Lauretta Amato

Cicatricial alopecia; a dermatopathologic and immunopathologic study of 33 patients (pseudopelade of brocq is not a specific clinico-pathologic entity)

Abstract

A case of chronic herpes gestationis: persistent disease or conversion to bullous pemphigoid?

We report the case of a 38-year-old woman with herpes gestationis (HG) persistent for 26 months postpartum with typical erythematous-edematous grouped lesions associated with vesicles, blisters, and scaled crusts on most parts of her body. Despite high doses of oral prednisolone, azathioprine, and dapsone, and a trial of 5 plasmaphereses, the disease has persisted to date. Histopathologic examination of lesional skin showed subepidermal blisters, focal basal cell necrosis, and a dermal inflammatory infiltrate including many eosinophils. Direct immunofluorescence showed linear C3 staining in the basement membrane zone and the complement fixation test demonstrated circulating antibasement membrane zone antibodies at a low titer (1:80). HLA typing demonstrated an A2, A24, B35, B52, DR4,5, DR13,15, DRW52,53 phenotype. We present this case as chronic persistent HG and discuss the differential diagnosis between chronic persistent HG and HG evolving to bullous pemphigoid, together with a careful examination of similar cases reported in literature.

Subacute Cutaneous Lupus Erythematosus in Childhood

Abstract: We report a 7‐year‐old girl who presented with erythematous‐infiltrated, figurate, well‐defined lesions over sun‐exposed skin and antinuclear and Ro/SSA antibodies. Lupus band test revealed granular IgM and microgranular C3 deposits at the dermoepidermal junction. Histopathologic examination of lesional skin showed orthohyperkeratosis, epidermal atrophy, widespread hydropic degeneration of the epidermal basal cell layer, and a dermal perivascular lymphohistiocytic infiltrate. The clinical, immunopathologic, and histopathologic findings were consistent with the diagnosis of annular‐polycyclic variant of subacute cutaneous lupus erythematosus (SCLE). Treatment with hydroxychloroquine cleared the cutaneous lesions. We report this patient because SCLE is extremely rare in childhood, and discuss the two previous cases reported in the literature.

The role of T lymphocytes and cytokines in the pathogenesis of pemphigoid gestationis

Summary Background Pemphigoid gestationis (PG), also known as herpes gestationis, is a rare autoantibody‐mediated bullous disease, usually associated with pregnancy and the postpartum period. However, infiltrating cells have recently been suggested to also contribute to the pathogenesis of cutaneous lesions.

Evaluation of inflammatory infiltrate and fibrogenic cytokines in pseudopelade of Brocq suggests the involvement of T‐helper 2 and 3 cytokines

Background  Pseudopelade of Brocq (PB) is an acquired progressive cicatricial alopecia which is characterized by some distinctive clinical features. It may represent either a distinct entity, i.e. an idiopathic primary scarring alopecia, or the end stage of various forms of scarring alopecia such as discoid lupus erythematosus (DLE) or lichen planopilaris (LPP).

Familial cutaneous collagenoma: report of an affected family.

A 44‐year‐old white woman was admitted to our hospital for the investigation of many roundish, pale‐colored, nodular lesions of different sizes, ranging from a few millimeters to some centimeters, symmetrically grouped on the shoulders ( Fig. 1 ), trunk, upper limbs, and abdomen. The lesions first appeared at 20 years of age on the upper chest around the clavicular region, and thereafter gradually increased on the trunk and upper limbs; they were completely asymptomatic, appearing on normal skin without a previous history of inflammation and/or trauma. No scarring or atrophy was evident. The smaller lesions exhibited a miliaria‐like shape, being evident in particular on stretching of the skin; the larger ones showed a firm‐elastic density, no adhesion to surrounding tissue, and deep localization in the skin. No hypopigmented macules or sebaceous adenoma‐like lesions were present. Skin biopsy of a nodular lesion from the right shoulder revealed a normal epidermis and a dermis filled with bundles of dense and coarse collagen fibers ( Fig. 2 ). Elastic fibers appeared thin and decreased in number under Verhoeff–van Gieson stain ( Fig. 3 ). A diagnosis of cutaneous collagenoma was proposed. Routine laboratory investigations were within the normal range. Chest and skeleton (hands, pelvis, and long bones) X‐ray, electrocardiogram, and echocardiogram showed no abnormality.

Erythrodermic bullous pemphigoid.

Case 1 An 87‐year‐old man, with a 2‐year history of a generalized exfoliative erythroderma involving approximately 90% of the body, presented with an onset of tense blisters symmetrically distributed on most of his body.

A case of vesiculo-bullous Darier's disease associated with bipolar psychiatric disorder.

We describe the case of a 71‐year‐old men suffering from Dariers disease since his youth and presenting with intensely itching, vesicular and pustular lesions, localized on his arms, legs and on the ulnar side of his right hand. The patient also had a 30 year‐history of bipolar psychiaric disorders. The histopathological examination of lesional skin showed suprabasal acantholytic clefting, hyperparakeratosis, and dyskeratosis. Histopathological and immunopathological (direct and indirect immunofluorescence) results, were consistent with vesiculo‐bullous Dariers disease. Corticosteroid therapy with methylprednisolone at low dosage (0.2 mg/kg/day) was started with a quick resolution of the vesicular lesions on his arms and legs.

A multiparametric approach is essential to define different clinicopathological entities within pseudopelade of Brocq: reply from authors

SIR, We read with great interest the article ‘Reactive multiple keratoacanthoma in a patient with chronic renal insufficiency’ by Karakas et al. in which the authors described multiple keratoacanthomas at sites of previous injury found on a 40-year-old woman undergoing haemodialysis. The authors describe a single biopsy from one of this patient’s lesions that showed histopathological changes of a keratoacanthoma. We would like to suggest that in this clinical setting additional biopsies might provide evidence of a perforating dermatosis in some of the other lesions as previously reported by Rapini et al. In that study, skin biopsies from four patients demonstrated evidence of transepidermal elimination of both collagen and elastic fibres; thus Rapini et al. coined the term ‘acquired perforating dermatosis’ (APD) to encompass a variety of perforating diseases that have been associated with renal disease and diabetes mellitus. They suggested these varying pathological findings are manifestations of a single disease process or different stages of lesion development. We provide further evidence to support Rapini’s conclusions. We performed multiple biopsies from a 45-year-old white woman with a 10-year history of insulin-dependent diabetes, hypertension, and end-stage renal disease, secondary to diabetic nephropathy, treated with haemodialysis for 3 months. She presented with mildly pruritic lesions on her left forearm and face that had been present for several weeks. Physical examination revealed a cluster of rough papules on the left forearm (Fig. 1) with several scattered papules on her right cheek (Fig. 2), forehead, trunk and lower extremities. Several of these lesions had a clinical appearance suggestive of keratoacanthoma. Histopathology of a lesion from her left forearm and right cheek showed large keratotic plugs which formed curved channels into and through an acanthotic epidermis. Increased numbers of thickened, degenerated elastic fibres were present at the dermal–epidermal junction. A Verhoeff–van Gieson stain showed transepidermal migration of abnormal, thickened elastic fibres within the epidermis inside narrow perforating channels. Bacterial and fungal cultures were negative. These findings were interpreted as being most consistent with elastosis perforans serpiginosa. One month later, the patient complained of development of new lesions on her trunk and lower extremities. Again, several of these lesions had the clinical appearance of keratoacanthomas. Histopathology of lesions from her right cheek, left hip and left knee showed irregular epidermal acanthosis extending downward into the dermis forming endophytic lesions containing keratotic plugs and basophilic cellular debris. Eosinophilic collagen fibres were present within narrow perforating channels in the epidermis. These features were interpreted as being most consistent with a reactive perforating collagenosis. Additional biopsies from the left arm and from another lesion of the left knee showed similar endophytic lesions, but with epidermal rete ridge attenuation or disruption beneath the keratotic plug and associated granulation tissue and inflammation in the subjacent dermis. These changes were most consistent with hyperkeratosis follicularis et parafollicularis in cutem penetrans (Kyrle’s disease). The aetiology of APD is unknown; however, the pathogenesis may involve xerosis and chronic irritation secondary to pruritus leading to epithelial hyperplasia and eventually a disorder of perforation. Treatment modalities include ultraviolet B irradiation, photochemotherapy, topical and systemic retinoids, liquid nitrogen therapy and topical keratolytics such as salicylic acid.

A case of idiopathic calcinosis universalis.

Sir, Calcinosis is the result of disorganized amorphous deposition of calcium and phosphate in organic matrices of the biological tissues with no tendency to normal bone formation. The process starts with deposition of a small focus of hydroxyapatite and amorphous calcium phosphate. Once formed, it increases in size by growth and may result in disorganized masses as nodules or plaques (1, 2). We report here on a patient affected by idiopathic calcinosis universalis.