / day, with favorable evolution. Follicular mucinosis is a rare inflammatory disease, characterized by mucin deposits located in the follicular epithelium and sebaceous glands. There are two clinical forms: a primary, benign, limited to the skin, and a secondary form, commonly associated with cutaneous T-cell lymphoma. The evolution of both forms of follicular mucinosis is a chronic one, and the correct therapeutic attitude consists primarily in the periodic surveillance of the patient, with possible repeat biopsy, to exclude the form associated with a mycosis fungoides. The management of such patients is extremely difficult, as a number of conditions must be evaluated, such as: screening for collagenosis, diabetes, inflammatory bowel disease, evaluation of thyroid function, tumor markers.

2343 Trimethoprim-Sulfamethoxazole Induced DRESS Requiring Liver Transplant

INTRODUCTION: Trimethoprim-Sulfamethoxazole induced DRESS requiring Liver Transplant. CASE DESCRIPTION/METHODS: Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome can occur in the setting of DILI and is indicative of an idiosyncratic reaction which is now increasingly recognized with antibiotic use. The pathophysiology of DRESS is incompletely understood, but thought to involve reactivation of herpes viruses, genetic deficiency of detoxifying enzymes and associations between human leukocyte antigen (HLA) and drug hypersensitivity, leading to overwhelming response by the immune system. DILI-associated DRESS is rarely refractory to medical treatment requiring liver transplantation. We report a case of a 21-year-old male who presented with acute liver injury preceded by two weeks of nausea and vomiting. Prior to onset of symptoms, he was started on trimethoprim-sulfamethoxazole (TMP-SMX) for penile discharge. On presentation, he had a fever of 100.5°F, right upper quadrant abdominal pain and an erythematous rash involving upper extremities and later spreading to his face, trunk and lower extremities. Review of his records showed normal aspartate aminotransferase (AST) and alanine aminotransferase (ALT) from a week prior to admission but repeat labs showed an AST of 1480, and ALT of 764. He was found to have an elevated prothrombin time (PT) and INR but no signs of encephalopathy. Complete blood count with differential showed peripheral eosinophilia. Workup for infectious, autoimmune, and metabolic etiologies returned negative and broad-spectrum antibiotics were started for recurring fevers. European Registry of Severe Cutaneous Adverse Reactions (RegiSCAR) score of 4-5 placed the patient as ‘Probable DRESS’ with the most likely culprit being TMP-SMX. Skin punch biopsy showed subacute spongiotic dermatitis with eosinophils consistent with DRESS. Due to worsened liver function a liver biopsy was obtained which showed lobular hepatitis with sub-massive hepatocyte necrosis and increased eosinophils. The patient developed encephalopathy and successfully underwent liver transplantation with resolution of his symptoms post-transplant. DISCUSSION: This case demonstrates the importance of early evaluation, diagnosis and management of acute liver in patients with indication of DRESS Syndrome. It also emphasizes the importance of early transfer of patients with significant liver involvement to a liver transplant center as it can improve clinical outcomes.

Histopathologic spectrum of hypersensitivity reactions associated with anti‐CD52 therapy (alemtuzumab)

Alemtuzumab is a humanized monoclonal antibody directed against CD52, a cell surface antigen on B and T lymphocytes, and used to treat B-cell chronic lymphocytic leukemia and cutaneous T-cell lymphoma. Skin rash is a common adverse reaction following treatment with alemtuzumab. However, the clinicopathologic features and immunologic basis for the reaction have not been previously reported. Our hospital's electronic pathology database was searched for cases with documentation of 'alemtuzumab' or 'anti-CD52' in the clinical history provided by either the ordering physician or the pathologist. Clinical and histopathologic review of the cases was performed. Five patients with cutaneous T-cell lymphoma (CTCL) or chronic lymphocytic leukemia (CLL) were treated with alemtuzumab, and developed pruritic, erythematous papules and plaques. Histopathology of the skin lesions revealed subacute spongiotic dermatitis with multifocal parakeratosis, endothelial activation and perivascular lymphocytic infiltrate. Eosinophils were not a prominent feature. We describe the clinicopathologic features of a novel hypersensitivity reaction to alemtuzumb, and hypothesize it may be due to an immunologic response precipitated by the persistence of resident memory T-cells (TRM ) in the skin. Our findings raise awareness for a novel reaction pattern and guide the histopathologic interpretation of lesions which may clinically mimic residual or recurrent cutaneous lymphoproliferative disorders.

Hypertrophic lichen planus-like atopic dermatitis: a case report

Results The lesions started at age 18 during the patient s first pregnancy and had a predominant flexural distribution pattern. At the physical exam, the patient presented with erythema and infiltrative lesions in forearm and periorbital regions, and scaly erythematous papules, especially in inferior extremities. Some of these lesions presented an ulcerous center and had linear form. The patient had high specific serum IgE levels for house dust mites (Dermatophagoides farinae and D. pteronyssinus), grass and dog; sea food, fish, soy, wheat and latex. Serum total IgE was 11,100kU/L, and serology for hepatitis B and C was negative. The patient was treated with antihistamines, topical emollients and oral corticosteroids with low improvement. Because of this atypical presentation, other differential diagnoses were considered, including hypertrophic lichen planus. Skin biopsy was performed, showing hyperkeratosis, sub-acute spongiotic dermatitis and moderate acanthosis, compatible with atopic dermatitis and an evolution to lichen simplex chronicus. Conclusions Hypertrophic lichen planus is one of the differential diagnosis of atopic dermatitis and it commonly involves the flexor surfaces of the extremities bilaterally. Adultonset AD can present with non-typical morphology and localization, therefore it is important to distinguish these two entities, since lichen planus can be associated with other diseases, such as viral hepatitis.

A case of autoimmune progesterone dermatitis diagnosed by progesterone pessary

Autoimmune progesterone dermatitis is a rare, cyclical eruption that occurs in the luteal phase of the menstrual cycle and during pregnancy. Many manifestations have been reported including cyclical eczema, urticaria, erythema multiforme, stomatitis and even anaphylaxis. The condition spontaneously resolves after menopause. As histopathology is non-specific, the diagnosis rests on history with precipitation of the eruption by a progesterone challenge, usually by the intradermal, intramuscular or oral route. We present the case of a 34-year-old woman with a premenstrual papular and eczematous eruption that was exacerbated after pregnancy. Biopsy showed subacute spongiotic dermatitis. To confirm the diagnosis, we used an intravaginal progesterone pessary as a provocation challenge. There was recurrence of the rash 12 h after insertion of the pessary with spontaneous resolution thereafter. We propose that use of a progesterone pessary is an effective tool in the diagnosis of autoimmune progesterone dermatitis.

Superimposed segmental dermatitis with chronic prurigo

Common acquired skin diseases with a polygenic background, such as lichen planus, may show linear or segmental manifestations of underlying systemic skin disease. The linear arrangement in such cases is usually consistent with the lines of Blaschko. Happle summarized the various types of segmental arrangement of common polygenic diseases and proposed a novel designation of superimposed segmental dermatosis. Here, we report a unilateral linear dermatitis distributed along the lines of Blaschko on the leg, which was not self-healing and persisted for at least 6 years without complete remission, and was accompanied by preceding chronic prurigo on the extremities. Histological examination showed subacute spongiotic dermatitis and epidermal infiltration of CD4-positive cells. This case report presents a superimposed segmental dermatitis that arose based on systemic eczematous conditions, such as chronic prurigo.

Pityriasis Alba: A Condition with Possibly Multiple Etiologies

Pityriasis alba is a common hypomelanosis which is generally seen in young children. There is no specific known cause for this condition; however, studies to elucidate pathogenic factors seem to suggest that there are at least five separate causes for the condition. This list includes dermatitis, photosensitivity, fungal and bacterial pathogenesis, and post-inflammatory alterations. An evaluation into causative factors translates into alternative therapeutic options, which may be advantageous with affected individuals. When a disease has diverse clinical presentations and no known cause, it is possible that one is looking at slightly different diseases with similar clinical characteristics. Pityriasis alba is a common condition in practice mostly occurring in children between the ages of 3 and 16, but can be additionally seen in young adults. It usually appears as dry, fine scaled, pale patches, 0.5 to 6 cm in diameter; how- ever, often the flakiness is not present. The rash is limited to the face in 50 % of cases, particularly the mid-forehead, ma- lar ridges, and around the eyes and mouth; nevertheless, in- volvement of the shoulders, neck, back, and upper chest may predominate in others. It is symmetrical in distribution, but sometimes only marginally. The condition often begins as a pale pink or light brown macule with very indistinct margins, but it often just appears suddenly with decreased pigmenta- tion. Macules vary from 5 to 30 mm or larger. The condition is so named as 'pityriasis' means scaly and 'alba' is the word for white in Latin. Of note, the patches in pityriasis alba are not totally depigmented as with vitiligo. The hypopigmentation with pityriasis alba is due to both reduced activity of melanocytes as well as fewer and smaller melanosomes; in vitiligo, on the other hand, there is total loss of both melanocytes and melanosomes. Histologically, the condition reveals subacute spongiotic dermatitis with decreased melanin within the epidermis. A differential diag- nosis would include vitiligo, psoriatic leukoderma, tinea ver- sicolor, follicular mucinosis, pityriasis lichenoides chronica, sarcoidosis, adult T-cell leukemia/lymphoma, and mycosis fungoides. There is no specific known cause for this condition; how- ever, studies to elucidate pathogenic factors seem to suggest that there are at least five separate causes for the condition. First, there is an increased incidence in individuals who bath excessively, defined as showering over once daily (1). This could infer that removal of normal epidermal defensins and other natural protective substances from the skin surface,

Acquired Blaschkoid dermatitis

Acquired Blaschkoid dermatitis characterised by unilateral relapsing inflammatory disease along the lines of Blaschko. A 40-year-old Indian male presented with unilateral erythematous, itchy grouped papules on the left side of the chest, abdomen, back and left arm of 15 days duration. The eruption stopped abruptly at the midline of the torso, completely sparing the right side of the body. The lesions were arranged in whorls and streaks corresponding to the lines of Blaschko. Skin biopsy showed hyperkeratosis and features suggestive of sub-acute spongiotic dermatitis with lymphocytic infiltrate around the blood vessels in the dermis. Patient was diagnosed as a case of Blaschkoid dermatitis. To the best of our knowledge, this is the first case of this condition being reported from India.

The varieties of “eczema”: clinicopathologic correlation

Eczema is defined in the third Webster’s unabridged dictionary as an “acute or chronic noncontagious inflammatory condition of the skin characterized by redness, itching, oozing...associated with exposure to chemicals or irritants.” The word derives from Greek, meaning literally to erupt, ferment, or boil. The broadness of this term and its association with just about any inflammatory condition of the skin has prompted some authors to opine that “ it is devoid of meaning” and that it should be “expunged” from the dermatologic lexicon. The consensus among many dermatopathologists and a few dermatologists is that the expression eczema should be replaced with the term “spongiotic dermatitis.” Spongiosis refers to the histopathologic changes that underlie most of the so-called eczemas, ie, edema between the keratinocytes of the stratum malpighii, which gives a spongy appearance to the epidermis. Although most authors have accepted this concept, it is actually a gross oversimplification. Spongiosis occurs in numerous other dermatoses that are not, by conventional wisdom, classified as “eczema.” Examples include, but are not limited to pityriasis rosea, Gianotti Crosti syndrome, the annular erythemas, miliaria, Grover’s disease, polymorphous light eruptions, papular urticaria, lichen striatus, and some pigmented purpuras. Because a full discussion of this issue is beyond the scope of this article, for purposes of discussion, eczema is defined here as “an inflammatory skin disorder that histologically shows spongiosis and is related etiologically to some type of immunologic perturbation.” This review will cover two major points concerning spongiotic dermatitis. First, spongiotic dermatitis will be examined as a histologic continuum. If the antigenic insult is severe and reaction time is short, the resulting dermatitis is designated as acute. If the antigenic stimulus is weaker and takes place over an extended period of time, then it is referred to as chronic. Severe chronic reactions typified by maximal physical changes are termed lichen simplex chronicus and prurigo nodularis. Although this histopathologic schema is a convention in common use by dermatopathologists, it should be understood that pathologic changes don’t always correlate exactly with a defined time course in a clinical dermatitis. The second major consideration of this paper will be the varieties of spongiotic dermatitis that occur at different anatomic sites and that may have varying etiologies. An attempt will be made to differentiate the characteristic histopathologic changes occurring at different anatomic locations and those occurring within different subtypes of spongiotic dermatitis; we will also discuss whether such changes can be readily identified by the dermatopathologist. For some of the dermatitides, a short summary of pathogenesis is included if it is relevant to understanding the pathology. There are five general categories of spongiotic dermatitis: 1) acute spongiotic dermatitis; 2) subacutespongiotic dermatitis; 3) chronic spongiotic dermatitis; 4) lichen simplex chronicus; and 5) prurigo nodularis. The first three categories actually represent a pathologic continuum, whereas the last two are a pathologic response to trauma superimposed by the patient on the underlying dermatitis. In acute spongiotic dermatitis, the spongiosis is typified by massive intercellular edema of the epidermis with widening of the intercellular spaces. The edema produces disruption of desmosomal attachments, and as a consequence, numerous microvesicles form. Although these vesicles are usually intraepidermal, with sufficient vesiculation they can become subepidermal. In general, the degree of epidermal acanthosis is slight. Dermal edema is common, and it is often accompanied by a pronounced inflammatory infiltrate of mononuclear cells and eosinophils. Although there may be some parakeratosis, in general, the changes in the horn are slight given the rapidity at which the process usually develops. A frequent accompaniment is a scale crust composed of neutrophils, plasma, and bacterial colonies ie, so-called impetiginization (Fig. 1). In subacute spongiotic dermatitis, the vesicles are smaller and there is greater acanthosis of the epidermis. From the Departments of Dermatology and Pathology, Mount Sinai School of Medicine, New York, New York. Address correspondence to Dr. Robert G. Phelps, Dermatopathology Division, Box 1194, 1 Gustave L. Levy Place, New York, NY 10029. E-mail address: bobpderm@aol.com.

Acquired relapsing self-healing Blaschko dermatitis

We describe a 44-year-old woman who had a unilateral relapsing linear dermatosis for 12 years. The lesions consisted of erythematous, discrete and grouped papules and papulovesicles that were localized to the left side of the upper and lower limbs, chest, abdomen, and back. They were distributed along Blaschko's lines. There was also a diffuse erythematous scaly hyperkeratosis of both palms. The lesions healed spontaneously. Examination of biopsy specimens from the back and the left palm revealed acute and subacute spongiotic dermatitis, respectively. This dermatosis was first described by Grosshans and Marot in 1990 and termed "Blaschkite de l'adulte." Because the disease is acquired, relapsing, heals spontaneously, follows Blaschko's lines, and is characterized histopathologically by a spongiotic dermatitis, we propose the term "acquired relapsing self-healing Blaschko dermatitis."

Saphenous Vein Graft Donor Site Dermatitis

Coronary artery bypass grafting for atherosclerotic heart disease is commonly performed throughout the world. Complications of coronary artery bypass grafting include saphenous neuralgia due to injury to the saphenous nerve during harvest of the saphenous vein. Dermatologic complications of coronary revascularization are infrequently reported and include an eruption overlying the vein donor-site scar. We describe two cases of saphenous vein donor site dermatitis associated with sensory peripheral neuropathy in the distribution of the dermatitis. Histopathologic studies revealed a subacute spongiotic dermatitis. The course of the eruption was characterized by exacerbations and remissions with gradual resolution of both the dermatitis and neuropathy over a 1- to 2-year period. Our cases are unique because the dermatitis developed in the area of the neurologic changes. We propose that the dermatitis may be a trophic change secondary to saphenous neuralgia.

Psoriasiform acral dermatitis. Report of three cases.

The authors report 3 patients affected by psoriasiform acral dermatitis, a distinctive clinical entity characterized by a chronic dermatitis of the terminal phalanges, associated with marked shortening of the nail beds of the affected fingers. The skin biopsy showed in all cases the pathological features of a subacute spongiotic dermatitis. X-ray examination of affected fingers showed no bone or soft tissue changes. Differential diagnosis of psoriasiform acral dermatitis included psoriasis, atopic or contact dermatitis and corticosteroid-induced distal phalangeal atrophy.