Diagnostic approach of eosinophilic spongiosis

Abstract Eosinophilic spongiosis is a histological feature shared by some distinct inflammatory disorders, and is characterized by the presence of intraepidermal eosinophils associated with spongiosis. Most often, isolated eosinophilic spongiosis indicates the early stages of a subjacent autoimmune bullous dermatosis, such as the pemphigus group and bullous pemphigoid. Herein, the main causes of eosinophilic spongiosis are discussed, as well as the supplementary investigation needed to elucidate its etiology.

Adult linear IgA bullous dermatosis: report of three cases

Abstract: Linear immunoglobulin A bullous dermatosis is a rare autoimmune disease that usually has an excellent prognosis in childhood; however, its control is more difficult in adults. It presents heterogeneous clinical manifestations and is frequently confused with other bullous diseases such as bullous pemphigoid and Duhring's dermatitis herpetiformis. Dermatologists' awareness of this disease contributes to early diagnosis and appropriate treatment. We thus report three cases of linear immunoglobulin A dermatosis in adults.

Atypical Gianotti-Crosti syndrome

Abstract: We report the case of a male 22-month-old child, with atypical presentation of Gianotti-Crosti syndrome after infection with Epstein-Barr virus.

Subcorneal pustular dermatosis in the pediatric age

Abstract: Subcorneal pustular dermatosis is a rare pustular eruption which occurs mainly in middle-aged women and rarely during childhood. We report a case of a 15-year-old female with a 4-year history of pustular lesions on the proximal region of the upper limbs with subsequent impairment of the trunk. Physical examination revealed small pustules distributed on the trunk and proximal region of the limbs. Histopathology showed a subcorneal pustule and direct immunofluorescence for IgA, IgM, IgG and fibrinogen was negative, confirming the diagnosis of subcorneal pustular dermatosis. The patient was treated with dapsone with good clinical response after one month. Subcorneal pustular dermatosis is a rare condition and there are only isolated cases reported in the literature in pediatric patients. Thus, we discuss the main clinical aspects and treatment response of this condition during childhood.

Bullous pyoderma gangrenosum as a predictor of hematological malignancies

Abstract: Pyoderma gangrenosum may precede, coexist or follow diverse systemic diseases. The bullous variety is usually associated with hematologic disorders. From 31 patients with pyoderma gangrenosum diagnosed in our hospital during 10 years, only 2 presented with the bullous type. One patient had previous diagnosis of chronic myeloid leukemia. Both had fast-progressive, painful lesions, and both showed an excellent response to systemic corticosteroids. In less than 3 months the lesions recurred simultaneously with the progression of chronic myeloid leukemia in one patient and with the initial diagnosis of acute myeloid leukemia in the other one. They died in less than four weeks. These findings emphasize the importance of its timely diagnosis. Cutaneous lesions could be the first predictor of an underlying disease.

Diabetes mellitus and the skin

Abstract: Several dermatoses are routinely associated with diabetes mellitus, especially in patients with chronic disease. This relationship can be easily proven in some skin disorders, but it is not so clear in others. Dermatoses such necrobiosis lipoidica, granuloma annulare, acanthosis nigricans and others are discussed in this text, with an emphasis on proven link with the diabetes or not, disease identification and treatment strategy used to control those dermatoses and diabetes.

Vancomycin-associated linear IgA disease mimicking toxic epidermal necrolysis

Abstract Linear IgA dermatosis is a rare subepidermal autoimmune blistering disease characterized by linear deposition of IgA along the basement membrane zone. In the last three decades, many different drugs have been associated with the drug-induced form of the disease, especially vancomycin. We report a case of vancomycin-induced linear IgA disease mimicking toxic epidermal necrolysis. The aim of this work is to emphasize the need to include this differential diagnosis in cases of epidermal detachment and to review the literature on the subject and this specific clinical presentation.

Linear IgA and IgG bullous dermatosis

Abstract Childhood linear immunoglobulin A dermatosis is a rare autoimmune vesiculobullous disease. It results in linear deposition of autoantibodies (immunoglobulin A) against antigens in the basal membrane zone, leading to subepidermal cleavage. Additional depositions of immunoglobulin G and complement-3 might occur. It is still debated whether concomitant findings of immunoglobulins A and G should be considered a subtype of this dermatosis or a new entity. Further studies are needed to recognize this clinical variant.

Ofuji disease: a rare dermatosis and its challenging therapeutic approach

Abstract: Eosinophilic pustular folliculitis (EPF) or Ofuji disease is a rare dermatosis, prone to recurrence and chronicity. The peak incidence occurs in the third decade of life and its exact etiology remains unknown. Evidence suggests that the expression of adhesion molecules and the production of cytokines activate the follicular unit, but the stimulus that triggers these changes remains unclear. The three clinical variants reported in the literature include classic EPF, immunosuppression-associated EPF, and infancy-associated EPF. We report a case of eosinophilic pustular folliculitis with peculiar epidemiological characteristics, which represents a challenging therapeutic scenario.

Rheumatoid arthritis and pseudo-vesicular skin plaques: rheumatoid neutrophilic dermatosis

Abstract: A 54 year-old woman with a 3-year history of rheumatoid arthritis (RA) consulted us because of weight loss, fever and skin eruption. On physical examination, erythematous plaques with a pseudo-vesicular appearance were seen on the back of both shoulders. Histological examination was consistent with rheumatoid neutrophilic dermatosis (RND). After 3 days of prednisone treatment, the skin eruption resolved. RND is a rare cutaneous manifestation of seropositive RA, characterized by asymptomatic, symmetrical erythematous plaques with a pseudo-vesicular appearance. Histology characteristically reveals a dense, neutrophilic infiltrate with leucocitoclasis but without other signs of vasculitis. Lesions may resolve spontaneously or with RA treatment. This case illustrates an uncommon skin manifestation of active rheumatoid arthritis.

Benign skin disease with pustules in the newborn

Abstract: The neonatal period comprises the first four weeks of life. It is a period of adaptation where the skin often presents several changes: transient lesions, resulting from a physiological response, others as a consequence of transient diseases and some as markers of severe disorders. The presence of pustules in the skin of the newborn is always a reason for the family and for the assisting doctor to be worried, since the newborn is especially vulnerable to bacterial, viral or fungal infection. However, the majority of neonatal skin pustules is not infectious, comprising the benign neonatal pustulosis. Benign neonatal pustuloses are a group of clinical disease characterized by pustular eruptions in which a contagious agent is not responsible for its etiology. The most common ones are erythema toxicum neonatorum, the transient neonatal pustular melanosis and the benign cephalic pustulosis. These dermatoses are usually benign, asymptomatic and self-limited. It is important that the dermatologist and the neonatologist can identify benign and transient lesions, those caused by genodermatoses, and especially differentiate between neonates with systemic involvement from those with benign skin lesions, avoiding unnecessary diagnostic tests and worries.

Epidermolytic Hyperkeratosis - case report

Abstract: Epidermolytic hipercetarose is a rare genodermatosis, with a prevalence of 1:100.000 to 1:300.000, with autosomal dominant inheritance. We report the case of a 5 year old girlwho presented an hypertrophic verrucous plaques in the neck, under arm, buttocks, knees, pelvis, legs, dorsum of the right foot and elbows. Histological examination of the skin lesions showed typical changes of epidermolytic hyperkeratosis. Because it is an autosomal dominant disorder with complete penetrance, the individual carrying the mutation will necessarily develop the disease. However, in 50% of cases postzygotic mutation occur. The case report emphasizes early diagnosis and differential diagnoses with ichthyosis and other bullous diseases of childhood, as well as discussing the therapeutic possibilities.

Incontinentia pigmenti or Bloch-Sulzberger syndrome: a rare X-linked genodermatosis

Incontinentia pigmenti is a rare X-linked genodermatosis that affects mainly female neonates. The first manifestation occurs in the early neonatal period and progresses through four stages: vesicular, verruciform, hyperpigmented and hypopigmented. Clinical features also manifest themselves through changes in the teeth, eyes, hair, central nervous system, bone structures, skeletal musculature and immune system. The authors report the case of a patient with cutaneous lesions and histological findings that are compatible with the vesicular stage, emphasizing the importance of early diagnosis and appropriate therapeutic management.

Tumourlike Lesions of Skin - A Clinicopathological Analysis of 175 Cases.

As the skin is the largest and complex organ, a wide variety of tumourlike lesions are encountered in the clinical practice. The ability to properly diagnose & treat these common lesions & to distinguish them from malignant tumours is the vital skills for all clinicians. Objective: To analyse retrospectively tumourlike lesions of skin with respect to age, sex, clinical features and histopathological features in a tertiary referral centre in Maharashtra, India. Methods : The present study consisted of analysis of tumourlike lesions of skin received in the histopathology section of department of pathology over a period of 5 years that is from August 2005 to July 2010 .The material comprised of biopsies and excision specimens. The clinical and histopathological details were noted. The findings were compared with those reported by other authors. Results : One hundred and seventy five (175) cases of tumourlike lesions of skin were seen .These lesions presented as skin swellings or tumours. Maximum cases (34.8%) of tumourlike lesions occurred in fourth & fifth decades with male preponderance (1.35:1). The maximum number of cases was encountered in the head & neck region (63.4%). Their size ranged from 0.4 to 9 cms. Epidermal cyst was the commonest tumourlike lesion (59.4%) followed by dermoid cyst (13.1%).Other lesions were trichilemmal cyst, fibroepithelial polyp, keloid, hypertrophic scar and epidermal nevus. Conclusion : Tumourlike lesions are clinically diagnosed by their presentation. However, the histopathological examination confirms the clinical diagnosis. The pathologic evaluation of all tumourlike lesions is mandatory to avoid patient’s and family’s anxiety.

Comparative scanning electron microscopy of bullous diseases

The purpose of this study is to compare scanning electron microscopy findings of the blister roof in three distinct bullous diseases: one intraepidermal acantholytic (pemphigus foliaceus); one due to hemidesmosomal dysfunction (bullous pemphigoid); and one secondary to anchoring fibril dysfunction - type VII collagen (dystrophic epidermolysis bullosa). In pemphigus foliaceus, acantholytic phenomena were readily demonstrated. In bullous pemphigoid, the epidermis had a solid aspect. In dystrophic epidermolysis bullosa a net was seen in the blister roof.

Case for diagnosis / Caso para diagnostico

Linear Darier is a rare variant of Darier's Disease. The keratotic papules follow Blaschko's lines, characterizing the cutaneous mosaicism. We report the case of a 42-year-old woman who presented with small, linearly distributed, reddish-brown papules on the left mammary region, from the left flank to the hypogastrium and on the pretibial area of the left leg. Histologically, suprabasal acantholysis and dyskeratotic cells were seen, confirming the diagnosis.

Darier linear é uma variante rara da Doença de Darier. As pápulas ceratósicas se distribuem seguindo as linhas de Blaschko, caracterizando o mosaicismo do envolvimento cutâneo. Relatamos o caso de uma mulher de 42 anos que apresentava pápulas acastanhadas, distribuídas linearmente na região mamária esquerda, do flanco esquerdo ao hipogástrio e na região prétibial esquerda. Histologicamente, foram observadas acantólise suprabasal e células disceratósicas, confirmando a suposição diagnóstica.

Case for diagnosis / Caso para diagnóstico

Lichen sclerosus is a chronic inflammatory mucocutaneous disorder of unknown etiology that most commonly affects the female genitalia. Cutaneous involvement with nonhaemorrhagic bullous is very unusual. We describe a case of bullous lichen sclerosus.

Líquen escleroso é uma doença mucocutânea inflamatória, crônica, de etiologia desconhecida, que afeta mais comumente a genitália feminina. O envolvimento cutâneo com bolhas não hemorrágicas é muito raro. Descrevemos um caso de líquen escleroso bolhoso.

Pustulosis subcórnea (enfermedad de Sneddon-Wilkinson): Caso clínico / Subcorneal pustular dermatosis: Report of one case

Background: We report a 35-year-old female patient with a one year history of a pustular and painful erythematous dermatitis, located in great folds, pubis and abdomen. She was evaluated in primary health care, receiving antifungal treatment, antimicrobials, topical and systemic non-steroidal anti-inflammatory drugs, with no response. A skin biopsy was compatible with subcorneal pustular dermatosis. She was initially treated with prednisone (0.8 mg/kg), observing a partial response. Therefore clotrimoxazole was initiated, obtaining an excellent response after 30 days of treatment.