A female child, a product of a nonconsanguineous parentage and normal pregnancy, was seen first at the age of 4 years. She had suffered from recurrent episodes of multiple, erythematous, confluent plaques and a few bu...A female child, a product of a nonconsanguineous parentage and normal pregnancy, was seen first at the age of 4 years. She had suffered from recurrent episodes of multiple, erythematous, confluent plaques and a few bullous lesions all over the body since infancy. The child was irritable but had no history of flushing, dyspnea, wheezing, or frequent diarrhea. Darier’s sign was strongly positive and systemic examination did not reveal any abnormality. Routine laboratory investigations were normal and a skin biopsy specimen revealed an unremarkable epidermis and dense proliferation of round to spindle-shaped mast cells which stained positive for metachromatic granules (Fig. 1). With the diagnosis of urticaria pigmentosa, she was managed with antihistaminic drugs and the parents were advised to handle the child gently. She did not report for further follow-up. Recently, the child, now 8 years of age, was brought again with finely wrinkled, lax, and redundant skin, especially of the face, giving her a senile appearance (Fig. 2). Systemic examination did not show any abnormality. History revealed that the child had been asymptomatic during the last 4 years even without treatment, but developed slowly progressive lax and pendulous skin. A repeat biopsy from the lax facial skin revealed sparse and fragmented elastic fibres, normal collagen, and a perivascular and intradermal cellular infiltrate composed of metachromatic mast cells. Her serum α1-antitrypsin levels were 190.0 mg/dL (normal, 93-224 mg/dL) and a pulmonary function test showed no abnormality. Treatment with antihistaminic drugs was started again and she was advised to have a cosmetic facial uplift at a later stage.展开更多
Background: Neurofibromatosis, type 1, is associated with cutaneous melanin pigmentation, but an association with ordinary melanocytic nevi has not been described. Methods: This retrospective case-control study was de...Background: Neurofibromatosis, type 1, is associated with cutaneous melanin pigmentation, but an association with ordinary melanocytic nevi has not been described. Methods: This retrospective case-control study was designed to see if neurofibromas in patients with neurofibromatosis, type 1 (NF-1) differ from sporadic neurofibromas (SN) in their incidence of associated melanocytic nevi and other histologic features. Slides from 114 NF-1 were compared with 112 SN and 300 intradermal melanocytic nevi (IDN). Results: Small lentiginous melanocytic nevi were identified over 13 NF-1 (11%) but no SN (P=0.0002). Compared with other NF-1, NF-1 with nevi were more frequently associated with melanocytic hyperplasia, giantmelanosomes and diffuse neurofibroma (P < 0.03). Compared with SN, NF-1 were also more frequently assoc iated with melanocytic hyperplasia, lentigo simplex-like changes, diffuse neuro fibroma and plexiform neurofibroma (P < 0.001). Sebaceous hyperplasia (14%), de rmal elastosis (9%), lipomatous change (8%), epithelial cysts (4%) and kerati n granulomas or folliculitis (3%) were not significantly different in prevalenc e between NF-1, SN and the control group of IDN. Conclusions: This study sugges ts that there is a difference in the potential for melanocytic proliferation in NF-1 compared with SN. NF-1, SN and IDN are associated with a similar range of incidental histologic changes.展开更多
Background: Pseudoepitheliomatous hyperplasia (PEH), a histological mimic of squamous cell carcinoma, is an exuberant reactive epithelial proliferation that may be induced by a variety of infectious, traumatic, inflam...Background: Pseudoepitheliomatous hyperplasia (PEH), a histological mimic of squamous cell carcinoma, is an exuberant reactive epithelial proliferation that may be induced by a variety of infectious, traumatic, inflammatory and neoplastic conditions of the skin and mucous membranes. PEH has been described in association with Spitz nevi and intramucosal nevi but not with oral malignant melanoma. Methods and results: A case of PEH in malignant melanoma of the palate in a 46-year-old female patient has been described. A search of the English literature did not disclose any previously reported case of such event. Conclusions: PEH associated with oral malignant melanoma is apparently very rare and most likely originates from the surface epithelium. This is in contrast with PEH in cutaneous melanoma where follicular or eccrine units have been suggested to be the origin.展开更多
Cutaneous mastocytosis (CM) or urticaria pigmentosa is characterized by abnormal proliferation and accumulation of mast cells. Clinically, CM usually presents as symmetrically distributed red- brown macules or papules...Cutaneous mastocytosis (CM) or urticaria pigmentosa is characterized by abnormal proliferation and accumulation of mast cells. Clinically, CM usually presents as symmetrically distributed red- brown macules or papules that develop weals, erythema and often pruritus on stroking (Darier’ s sign). The histological hallmark of the disease is an increase in oval to spindle- shaped mast cells in the dermis located around blood vessels and skin appendages. We describe three patients with a new clinicopathological type of CM, which clinically mimics a histiocytic disorder and histologically mimics leucocytoclastic vasculitis (LV). Three infants (two boys and one girl) developed generalized reddish- yellow- brown macules of 3- 10 cm with occasional scaling and crusting on the trunk and extremities without further symptoms or organ involvement except variable itching. Histology revealed diffuse and dense dermal infiltrates of eosinophils, neutrophils and nuclear debris with perivascular accentuation, imitating LV. This infiltrate masked large epithelioid cells, positive for macrophage markers, which by special histochemical stains for metachromatic granules turned out to be mast cells. This is the first report of this new variant of CM, which may cause considerable diagnostic difficulties both clinically and histopathologically.展开更多
文摘A female child, a product of a nonconsanguineous parentage and normal pregnancy, was seen first at the age of 4 years. She had suffered from recurrent episodes of multiple, erythematous, confluent plaques and a few bullous lesions all over the body since infancy. The child was irritable but had no history of flushing, dyspnea, wheezing, or frequent diarrhea. Darier’s sign was strongly positive and systemic examination did not reveal any abnormality. Routine laboratory investigations were normal and a skin biopsy specimen revealed an unremarkable epidermis and dense proliferation of round to spindle-shaped mast cells which stained positive for metachromatic granules (Fig. 1). With the diagnosis of urticaria pigmentosa, she was managed with antihistaminic drugs and the parents were advised to handle the child gently. She did not report for further follow-up. Recently, the child, now 8 years of age, was brought again with finely wrinkled, lax, and redundant skin, especially of the face, giving her a senile appearance (Fig. 2). Systemic examination did not show any abnormality. History revealed that the child had been asymptomatic during the last 4 years even without treatment, but developed slowly progressive lax and pendulous skin. A repeat biopsy from the lax facial skin revealed sparse and fragmented elastic fibres, normal collagen, and a perivascular and intradermal cellular infiltrate composed of metachromatic mast cells. Her serum α1-antitrypsin levels were 190.0 mg/dL (normal, 93-224 mg/dL) and a pulmonary function test showed no abnormality. Treatment with antihistaminic drugs was started again and she was advised to have a cosmetic facial uplift at a later stage.
文摘Background: Neurofibromatosis, type 1, is associated with cutaneous melanin pigmentation, but an association with ordinary melanocytic nevi has not been described. Methods: This retrospective case-control study was designed to see if neurofibromas in patients with neurofibromatosis, type 1 (NF-1) differ from sporadic neurofibromas (SN) in their incidence of associated melanocytic nevi and other histologic features. Slides from 114 NF-1 were compared with 112 SN and 300 intradermal melanocytic nevi (IDN). Results: Small lentiginous melanocytic nevi were identified over 13 NF-1 (11%) but no SN (P=0.0002). Compared with other NF-1, NF-1 with nevi were more frequently associated with melanocytic hyperplasia, giantmelanosomes and diffuse neurofibroma (P < 0.03). Compared with SN, NF-1 were also more frequently assoc iated with melanocytic hyperplasia, lentigo simplex-like changes, diffuse neuro fibroma and plexiform neurofibroma (P < 0.001). Sebaceous hyperplasia (14%), de rmal elastosis (9%), lipomatous change (8%), epithelial cysts (4%) and kerati n granulomas or folliculitis (3%) were not significantly different in prevalenc e between NF-1, SN and the control group of IDN. Conclusions: This study sugges ts that there is a difference in the potential for melanocytic proliferation in NF-1 compared with SN. NF-1, SN and IDN are associated with a similar range of incidental histologic changes.
文摘Background: Pseudoepitheliomatous hyperplasia (PEH), a histological mimic of squamous cell carcinoma, is an exuberant reactive epithelial proliferation that may be induced by a variety of infectious, traumatic, inflammatory and neoplastic conditions of the skin and mucous membranes. PEH has been described in association with Spitz nevi and intramucosal nevi but not with oral malignant melanoma. Methods and results: A case of PEH in malignant melanoma of the palate in a 46-year-old female patient has been described. A search of the English literature did not disclose any previously reported case of such event. Conclusions: PEH associated with oral malignant melanoma is apparently very rare and most likely originates from the surface epithelium. This is in contrast with PEH in cutaneous melanoma where follicular or eccrine units have been suggested to be the origin.
文摘Cutaneous mastocytosis (CM) or urticaria pigmentosa is characterized by abnormal proliferation and accumulation of mast cells. Clinically, CM usually presents as symmetrically distributed red- brown macules or papules that develop weals, erythema and often pruritus on stroking (Darier’ s sign). The histological hallmark of the disease is an increase in oval to spindle- shaped mast cells in the dermis located around blood vessels and skin appendages. We describe three patients with a new clinicopathological type of CM, which clinically mimics a histiocytic disorder and histologically mimics leucocytoclastic vasculitis (LV). Three infants (two boys and one girl) developed generalized reddish- yellow- brown macules of 3- 10 cm with occasional scaling and crusting on the trunk and extremities without further symptoms or organ involvement except variable itching. Histology revealed diffuse and dense dermal infiltrates of eosinophils, neutrophils and nuclear debris with perivascular accentuation, imitating LV. This infiltrate masked large epithelioid cells, positive for macrophage markers, which by special histochemical stains for metachromatic granules turned out to be mast cells. This is the first report of this new variant of CM, which may cause considerable diagnostic difficulties both clinically and histopathologically.
