We present the case of a patient with IgA paraprotein who developed hemorrhagic subepidermal vesicles and bullae with numerous neutrophils. Direct immunofluorescence test (DIF) showed weak deposits ...of IgA lambda paraprotein at the dermal–epidermal junction and at the intercellular level in the basal layer of the epidermis, and stronger deposits in a perivascular and diffuse pattern in the dermis. Indirect immunofluorescence (IIF) test revealed the presence of circulating IgA lambda antibodies reacting with the intercellular space of monkey and guinea pig esophagus and human skin. A blood test revealed an IgA lambda paraprotein and multiple myeloma stage I(0) was diagnosed in a later hematological study. Dapsone was prescribed and cutaneous lesions improved. This is the second report of subepidermal vesicles and bullae with dermal deposits of IgA paraprotein appearing prior to diagnosis of an IgA multiple myeloma, and it is a unique case with circulating IgA lambda antibodies reacting with the intercellular space of epithelia.
Barnadas MA, Gelpí C, Martínez L, Curell R, Martino R, Alomar A. Acquired bullous dermatosis associated with IgA multiple myeloma: a case report.
Introduction: Paraneoplastic pemphigus (PNP) is considered an autoimmune, multiorgan disease caused by antiplakin antibodies. We present three PNP patients who had negative epithelial direct ...immunofluorescence (DIF) findings in one or more biopsies.
Patients: An early lip biopsy of uninvolved oral epithelia in patient 1 was negative. A later biopsy from foreskin showed intense intercellular immunoglobulin G (IgG) deposits in the epithelia. In the early phase of the disease in patient 2, the intercellular fluorescence was negative in the epidermis, while intercellular IgG and C3 were observed in the sweat ducts. A later biopsy showed weak intercellular epidermal IgG and C3 fluorescence. Patient 3 showed intercellular IgG and/or C3 in follicular, sebaceous and sweat duct structures in several biopsies. No intercellular IgG or C3 was observed in the epithelia.
Discussion: The presence of immunoreactants in adnexal structures suggests that desmoplakins can be more strongly expressed in adnexa than in the epidermis, facilitating visualization of antibody deposits.
Conclusions: Negative DIF findings in epithelia do not rule out the diagnosis of PNP, and the presence of IgG and/or C3 at the intercellular level of adnexal structures can help establish this diagnosis.
We report the case of a 75-year old woman with collagenous colitis who presented with erythematous and edematous plaques on the periorbital and eyelid regions, accompanied by oral ulcers. ...Histopathology showed a dermal neutrophilic infiltrate plus mild septal and lobular panniculitis with lymphocytes, neutrophils and eosinophils. Five years earlier she had presented a flare of papules and vesicles on the trunk, together with oral ulcers; a skin biopsy revealed a neutrophilic dermal infiltrate and Sweet's syndrome was diagnosed. Both the neutrophilic panniculitis and the Sweet's syndrome were accompanied by fever, malaise and diarrhea. Cutaneous and intestinal symptoms disappeared with corticoid therapy. The two types of neutrophilic dermatoses that appeared in periods of colitis activity suggest that intestinal and cutaneous manifestations may be related.
We report a case of a 45-year-old woman who presented a simultaneous foreign-body granuloma reaction to silicone in her face and to silica in the elbow and knee. The patient had received silicone ...injections in her face 7 years earlier and had suffered a motorcycle accident when she was young. Changes suggestive of silicone were observed in the biopsy obtained from the face, and silica was detected in the biopsy taken from the elbow, confirmed by polarized light and X-ray microanalysis. The presence of polarizable foreign matter in cutaneous epithelioid granulomas should alert to the diagnosis of sarcoidosis.
We report the case of a 75-year old woman with collagenous colitis who presented with erythematous and edematous plaques on the periorbital and eyelid regions, accompanied by oral ulcers. ...Histopathology showed a dermal neutrophilic infiltrate plus mild septal and lobular panniculitis with lymphocytes, neutrophils and eosinophils. Five years earlier she had presented a flare of papules and vesicles on the trunk, together with oral ulcers; a skin biopsy revealed a neutrophilic dermal infiltrate and Sweet’s syndrome was diagnosed. Both the neutrophilic panniculitis and the Sweet’s syndrome were accompanied by fever, malaise and diarrhea. Cutaneous and intestinal symptoms disappeared with corticoid therapy. The two types of neutrophilic dermatoses that appeared in periods of colitis activity suggest that intestinal and cutaneous manifestations may be related.
It has been suggested that the use of sunscreens to prevent skin cancer may put the population at risk of vitamin D deficiency, which in turn may lead to secondary hyperparathyroidism, loss of ...cortical bone and, ultimately, osteoporotic fractures.
To investigate whether sunscreen SPF15 may lead to loss of bone mass.
We followed 10 sunscreen users and 18 controls over 2 years, including two summers, two winters and a basal period (winter). Bone mass was evaluated each season with dual x-ray absorptiometry.
During follow-up, mild fluctuations in bone mass could be seen at Ward's site in both groups, without a definitive pattern. At the final visit, no significant loss of bone mass was observed in sunscreen users or in the control group. We did not observe any significant differences between groups throughout the study.
Although the study samples in this work are small, and a slight variation in bone mass may not be detected, in a clinical setting, sunscreen SPF15 protection does not seem to increase the risk of osteoporosis.
We report a patient who presented with a papular pruritic eruption of a 3‐month duration that histologically showed suprabasal acantholysis accompanied of an eosinophilic inflammatory infiltrate that ...was consistent with the diagnosis of Grover's disease. Later, erythematous plaques and vesicles appeared which showed a histopathological pattern of eosinophilic spongiosis. The direct immunofluorescence (DIF) study showed lineal IgG and C′3 at the dermal epidermal junction which was consistent with the diagnosis of bullous pemphigoid. No anti‐intercellular deposits of immunoglobulin G (IgG) or C′3 were observed. We consider that suprabasal acantholysis may represent the early phase of bullous pemphigoid.
Post-radiotherapy cutaneous angiosarcomas have been described in different locations, including the breast. We present a case of cutaneous angiosarcoma of the breast, diagnosed 6 years after a ...carcinoma of the breast had been treated with radiation. The patient was a 44-year-old female with a history of ductal carcinoma (CA) of the right breast treated with tumorectomy, axillary lymphadenectomy, chemotherapy (FEC) and radiotherapy, who 6 years later presented with a violaceous, indurated plaque with a satellite nodule on the same breast. The histology of the skin biopsies showed angiomatous proliferation throughout the entire dermis, with no cellular atypia. Considering the patient's symptoms, history and the changes observed via mammography, it was decided to completely excise the lesion followed by a simple mastectomy, with the diagnosis of angiosarcoma being confirmed. The patient was later treated with paclitaxel, and the disease was apparently controlled. Despite this fact, two years and one month later, the angiosarcoma recurred on the internal area of the mastectomy scar. The patient was treated with paclitaxel, and surgical excision of the lesion area was once again performed. This complication usually appears 5-10 years after treatment with radiotherapy, so angiosarcoma should be ruled out if any angiomatous lesions later appear on skin that had been irradiated.