Progressive mucinous histiocytosis treated successfully with thalidomide: a rare case report

Figures & data

Figure 1. Erythematous papular lesions on the face.

Figure 2. (A) H&E staining shows a dense infiltrate involving the full thickness of the dermis. There is no grenz zone between the infiltrate and surface/follicular epithelium. -The infiltrate has a vaguely storiform growth pattern (x100) and is composed of epithelioid and spindle-shaped histiocytes arranged in a collagenous stroma with mucin deposition. There is also a perivascular lymphoplasmacytic infiltration in the dermis (x400). (B) CD68; Immunohistochemistry staining for CD68 markers shows positive staining in most histiocytes. (C) CD1a; CD1a is negative in tumoral cells but positive in normal tissue Langerhans cells. (D) S100; S100 is negative in tumoral cells and highlights normal tissue dendritic cells and Langerhans cells (x100).

Figure 3. There is abundant mucin deposition in the dermis (alcian blue stain, x400).

Figure 4. (A/B) Decrease in number and size of papules following one month of treatment with thalidomide.

Table 1. Review of literature on progressive mucinous histiocytosis hereditary and sporadic cases in chronological order.

	Author; year	Age (y/o)/sex	Clinical presentation	Other diseases	Time of presentation	Course
1	Misty M. Hobbs, 2021 (9)	30/female	Asymptomatic papules on the dorsal hands with recent involvement of both forearms	None	Childhood	No regression
2	A. Onoufriadis, 2020 (10)	30/female	Red skin tumors ranging from 2 to 10 mm developed on forearms then progressed to the trunk	None	Started at the age of 6	No regression
3	C Requena, 2017 (11)	32/female	Skin-colored asymptomatic 1–8 mm papules on the hands, forearms, face, and thighs	None	Started at the age of 5	Slow progression / no tendency to regress
4	C Requena, 2017 (11)	25/male	Symmetrical, firm, red-brown papules with nodules on hands, forearms, face, and legs	None	Started at the age of 6	Slow progression / no tendency to regress
5	C Requena, 2017 (11)	55/female	1–2 cm skin-colored to pink nodules on both arms	HIV and TB		No tendency to regress
6	MrCascarino/2017 (6)	33/female	Symmetrical multiple papules on both legs	None	Started at the age of 18	No regression
7	Jihane Abou Rahal/2016 (12)	20/female	Multiple facial papules	None	Childhood	Thalidomide (100 mg/d) decreased the size of the lesions and no new lesions developed
8	Nicholas V/ 2015 (5)	5/male	Skin-colored, asymptomatic, dome-shaped papules on the face and scalp	None	Four months after birth	Progressive
9	Nicholas V/ 2015 (5)	The father of the previous patient	Dome-shaped, erythematous papules on the face	None	Childhood	Removed using shave excision or electrodesiccation and curettage
10	Verónica Narváez-Rosales 2013 (13)	33/female	Red to brown dome-shaped nodules on the scalp, forearms, and lower extremities	None	Started at the age of 28	Treated with thalidomide (100 mg/day); no new lesions during one year of treatment
11	Christiane Schlegel/2010 (14)	65/female	Symmetrical, pruritic, small, red papules on forearms and scalp	HTN, hyperlipidemia, and depression	Started at the age of 25	Progressive; no tendency to resolve spontaneously
12	Christiane Schlegel/2010 (14)	40/male	Asymptomatic papules (less than 5 mm) on forearms and scalp	None	Started at the age of 20	Progressive; no tendency to resolve spontaneously
13	Iman Hemmati/2010 (7)	60/female	Asymptomatic papules (less than 5 mm) on arms, forearms, and dorsal hands	Hypothyroidism, GERD, migraine, depression, and sleep apnea	From several years	No regression
14	Young A/2006 (15)	48/female	Asymptomatic papules on the face, arms, and legs	None	Started at the age of 41	No spontaneous regression
15	Young A/2006 (15)	55/female	Asymptomatic papules on the face, arms, and legs	Asthma, COPD, HTN, osteoarthritis, sleep apnea, GERD, DM, and bipolar disorder	Started at the age of 50	No spontaneous regression
16	N Antoni-Bach/2000 (16)	49/female	Asymptomatic papules on the hands	None	Childhood	No spontaneous regression
17	U.SASS, J.ANDRE/2000 (17)	64/female	Dark-red, dome-shaped papulonodules on hands, forearms, and thighs	None	Childhood	No spontaneous resolution
18	D Wong/1999 (18)	61/female	Symmetrical papules on hands and forearms	None	Started at the age of 20	Persistent
19	Junichi Mizushima/ 1997 (19)	6/female	Multiple red to yellow nodules on extremities, scalp, face, and buttocks	None	From birth	No spontaneous regression
20	Kathrin Schroder/1996 (20)	20/female	2 mm asymptomatic papules on face, hands, and forearms	None	Started at the age of 27	No spontaneous regression
21	Kathrin Schroder/1996 (20)	46/female	Forearms and face	None	Started at age of 20	No spontaneous regression
22	K Bork/ 1994 (21)	52/female	Multiple papules	None	Longstanding lesions	No regression
23	K Bork/ 1994 (21)	25/female	Multiple papules	None	Longstanding lesions	No regression
24	K Bork/ 1988 (1)	Elderly/female	Extremities, trunk, face, and scalp	None	Early adolescence	No regression
25	K Bork/ 1988 (1)	Elderly/female	Extremities, trunk, face, and scalp	None	Early adolescence	No regression
26	K Bork/ 1988 (1)	Infant	Extremities, trunk, face, and scalp	None	Early adolescence	No regression

COPD: Chronic Obstructive Pulmonary Disease; DM: Diabetes Mellitus; GERD: Gastroesophageal Reflux Disease; HIV: Human Immunodeficiency Virus; HTN: Hypertension; TB: Tuberculosis.

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